Physician compensation continued to rise in 2022 after suffering a dip in 2020, according to the Medscape Physician Compensation Report 2023: Your Income Versus Your Peers’. In addition, gender-based pay disparity among primary care physicians shrank, and the number of physicians who declined to take new Medicare patients rose.
 

The annual report is based on a survey of more than 10,000 physicians in over 29 specialties who answered questions about their income, workload, challenges, and level of satisfaction.

Average compensation across specialties rose to $352,000 – up nearly 17% from the 2018 average of $299,000. Fallout from the COVID-19 public health emergency continued to affect both physician compensation and job satisfaction, including Medicare reimbursements and staffing shortages due to burnout or retirement.

“Many physicians reevaluated what drove them to be a physician,” says Marc Adam, a recruiter at MASC Medical, a Florida physician recruiting firm.

Adam cites telehealth as an example. “An overwhelming majority of physicians prefer telehealth because of the convenience, but some really did not want to do it long term. They miss the patient interaction.”

The report also revealed that the gender-based pay gap in primary physicians fell, with men earning 19% more – down from 25% more in recent years. Among specialists, the gender gap was 27% on average, down from 31% last year. One reason may be an increase in compensation transparency, which Mr. Adam says should be the norm.

Income increases will likely continue, owing in large part to the growing disparity between physician supply and demand.

The projected physician shortage is expected to grow to 124,000 by 2034, according to the American Association of Medical Colleges. Federal lawmakers are considering passing the Resident Physician Shortage Reduction Act of 2023, which would add 14,000 Medicare-funded residency positions to help alleviate shortages.
 

Patient needs, Medicare rules continue to shift

Specialties with the biggest increases in compensation include oncology, anesthesiology, gastroenterology, radiology, critical care, and urology. Many procedure-related specialties saw more volume post pandemic.

Some respondents identified Medicare cuts and low reimbursement rates as a factor in tamping down compensation hikes. The number of physicians who expect to continue to take new Medicare patients is 65%, down from 71% 5 years ago.

For example, Medicare reimbursements for telehealth are expected to scale down in May, when the COVID-19 Public Health Emergency, which expanded telehealth services for Medicare patients, winds down.

“Telehealth will still exist,” says Mr. Adam, “but certain requirements will shape it going forward.”

Medicare isn’t viewed negatively across the board, however. Florida is among the top-earning states for physicians – along with Indiana, Connecticut, and Missouri. One reason is Florida’s unique health care environment, explains Mr. Adam, whose Florida-based firm places physicians nationwide.

“Florida is very progressive in terms of health care. For one thing, we have a large aging population and a large Medicare population.” Several growing organizations that focus on quality-based care are based in Florida, including ChenMed and Cano Health. Add to that the fact that owners of Florida’s health care organizations don’t have to be physicians, he explains, and the stage is set for experimentation.

“Being able to segment tasks frees up physicians to be more focused on medicine and provide better care while other people focus on the business and innovation.”

If Florida’s high compensation ranking continues, it may help employers there fulfill a growing need. The state is among those expected to experience the largest physician shortages in 2030, along with California, Texas, Arizona, and Georgia.
 

Side gigs up, satisfaction (slightly) down

In general, physicians aren’t fazed by these challenges. Many reported taking side gigs, some for additional income. Even so, 73% say they would still choose medicine, and more than 90% of physicians in 10 specialties would choose their specialty again. Still, burnout and stressors have led some to stop practicing altogether.

More and more organizations are hiring “travel physicians,” Mr. Adam says, and more physicians are choosing to take contract work (“locum tenens”) and practice in many different regions. Contract physicians typically help meet patient demand or provide coverage during the hiring process as well as while staff are on vacation or maternity leave.

Says Mr. Adam, “There’s no security, but there’s higher income and more flexibility.”

According to CHG Healthcare, locum tenens staffing is rising – approximately 7% of U.S. physicians (around 50,000) filled assignments in 2022, up 88% from 2015. In 2022, 56% of locum tenens employers reported a reduction in staff burnout, up from 30% in 2020.

The report indicates that more than half of physicians are satisfied with their income, down slightly from 55% 5 years ago (prepandemic). Physicians in some of the lower-paying specialties are among those most satisfied with their income. It’s not very surprising to Mr. Adam: “Higher earners generally suffer the most from burnout.

“They’re overworked, they have the largest number of patients, and they’re performing in high-stress situations doing challenging procedures on a daily basis – and they probably have worse work-life balance.” These physicians know going in that they need to be paid more to deal with such burdens. “That’s the feedback I get when I speak to high earners,” says Mr. Adam.

“The experienced ones are very clear about their [compensation] expectations.”

A version of this article first appeared on Medscape.com.

Physician compensation continued to rise in 2022 after suffering a dip in 2020, according to the Medscape Physician Compensation Report 2023: Your Income Versus Your Peers’. In addition, gender-based pay disparity among primary care physicians shrank, and the number of physicians who declined to take new Medicare patients rose.
 

The annual report is based on a survey of more than 10,000 physicians in over 29 specialties who answered questions about their income, workload, challenges, and level of satisfaction.

Average compensation across specialties rose to $352,000 – up nearly 17% from the 2018 average of $299,000. Fallout from the COVID-19 public health emergency continued to affect both physician compensation and job satisfaction, including Medicare reimbursements and staffing shortages due to burnout or retirement.

“Many physicians reevaluated what drove them to be a physician,” says Marc Adam, a recruiter at MASC Medical, a Florida physician recruiting firm.

Adam cites telehealth as an example. “An overwhelming majority of physicians prefer telehealth because of the convenience, but some really did not want to do it long term. They miss the patient interaction.”

The report also revealed that the gender-based pay gap in primary physicians fell, with men earning 19% more – down from 25% more in recent years. Among specialists, the gender gap was 27% on average, down from 31% last year. One reason may be an increase in compensation transparency, which Mr. Adam says should be the norm.

Income increases will likely continue, owing in large part to the growing disparity between physician supply and demand.

The projected physician shortage is expected to grow to 124,000 by 2034, according to the American Association of Medical Colleges. Federal lawmakers are considering passing the Resident Physician Shortage Reduction Act of 2023, which would add 14,000 Medicare-funded residency positions to help alleviate shortages.
 

Patient needs, Medicare rules continue to shift

Specialties with the biggest increases in compensation include oncology, anesthesiology, gastroenterology, radiology, critical care, and urology. Many procedure-related specialties saw more volume post pandemic.

Some respondents identified Medicare cuts and low reimbursement rates as a factor in tamping down compensation hikes. The number of physicians who expect to continue to take new Medicare patients is 65%, down from 71% 5 years ago.

For example, Medicare reimbursements for telehealth are expected to scale down in May, when the COVID-19 Public Health Emergency, which expanded telehealth services for Medicare patients, winds down.

“Telehealth will still exist,” says Mr. Adam, “but certain requirements will shape it going forward.”

Medicare isn’t viewed negatively across the board, however. Florida is among the top-earning states for physicians – along with Indiana, Connecticut, and Missouri. One reason is Florida’s unique health care environment, explains Mr. Adam, whose Florida-based firm places physicians nationwide.

“Florida is very progressive in terms of health care. For one thing, we have a large aging population and a large Medicare population.” Several growing organizations that focus on quality-based care are based in Florida, including ChenMed and Cano Health. Add to that the fact that owners of Florida’s health care organizations don’t have to be physicians, he explains, and the stage is set for experimentation.

“Being able to segment tasks frees up physicians to be more focused on medicine and provide better care while other people focus on the business and innovation.”

If Florida’s high compensation ranking continues, it may help employers there fulfill a growing need. The state is among those expected to experience the largest physician shortages in 2030, along with California, Texas, Arizona, and Georgia.
 

Side gigs up, satisfaction (slightly) down

In general, physicians aren’t fazed by these challenges. Many reported taking side gigs, some for additional income. Even so, 73% say they would still choose medicine, and more than 90% of physicians in 10 specialties would choose their specialty again. Still, burnout and stressors have led some to stop practicing altogether.

More and more organizations are hiring “travel physicians,” Mr. Adam says, and more physicians are choosing to take contract work (“locum tenens”) and practice in many different regions. Contract physicians typically help meet patient demand or provide coverage during the hiring process as well as while staff are on vacation or maternity leave.

Says Mr. Adam, “There’s no security, but there’s higher income and more flexibility.”

According to CHG Healthcare, locum tenens staffing is rising – approximately 7% of U.S. physicians (around 50,000) filled assignments in 2022, up 88% from 2015. In 2022, 56% of locum tenens employers reported a reduction in staff burnout, up from 30% in 2020.

The report indicates that more than half of physicians are satisfied with their income, down slightly from 55% 5 years ago (prepandemic). Physicians in some of the lower-paying specialties are among those most satisfied with their income. It’s not very surprising to Mr. Adam: “Higher earners generally suffer the most from burnout.

“They’re overworked, they have the largest number of patients, and they’re performing in high-stress situations doing challenging procedures on a daily basis – and they probably have worse work-life balance.” These physicians know going in that they need to be paid more to deal with such burdens. “That’s the feedback I get when I speak to high earners,” says Mr. Adam.

“The experienced ones are very clear about their [compensation] expectations.”

A version of this article first appeared on Medscape.com.

Physician compensation continued to rise in 2022 after suffering a dip in 2020, according to the Medscape Physician Compensation Report 2023: Your Income Versus Your Peers’. In addition, gender-based pay disparity among primary care physicians shrank, and the number of physicians who declined to take new Medicare patients rose.
 

The annual report is based on a survey of more than 10,000 physicians in over 29 specialties who answered questions about their income, workload, challenges, and level of satisfaction.

Average compensation across specialties rose to $352,000 – up nearly 17% from the 2018 average of $299,000. Fallout from the COVID-19 public health emergency continued to affect both physician compensation and job satisfaction, including Medicare reimbursements and staffing shortages due to burnout or retirement.

“Many physicians reevaluated what drove them to be a physician,” says Marc Adam, a recruiter at MASC Medical, a Florida physician recruiting firm.

Adam cites telehealth as an example. “An overwhelming majority of physicians prefer telehealth because of the convenience, but some really did not want to do it long term. They miss the patient interaction.”

The report also revealed that the gender-based pay gap in primary physicians fell, with men earning 19% more – down from 25% more in recent years. Among specialists, the gender gap was 27% on average, down from 31% last year. One reason may be an increase in compensation transparency, which Mr. Adam says should be the norm.

Income increases will likely continue, owing in large part to the growing disparity between physician supply and demand.

The projected physician shortage is expected to grow to 124,000 by 2034, according to the American Association of Medical Colleges. Federal lawmakers are considering passing the Resident Physician Shortage Reduction Act of 2023, which would add 14,000 Medicare-funded residency positions to help alleviate shortages.
 

Patient needs, Medicare rules continue to shift

Specialties with the biggest increases in compensation include oncology, anesthesiology, gastroenterology, radiology, critical care, and urology. Many procedure-related specialties saw more volume post pandemic.

Some respondents identified Medicare cuts and low reimbursement rates as a factor in tamping down compensation hikes. The number of physicians who expect to continue to take new Medicare patients is 65%, down from 71% 5 years ago.

For example, Medicare reimbursements for telehealth are expected to scale down in May, when the COVID-19 Public Health Emergency, which expanded telehealth services for Medicare patients, winds down.

“Telehealth will still exist,” says Mr. Adam, “but certain requirements will shape it going forward.”

Medicare isn’t viewed negatively across the board, however. Florida is among the top-earning states for physicians – along with Indiana, Connecticut, and Missouri. One reason is Florida’s unique health care environment, explains Mr. Adam, whose Florida-based firm places physicians nationwide.

“Florida is very progressive in terms of health care. For one thing, we have a large aging population and a large Medicare population.” Several growing organizations that focus on quality-based care are based in Florida, including ChenMed and Cano Health. Add to that the fact that owners of Florida’s health care organizations don’t have to be physicians, he explains, and the stage is set for experimentation.

“Being able to segment tasks frees up physicians to be more focused on medicine and provide better care while other people focus on the business and innovation.”

If Florida’s high compensation ranking continues, it may help employers there fulfill a growing need. The state is among those expected to experience the largest physician shortages in 2030, along with California, Texas, Arizona, and Georgia.
 

Side gigs up, satisfaction (slightly) down

In general, physicians aren’t fazed by these challenges. Many reported taking side gigs, some for additional income. Even so, 73% say they would still choose medicine, and more than 90% of physicians in 10 specialties would choose their specialty again. Still, burnout and stressors have led some to stop practicing altogether.

More and more organizations are hiring “travel physicians,” Mr. Adam says, and more physicians are choosing to take contract work (“locum tenens”) and practice in many different regions. Contract physicians typically help meet patient demand or provide coverage during the hiring process as well as while staff are on vacation or maternity leave.

Says Mr. Adam, “There’s no security, but there’s higher income and more flexibility.”

According to CHG Healthcare, locum tenens staffing is rising – approximately 7% of U.S. physicians (around 50,000) filled assignments in 2022, up 88% from 2015. In 2022, 56% of locum tenens employers reported a reduction in staff burnout, up from 30% in 2020.

The report indicates that more than half of physicians are satisfied with their income, down slightly from 55% 5 years ago (prepandemic). Physicians in some of the lower-paying specialties are among those most satisfied with their income. It’s not very surprising to Mr. Adam: “Higher earners generally suffer the most from burnout.

“They’re overworked, they have the largest number of patients, and they’re performing in high-stress situations doing challenging procedures on a daily basis – and they probably have worse work-life balance.” These physicians know going in that they need to be paid more to deal with such burdens. “That’s the feedback I get when I speak to high earners,” says Mr. Adam.

“The experienced ones are very clear about their [compensation] expectations.”

A version of this article first appeared on Medscape.com.

Dercum disease (or adiposis dolorosa) is a rare condition of unknown etiology characterized by multiple painful lipomas localized throughout the body.^1,2 It typically presents in adults aged 35 to 50 years and is at least 5 times more common in women.³ It often is associated with comorbidities such as obesity, fatigue and weakness.¹ There currently are no approved treatments for Dercum disease, only therapies tried with little to no efficacy for symptom management, including analgesics, excision, liposuction,¹ lymphatic drainage,⁴ hypobaric pressure,⁵ and frequency rhythmic electrical modulation systems.⁶ For patients who continually develop widespread lesions, surgical excision is not feasible, which poses a therapeutic challenge. Deoxycholic acid (DCA), a bile acid that is approved to treat submental fat, disrupts the integrity of cell membranes, induces adipocyte lysis, and solubilizes fat when injected subcutaneously.⁷ We used DCA to mitigate pain and reduce lipoma size in patients with Dercum disease, which demonstrated lipoma reduction via ultrasonography in 3 patients.

Case Reports

Three patients presented to clinic with multiple painful subcutaneous nodules throughout several areas of the body and were screened using radiography. Ultrasonography demonstrated numerous lipomas consistent with Dercum disease. The lipomas were measured by ultrasonography to obtain 3-dimensional measurements of each lesion. The most painful lipomas identified by the patients were either treated with 2 mL of DCA (10 mg/mL) or served as a control with no treatment. Patients returned for symptom monitoring and repeat measurements of both treated and untreated lipomas. Two physicians with expertise in ultrasonography measured lesions in a blinded fashion. Photographs were obtained with patient consent.

Patient 1—A 45-year-old woman with a family history of lipomas was diagnosed with Dercum disease that was confirmed via ultrasonography. A painful 1.63×1.64×0.55-cm lipoma was measured on the volar aspect of the left forearm, and a 1.17×1.26×0.39-cm lipoma was measured on the volar aspect of the right wrist. At a follow-up visit 11 months later, 2 mL of DCA was administered to the lipoma on the volar aspect of the left forearm, while the lipoma on the volar aspect of the right wrist was monitored as an untreated control. Following the procedure, the patient reported 1 week of swelling and tenderness of the treated area. Repeat imaging 4 months after administration of DCA revealed reduction of the treated lesion to 0.80×1.48×0.60 cm and growth of the untreated lesion to 1.32×2.17×0.52 cm. The treated lipoma reduced in volume by 34.55%, while the lipoma in the untreated control increased in volume from its original measurement by 111.11% (Table). The patient also reported decreased pain in the treated area at all follow-up visits in the 1 year following the procedure.

Patient 2—A 42-year-old woman with Dercum disease received administration of 2 mL of DCA to a 1.90×1.70×0.90-cm lipoma of the lateral aspect of the left mid thigh and 2 mL of DCA to a 2.40×3.07×0.60-cm lipoma on the volar aspect of the right forearm 2 weeks later. A 1.18×0.91×0.45-cm lipoma of the volar aspect of the left forearm was monitored as an untreated control. The patient reported bruising and discoloration a few weeks following the procedure. At subsequent 1-month and 3-month follow-ups, the patient reported induration in the volar aspect of the right forearm and noticeable reduction in size of the lesion in the lateral aspect of the left mid thigh. At the 6-month follow-up, the patient reported reduction in size of both lesions and improvement of the previously noted side effects. Repeat ultrasonography approximately 6 months after administration of DCA demonstrated reduction of the treated lesion on the lateral aspect of the left mid thigh to 0.92×0.96×0.57 cm and the volar aspect of the right forearm to 1.56×2.18×0.79 cm, with growth of the untreated lesion on the volar aspect of the left forearm to 1.37×1.11×0.39 cm. The treated lipomas reduced in volume by 68.42% and 41.25%, respectively, and the untreated control increased in volume by 22.08% (Table).

Patient 3—A 75-year-old woman with a family history of lipomas was diagnosed with Dercum disease verified by ultrasonography. The patient was administered 2 mL of DCA to a 2.65×3.19×0.71-cm lipoma of the volar aspect of the left forearm. A 1.66×2.02×0.38-cm lipoma of the lateral aspect of the right forearm was monitored as an untreated control. Following the procedure, the patient reported initial swelling that persisted for a few weeks followed by notable pain relief and a decrease in lipoma size. At 2-month follow-up, the patient reported no pain or other adverse effects, while repeat imaging demonstrated reduction of the treated lesion on the volar aspect of the left forearm to 2.13×2.56×0.75 cm and growth of the untreated lesion on the lateral aspect of the right forearm to 1.95×2.05×0.37 cm. The treated lipoma reduced in volume by 30.29%, and the untreated control increased in volume by 15.05% (Table).

Comment

Deoxycholic acid is a bile acid naturally found in the body that helps to emulsify and solubilize fats in the intestines. When injected subcutaneously, DCA becomes an adipolytic agent that induces inflammation and targets adipose degradation by macrophages, and it has been manufactured to reduce submental fat.⁷ Off-label use of DCA has been explored for nonsurgical body contouring and lipomas with promising results in some cases; however, these prior studies have been limited by the lack of quantitative objective measurements to effectively demonstrate the impact of treatment.^8,9

We present 3 patients who requested treatment for numerous painful lipomas. Given the extent of their disease, surgical options were not feasible, and the patients opted to try a nonsurgical alternative. In each case, the painful lipomas that were chosen for treatment were injected with 2 mL of DCA. Injection-associated symptoms included swelling, tenderness, discoloration, and induration, which resolved over a period of months. Patient 1 had a treated lipoma that reduced in volume by approximately 35%, while the control continued to grow and doubled in volume. In patient 2, the treated lesion on the lateral aspect of the mid thigh reduced in volume by almost 70%, and the treated lesion on the volar aspect of the right forearm reduced in volume by more than 40%, while the control grew by more than 20%. In patient 3, the volume of the treated lipoma decreased by 30%, and the control increased by 15%. The follow-up interval was shortest in patient 3—2 months as opposed to 11 months and 6 months for patients 1 and 2, respectively; therefore, more progress may be seen in patient 3 with more time. Interestingly, a change in shape of the lipoma was noted in patient 3 (Figure)—an increase in its depth while the center became anechoic, which is a sign of hollowing in the center due to the saponification of fat and a possible cause for the change from an elliptical to a more spherical or doughnutlike shape. Intralesional administration of DCA may offer patients with extensive lipomas, such as those seen in patients with Dercum disease, an alternative, less-invasive option to assist with pain and tumor burden when excision is not feasible. Although treatments with DCA can be associated with side effects, including pain, swelling, bruising, erythema, induration, and numbness, all 3 of our patients had ultimate mitigation of pain and reduction in lipoma size within months of the injection. Additional studies should be explored to determine the optimal dose and frequency of administration of DCA that could benefit patients with Dercum disease.

Dercum disease (or adiposis dolorosa) is a rare condition of unknown etiology characterized by multiple painful lipomas localized throughout the body.^1,2 It typically presents in adults aged 35 to 50 years and is at least 5 times more common in women.³ It often is associated with comorbidities such as obesity, fatigue and weakness.¹ There currently are no approved treatments for Dercum disease, only therapies tried with little to no efficacy for symptom management, including analgesics, excision, liposuction,¹ lymphatic drainage,⁴ hypobaric pressure,⁵ and frequency rhythmic electrical modulation systems.⁶ For patients who continually develop widespread lesions, surgical excision is not feasible, which poses a therapeutic challenge. Deoxycholic acid (DCA), a bile acid that is approved to treat submental fat, disrupts the integrity of cell membranes, induces adipocyte lysis, and solubilizes fat when injected subcutaneously.⁷ We used DCA to mitigate pain and reduce lipoma size in patients with Dercum disease, which demonstrated lipoma reduction via ultrasonography in 3 patients.

Case Reports

Three patients presented to clinic with multiple painful subcutaneous nodules throughout several areas of the body and were screened using radiography. Ultrasonography demonstrated numerous lipomas consistent with Dercum disease. The lipomas were measured by ultrasonography to obtain 3-dimensional measurements of each lesion. The most painful lipomas identified by the patients were either treated with 2 mL of DCA (10 mg/mL) or served as a control with no treatment. Patients returned for symptom monitoring and repeat measurements of both treated and untreated lipomas. Two physicians with expertise in ultrasonography measured lesions in a blinded fashion. Photographs were obtained with patient consent.

Patient 1—A 45-year-old woman with a family history of lipomas was diagnosed with Dercum disease that was confirmed via ultrasonography. A painful 1.63×1.64×0.55-cm lipoma was measured on the volar aspect of the left forearm, and a 1.17×1.26×0.39-cm lipoma was measured on the volar aspect of the right wrist. At a follow-up visit 11 months later, 2 mL of DCA was administered to the lipoma on the volar aspect of the left forearm, while the lipoma on the volar aspect of the right wrist was monitored as an untreated control. Following the procedure, the patient reported 1 week of swelling and tenderness of the treated area. Repeat imaging 4 months after administration of DCA revealed reduction of the treated lesion to 0.80×1.48×0.60 cm and growth of the untreated lesion to 1.32×2.17×0.52 cm. The treated lipoma reduced in volume by 34.55%, while the lipoma in the untreated control increased in volume from its original measurement by 111.11% (Table). The patient also reported decreased pain in the treated area at all follow-up visits in the 1 year following the procedure.

Patient 2—A 42-year-old woman with Dercum disease received administration of 2 mL of DCA to a 1.90×1.70×0.90-cm lipoma of the lateral aspect of the left mid thigh and 2 mL of DCA to a 2.40×3.07×0.60-cm lipoma on the volar aspect of the right forearm 2 weeks later. A 1.18×0.91×0.45-cm lipoma of the volar aspect of the left forearm was monitored as an untreated control. The patient reported bruising and discoloration a few weeks following the procedure. At subsequent 1-month and 3-month follow-ups, the patient reported induration in the volar aspect of the right forearm and noticeable reduction in size of the lesion in the lateral aspect of the left mid thigh. At the 6-month follow-up, the patient reported reduction in size of both lesions and improvement of the previously noted side effects. Repeat ultrasonography approximately 6 months after administration of DCA demonstrated reduction of the treated lesion on the lateral aspect of the left mid thigh to 0.92×0.96×0.57 cm and the volar aspect of the right forearm to 1.56×2.18×0.79 cm, with growth of the untreated lesion on the volar aspect of the left forearm to 1.37×1.11×0.39 cm. The treated lipomas reduced in volume by 68.42% and 41.25%, respectively, and the untreated control increased in volume by 22.08% (Table).

Patient 3—A 75-year-old woman with a family history of lipomas was diagnosed with Dercum disease verified by ultrasonography. The patient was administered 2 mL of DCA to a 2.65×3.19×0.71-cm lipoma of the volar aspect of the left forearm. A 1.66×2.02×0.38-cm lipoma of the lateral aspect of the right forearm was monitored as an untreated control. Following the procedure, the patient reported initial swelling that persisted for a few weeks followed by notable pain relief and a decrease in lipoma size. At 2-month follow-up, the patient reported no pain or other adverse effects, while repeat imaging demonstrated reduction of the treated lesion on the volar aspect of the left forearm to 2.13×2.56×0.75 cm and growth of the untreated lesion on the lateral aspect of the right forearm to 1.95×2.05×0.37 cm. The treated lipoma reduced in volume by 30.29%, and the untreated control increased in volume by 15.05% (Table).

Comment

Deoxycholic acid is a bile acid naturally found in the body that helps to emulsify and solubilize fats in the intestines. When injected subcutaneously, DCA becomes an adipolytic agent that induces inflammation and targets adipose degradation by macrophages, and it has been manufactured to reduce submental fat.⁷ Off-label use of DCA has been explored for nonsurgical body contouring and lipomas with promising results in some cases; however, these prior studies have been limited by the lack of quantitative objective measurements to effectively demonstrate the impact of treatment.^8,9

We present 3 patients who requested treatment for numerous painful lipomas. Given the extent of their disease, surgical options were not feasible, and the patients opted to try a nonsurgical alternative. In each case, the painful lipomas that were chosen for treatment were injected with 2 mL of DCA. Injection-associated symptoms included swelling, tenderness, discoloration, and induration, which resolved over a period of months. Patient 1 had a treated lipoma that reduced in volume by approximately 35%, while the control continued to grow and doubled in volume. In patient 2, the treated lesion on the lateral aspect of the mid thigh reduced in volume by almost 70%, and the treated lesion on the volar aspect of the right forearm reduced in volume by more than 40%, while the control grew by more than 20%. In patient 3, the volume of the treated lipoma decreased by 30%, and the control increased by 15%. The follow-up interval was shortest in patient 3—2 months as opposed to 11 months and 6 months for patients 1 and 2, respectively; therefore, more progress may be seen in patient 3 with more time. Interestingly, a change in shape of the lipoma was noted in patient 3 (Figure)—an increase in its depth while the center became anechoic, which is a sign of hollowing in the center due to the saponification of fat and a possible cause for the change from an elliptical to a more spherical or doughnutlike shape. Intralesional administration of DCA may offer patients with extensive lipomas, such as those seen in patients with Dercum disease, an alternative, less-invasive option to assist with pain and tumor burden when excision is not feasible. Although treatments with DCA can be associated with side effects, including pain, swelling, bruising, erythema, induration, and numbness, all 3 of our patients had ultimate mitigation of pain and reduction in lipoma size within months of the injection. Additional studies should be explored to determine the optimal dose and frequency of administration of DCA that could benefit patients with Dercum disease.

Dercum disease (or adiposis dolorosa) is a rare condition of unknown etiology characterized by multiple painful lipomas localized throughout the body.^1,2 It typically presents in adults aged 35 to 50 years and is at least 5 times more common in women.³ It often is associated with comorbidities such as obesity, fatigue and weakness.¹ There currently are no approved treatments for Dercum disease, only therapies tried with little to no efficacy for symptom management, including analgesics, excision, liposuction,¹ lymphatic drainage,⁴ hypobaric pressure,⁵ and frequency rhythmic electrical modulation systems.⁶ For patients who continually develop widespread lesions, surgical excision is not feasible, which poses a therapeutic challenge. Deoxycholic acid (DCA), a bile acid that is approved to treat submental fat, disrupts the integrity of cell membranes, induces adipocyte lysis, and solubilizes fat when injected subcutaneously.⁷ We used DCA to mitigate pain and reduce lipoma size in patients with Dercum disease, which demonstrated lipoma reduction via ultrasonography in 3 patients.

Case Reports

Three patients presented to clinic with multiple painful subcutaneous nodules throughout several areas of the body and were screened using radiography. Ultrasonography demonstrated numerous lipomas consistent with Dercum disease. The lipomas were measured by ultrasonography to obtain 3-dimensional measurements of each lesion. The most painful lipomas identified by the patients were either treated with 2 mL of DCA (10 mg/mL) or served as a control with no treatment. Patients returned for symptom monitoring and repeat measurements of both treated and untreated lipomas. Two physicians with expertise in ultrasonography measured lesions in a blinded fashion. Photographs were obtained with patient consent.

Patient 1—A 45-year-old woman with a family history of lipomas was diagnosed with Dercum disease that was confirmed via ultrasonography. A painful 1.63×1.64×0.55-cm lipoma was measured on the volar aspect of the left forearm, and a 1.17×1.26×0.39-cm lipoma was measured on the volar aspect of the right wrist. At a follow-up visit 11 months later, 2 mL of DCA was administered to the lipoma on the volar aspect of the left forearm, while the lipoma on the volar aspect of the right wrist was monitored as an untreated control. Following the procedure, the patient reported 1 week of swelling and tenderness of the treated area. Repeat imaging 4 months after administration of DCA revealed reduction of the treated lesion to 0.80×1.48×0.60 cm and growth of the untreated lesion to 1.32×2.17×0.52 cm. The treated lipoma reduced in volume by 34.55%, while the lipoma in the untreated control increased in volume from its original measurement by 111.11% (Table). The patient also reported decreased pain in the treated area at all follow-up visits in the 1 year following the procedure.

Patient 2—A 42-year-old woman with Dercum disease received administration of 2 mL of DCA to a 1.90×1.70×0.90-cm lipoma of the lateral aspect of the left mid thigh and 2 mL of DCA to a 2.40×3.07×0.60-cm lipoma on the volar aspect of the right forearm 2 weeks later. A 1.18×0.91×0.45-cm lipoma of the volar aspect of the left forearm was monitored as an untreated control. The patient reported bruising and discoloration a few weeks following the procedure. At subsequent 1-month and 3-month follow-ups, the patient reported induration in the volar aspect of the right forearm and noticeable reduction in size of the lesion in the lateral aspect of the left mid thigh. At the 6-month follow-up, the patient reported reduction in size of both lesions and improvement of the previously noted side effects. Repeat ultrasonography approximately 6 months after administration of DCA demonstrated reduction of the treated lesion on the lateral aspect of the left mid thigh to 0.92×0.96×0.57 cm and the volar aspect of the right forearm to 1.56×2.18×0.79 cm, with growth of the untreated lesion on the volar aspect of the left forearm to 1.37×1.11×0.39 cm. The treated lipomas reduced in volume by 68.42% and 41.25%, respectively, and the untreated control increased in volume by 22.08% (Table).

Patient 3—A 75-year-old woman with a family history of lipomas was diagnosed with Dercum disease verified by ultrasonography. The patient was administered 2 mL of DCA to a 2.65×3.19×0.71-cm lipoma of the volar aspect of the left forearm. A 1.66×2.02×0.38-cm lipoma of the lateral aspect of the right forearm was monitored as an untreated control. Following the procedure, the patient reported initial swelling that persisted for a few weeks followed by notable pain relief and a decrease in lipoma size. At 2-month follow-up, the patient reported no pain or other adverse effects, while repeat imaging demonstrated reduction of the treated lesion on the volar aspect of the left forearm to 2.13×2.56×0.75 cm and growth of the untreated lesion on the lateral aspect of the right forearm to 1.95×2.05×0.37 cm. The treated lipoma reduced in volume by 30.29%, and the untreated control increased in volume by 15.05% (Table).

Comment

Deoxycholic acid is a bile acid naturally found in the body that helps to emulsify and solubilize fats in the intestines. When injected subcutaneously, DCA becomes an adipolytic agent that induces inflammation and targets adipose degradation by macrophages, and it has been manufactured to reduce submental fat.⁷ Off-label use of DCA has been explored for nonsurgical body contouring and lipomas with promising results in some cases; however, these prior studies have been limited by the lack of quantitative objective measurements to effectively demonstrate the impact of treatment.^8,9

We present 3 patients who requested treatment for numerous painful lipomas. Given the extent of their disease, surgical options were not feasible, and the patients opted to try a nonsurgical alternative. In each case, the painful lipomas that were chosen for treatment were injected with 2 mL of DCA. Injection-associated symptoms included swelling, tenderness, discoloration, and induration, which resolved over a period of months. Patient 1 had a treated lipoma that reduced in volume by approximately 35%, while the control continued to grow and doubled in volume. In patient 2, the treated lesion on the lateral aspect of the mid thigh reduced in volume by almost 70%, and the treated lesion on the volar aspect of the right forearm reduced in volume by more than 40%, while the control grew by more than 20%. In patient 3, the volume of the treated lipoma decreased by 30%, and the control increased by 15%. The follow-up interval was shortest in patient 3—2 months as opposed to 11 months and 6 months for patients 1 and 2, respectively; therefore, more progress may be seen in patient 3 with more time. Interestingly, a change in shape of the lipoma was noted in patient 3 (Figure)—an increase in its depth while the center became anechoic, which is a sign of hollowing in the center due to the saponification of fat and a possible cause for the change from an elliptical to a more spherical or doughnutlike shape. Intralesional administration of DCA may offer patients with extensive lipomas, such as those seen in patients with Dercum disease, an alternative, less-invasive option to assist with pain and tumor burden when excision is not feasible. Although treatments with DCA can be associated with side effects, including pain, swelling, bruising, erythema, induration, and numbness, all 3 of our patients had ultimate mitigation of pain and reduction in lipoma size within months of the injection. Additional studies should be explored to determine the optimal dose and frequency of administration of DCA that could benefit patients with Dercum disease.

Napping for more than half an hour during the day was associated with a 90% increased risk of atrial fibrillation (AFib), but shorter naps were linked to a reduced risk, based on data from more than 20,000 individuals.

“Short daytime napping is a common, healthy habit, especially in Mediterranean countries,” Jesus Diaz-Gutierrez, MD, of Juan Ramon Jimenez University Hospital, Huelva, Spain, said in a presentation at the annual congress of the European Association of Preventive Cardiology (EAPC).

Judith Shidlowsky/Pixabay

Previous studies have shown a potential link between sleep patterns and AFib risk, but the association between specific duration of daytime naps and AFib risk has not been explored, he said.

Dr. Diaz-Gutierrez and colleagues used data from the University of Navarra Follow-up (SUN) Project, a prospective cohort of Spanish university graduates, to explore the possible link between naps and AFib. The study population included 20,348 individuals without AFib at baseline who were followed for a median of 13.8 years. The average age of participants at baseline was 38 years; 61% were women.

Daytime napping patterns were assessed at baseline, and participants were divided into nap groups of short nappers (defined as less than 30 minutes per day), and longer nappers (30 minutes or more per day), and those who reported no napping.

The researchers identified 131 incident cases of AFib during the follow-up period. Overall, the relative risk of incident AFib was significantly higher for the long nappers (adjusted hazard ratio 1.90) compared with short nappers in a multivariate analysis, while no significant risk appeared among non-nappers compared to short nappers (aHR 1.26).

The researchers then excluded the non-nappers in a secondary analysis to explore the impact of more specific daily nap duration on AFib risk. In a multivariate analysis, they found a 42% reduced risk of AF among those who napped for less than 15 minutes, and a 56% reduced risk for those who napped for 15-30 minutes, compared with those who napped for more than 30 minutes (aHR 0.56 and 0.42, respectively).

Potential explanations for the associations include the role of circadian rhythms, Dr. Diaz-Gutierrez said in a press release accompanying the presentation at the meeting. “Long daytime naps may disrupt the body’s internal clock (circadian rhythm), leading to shorter nighttime sleep, more nocturnal awakening, and reduced physical activity. In contrast, short daytime napping may improve circadian rhythm, lower blood pressure levels, and reduce stress.” More research is needed to validate the findings and the optimum nap duration, and whether a short nap is more advantageous than not napping in terms of AFib risk reduction, he said.

The study results suggest that naps of 15-30 minutes represent “a potential novel healthy lifestyle habit in the primary prevention of AFib,” Dr. Diaz-Gutierrez said in his presentation. However, the results also suggest that daily naps be limited to less than 30 minutes, he concluded.
 

Sleep habits may serve as red flag

“As we age, most if not all of us will develop sleep disturbances, such as insomnia, obstructive sleep apnea (OSA), and other sleep issues,” Lawrence S. Rosenthal, MD, of the University of Massachusetts, Worcester, said in an interview.

Therefore, “this study is near and dear to most people, and most would agree that poor sleeping habits affect our health.” In particular, OSA has been linked to AFib, although that was not measured in the current study, he added.

Dr. Rosenthal said he was not surprised by the current study findings. “It seems that a quick recharge of your ‘battery’ during the day is healthier than a long, deep sleep daytime nap,” he said. In addition, “Longer naps may be a marker of OSA,” he noted.

For clinicians, the take-home message of the current study is the need to consider underlying medical conditions in patients who regularly take long afternoon naps, and to consider these longer naps as a potential marker for AFib, said Dr. Rosenthal.

Looking ahead, a “deeper dive into the makeup of the populations studied” would be useful as a foundation for additional research, he said.

The SUN Project disclosed funding from the Spanish Government-Instituto de Salud Carlos III and the European Regional Development Fund (FEDER), the Navarra Regional Government, Plan Nacional Sobre Drogas, the University of Navarra, and the European Research Council. The researchers, and Dr. Rosenthal, had no financial conflicts to disclose.

Napping for more than half an hour during the day was associated with a 90% increased risk of atrial fibrillation (AFib), but shorter naps were linked to a reduced risk, based on data from more than 20,000 individuals.

“Short daytime napping is a common, healthy habit, especially in Mediterranean countries,” Jesus Diaz-Gutierrez, MD, of Juan Ramon Jimenez University Hospital, Huelva, Spain, said in a presentation at the annual congress of the European Association of Preventive Cardiology (EAPC).

Judith Shidlowsky/Pixabay

Previous studies have shown a potential link between sleep patterns and AFib risk, but the association between specific duration of daytime naps and AFib risk has not been explored, he said.

Dr. Diaz-Gutierrez and colleagues used data from the University of Navarra Follow-up (SUN) Project, a prospective cohort of Spanish university graduates, to explore the possible link between naps and AFib. The study population included 20,348 individuals without AFib at baseline who were followed for a median of 13.8 years. The average age of participants at baseline was 38 years; 61% were women.

Daytime napping patterns were assessed at baseline, and participants were divided into nap groups of short nappers (defined as less than 30 minutes per day), and longer nappers (30 minutes or more per day), and those who reported no napping.

The researchers identified 131 incident cases of AFib during the follow-up period. Overall, the relative risk of incident AFib was significantly higher for the long nappers (adjusted hazard ratio 1.90) compared with short nappers in a multivariate analysis, while no significant risk appeared among non-nappers compared to short nappers (aHR 1.26).

The researchers then excluded the non-nappers in a secondary analysis to explore the impact of more specific daily nap duration on AFib risk. In a multivariate analysis, they found a 42% reduced risk of AF among those who napped for less than 15 minutes, and a 56% reduced risk for those who napped for 15-30 minutes, compared with those who napped for more than 30 minutes (aHR 0.56 and 0.42, respectively).

Potential explanations for the associations include the role of circadian rhythms, Dr. Diaz-Gutierrez said in a press release accompanying the presentation at the meeting. “Long daytime naps may disrupt the body’s internal clock (circadian rhythm), leading to shorter nighttime sleep, more nocturnal awakening, and reduced physical activity. In contrast, short daytime napping may improve circadian rhythm, lower blood pressure levels, and reduce stress.” More research is needed to validate the findings and the optimum nap duration, and whether a short nap is more advantageous than not napping in terms of AFib risk reduction, he said.

The study results suggest that naps of 15-30 minutes represent “a potential novel healthy lifestyle habit in the primary prevention of AFib,” Dr. Diaz-Gutierrez said in his presentation. However, the results also suggest that daily naps be limited to less than 30 minutes, he concluded.
 

Sleep habits may serve as red flag

“As we age, most if not all of us will develop sleep disturbances, such as insomnia, obstructive sleep apnea (OSA), and other sleep issues,” Lawrence S. Rosenthal, MD, of the University of Massachusetts, Worcester, said in an interview.

Therefore, “this study is near and dear to most people, and most would agree that poor sleeping habits affect our health.” In particular, OSA has been linked to AFib, although that was not measured in the current study, he added.

Dr. Rosenthal said he was not surprised by the current study findings. “It seems that a quick recharge of your ‘battery’ during the day is healthier than a long, deep sleep daytime nap,” he said. In addition, “Longer naps may be a marker of OSA,” he noted.

For clinicians, the take-home message of the current study is the need to consider underlying medical conditions in patients who regularly take long afternoon naps, and to consider these longer naps as a potential marker for AFib, said Dr. Rosenthal.

Looking ahead, a “deeper dive into the makeup of the populations studied” would be useful as a foundation for additional research, he said.

The SUN Project disclosed funding from the Spanish Government-Instituto de Salud Carlos III and the European Regional Development Fund (FEDER), the Navarra Regional Government, Plan Nacional Sobre Drogas, the University of Navarra, and the European Research Council. The researchers, and Dr. Rosenthal, had no financial conflicts to disclose.

Napping for more than half an hour during the day was associated with a 90% increased risk of atrial fibrillation (AFib), but shorter naps were linked to a reduced risk, based on data from more than 20,000 individuals.

“Short daytime napping is a common, healthy habit, especially in Mediterranean countries,” Jesus Diaz-Gutierrez, MD, of Juan Ramon Jimenez University Hospital, Huelva, Spain, said in a presentation at the annual congress of the European Association of Preventive Cardiology (EAPC).

Judith Shidlowsky/Pixabay

Previous studies have shown a potential link between sleep patterns and AFib risk, but the association between specific duration of daytime naps and AFib risk has not been explored, he said.

Dr. Diaz-Gutierrez and colleagues used data from the University of Navarra Follow-up (SUN) Project, a prospective cohort of Spanish university graduates, to explore the possible link between naps and AFib. The study population included 20,348 individuals without AFib at baseline who were followed for a median of 13.8 years. The average age of participants at baseline was 38 years; 61% were women.

Daytime napping patterns were assessed at baseline, and participants were divided into nap groups of short nappers (defined as less than 30 minutes per day), and longer nappers (30 minutes or more per day), and those who reported no napping.

The researchers identified 131 incident cases of AFib during the follow-up period. Overall, the relative risk of incident AFib was significantly higher for the long nappers (adjusted hazard ratio 1.90) compared with short nappers in a multivariate analysis, while no significant risk appeared among non-nappers compared to short nappers (aHR 1.26).

The researchers then excluded the non-nappers in a secondary analysis to explore the impact of more specific daily nap duration on AFib risk. In a multivariate analysis, they found a 42% reduced risk of AF among those who napped for less than 15 minutes, and a 56% reduced risk for those who napped for 15-30 minutes, compared with those who napped for more than 30 minutes (aHR 0.56 and 0.42, respectively).

Potential explanations for the associations include the role of circadian rhythms, Dr. Diaz-Gutierrez said in a press release accompanying the presentation at the meeting. “Long daytime naps may disrupt the body’s internal clock (circadian rhythm), leading to shorter nighttime sleep, more nocturnal awakening, and reduced physical activity. In contrast, short daytime napping may improve circadian rhythm, lower blood pressure levels, and reduce stress.” More research is needed to validate the findings and the optimum nap duration, and whether a short nap is more advantageous than not napping in terms of AFib risk reduction, he said.

The study results suggest that naps of 15-30 minutes represent “a potential novel healthy lifestyle habit in the primary prevention of AFib,” Dr. Diaz-Gutierrez said in his presentation. However, the results also suggest that daily naps be limited to less than 30 minutes, he concluded.
 

Sleep habits may serve as red flag

“As we age, most if not all of us will develop sleep disturbances, such as insomnia, obstructive sleep apnea (OSA), and other sleep issues,” Lawrence S. Rosenthal, MD, of the University of Massachusetts, Worcester, said in an interview.

Therefore, “this study is near and dear to most people, and most would agree that poor sleeping habits affect our health.” In particular, OSA has been linked to AFib, although that was not measured in the current study, he added.

Dr. Rosenthal said he was not surprised by the current study findings. “It seems that a quick recharge of your ‘battery’ during the day is healthier than a long, deep sleep daytime nap,” he said. In addition, “Longer naps may be a marker of OSA,” he noted.

For clinicians, the take-home message of the current study is the need to consider underlying medical conditions in patients who regularly take long afternoon naps, and to consider these longer naps as a potential marker for AFib, said Dr. Rosenthal.

Looking ahead, a “deeper dive into the makeup of the populations studied” would be useful as a foundation for additional research, he said.

The SUN Project disclosed funding from the Spanish Government-Instituto de Salud Carlos III and the European Regional Development Fund (FEDER), the Navarra Regional Government, Plan Nacional Sobre Drogas, the University of Navarra, and the European Research Council. The researchers, and Dr. Rosenthal, had no financial conflicts to disclose.

More than one in three children and nearly three in five adults take dietary supplements in the United States, a new report shows.

The new figures continue a 15-year trend of small, steady increases in how many people in the United States use the products that can deliver essential nutrients, but their usage includes a risk of getting more nutrients than recommended. In 2007, 48% of adults took supplements, and that figure has reached nearly 59% in this latest count.

The new report looked at whether people took a multivitamin, as well as other more specific supplements. Among children and adolescents aged 19 and under, 23.5% took a multivitamin, while 31.5% of adults reported taking one. The most common specialized supplement that people took was vitamin D.

The report, released by the CDC’s National Center for Health Statistics, compiled survey data from 2017 through 2020 in which 15,548 people reported their household’s usage of dietary supplements. Dietary supplements include vitamins, minerals, herbs, or other botanicals that are taken by mouth in pill, capsule, tablet, or liquid form. The researchers said the vitamin and supplement market is large and growing, totaling $55.7 billion in sales in 2020.

More than one-third of adults (36%) reported taking more than one supplement, and one in four people aged 60 and older said they took four or more.

The data showed demographic trends in who uses dietary supplements. Women and girls were more likely to take supplements than men and boys, although there were similar usage levels for both genders among 1- to 2-year-olds. People with higher education or income levels were more likely to use supplements. Asian people and White people were more likely to take supplements, compared with Hispanic people and Black people.

The authors wrote that monitoring trends in supplement use is important because the products “contribute substantially to nutrient intake as well as increase the risk of excessive intake of certain micronutrients.”

A version of this article originally appeared on WebMD.com.

More than one in three children and nearly three in five adults take dietary supplements in the United States, a new report shows.

The new figures continue a 15-year trend of small, steady increases in how many people in the United States use the products that can deliver essential nutrients, but their usage includes a risk of getting more nutrients than recommended. In 2007, 48% of adults took supplements, and that figure has reached nearly 59% in this latest count.

The new report looked at whether people took a multivitamin, as well as other more specific supplements. Among children and adolescents aged 19 and under, 23.5% took a multivitamin, while 31.5% of adults reported taking one. The most common specialized supplement that people took was vitamin D.

The report, released by the CDC’s National Center for Health Statistics, compiled survey data from 2017 through 2020 in which 15,548 people reported their household’s usage of dietary supplements. Dietary supplements include vitamins, minerals, herbs, or other botanicals that are taken by mouth in pill, capsule, tablet, or liquid form. The researchers said the vitamin and supplement market is large and growing, totaling $55.7 billion in sales in 2020.

More than one-third of adults (36%) reported taking more than one supplement, and one in four people aged 60 and older said they took four or more.

The data showed demographic trends in who uses dietary supplements. Women and girls were more likely to take supplements than men and boys, although there were similar usage levels for both genders among 1- to 2-year-olds. People with higher education or income levels were more likely to use supplements. Asian people and White people were more likely to take supplements, compared with Hispanic people and Black people.

The authors wrote that monitoring trends in supplement use is important because the products “contribute substantially to nutrient intake as well as increase the risk of excessive intake of certain micronutrients.”

A version of this article originally appeared on WebMD.com.

More than one in three children and nearly three in five adults take dietary supplements in the United States, a new report shows.

The new figures continue a 15-year trend of small, steady increases in how many people in the United States use the products that can deliver essential nutrients, but their usage includes a risk of getting more nutrients than recommended. In 2007, 48% of adults took supplements, and that figure has reached nearly 59% in this latest count.

The new report looked at whether people took a multivitamin, as well as other more specific supplements. Among children and adolescents aged 19 and under, 23.5% took a multivitamin, while 31.5% of adults reported taking one. The most common specialized supplement that people took was vitamin D.

The report, released by the CDC’s National Center for Health Statistics, compiled survey data from 2017 through 2020 in which 15,548 people reported their household’s usage of dietary supplements. Dietary supplements include vitamins, minerals, herbs, or other botanicals that are taken by mouth in pill, capsule, tablet, or liquid form. The researchers said the vitamin and supplement market is large and growing, totaling $55.7 billion in sales in 2020.

More than one-third of adults (36%) reported taking more than one supplement, and one in four people aged 60 and older said they took four or more.

The data showed demographic trends in who uses dietary supplements. Women and girls were more likely to take supplements than men and boys, although there were similar usage levels for both genders among 1- to 2-year-olds. People with higher education or income levels were more likely to use supplements. Asian people and White people were more likely to take supplements, compared with Hispanic people and Black people.

The authors wrote that monitoring trends in supplement use is important because the products “contribute substantially to nutrient intake as well as increase the risk of excessive intake of certain micronutrients.”

A version of this article originally appeared on WebMD.com.

The Diagnosis: Neonatal-Onset Multisystem Inflammatory Disorder (NOMID)

The punch biopsy demonstrated a predominantly deep but somewhat superficial, periadnexal, neutrophilic and eosinophilic infiltrate (Figure). The eruption resolved 3 days later with supportive treatment, including appropriate wound care. Genetic analysis revealed an autosomal-dominant NLR family pyrin domain containing 3 gene, NLRP3, de novo variant associated with neonatal-onset multisystem inflammatory disorder (NOMID). Additional workup to characterize our patient’s inflammatory profile revealed elevated IL-18, CD3, CD4, S100A12, and S100A8/A9 levels. On day 48 of life, she was started on anakinra, an IL-1 inhibitor, at a dose of 1 mg/kg subcutaneously, which eventually was titrated to 10 mg/kg at hospital discharge. Hearing screenings were within normal limits.

Cryopyrin-associated periodic syndromes (CAPS) consist of 3 rare, IL-1–associated, autoinflammatory disorders, including familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), and NOMID (also known as chronic infantile neurologic cutaneous and articular syndrome). These conditions result from a sporadic or autosomal-dominant gain-of-function mutations in a single gene, NLRP3, on chromosome 1q44. NLRP3 encodes for cryopyrin, an important component of an IL-1 and IL-18 activating inflammasome.¹ The most severe manifestation of CAPS is NOMID, which typically presents at birth as a migratory urticarial eruption, growth failure, myalgia, fever, and abnormal facial features, including frontal bossing, saddle-shaped nose, and protruding eyes.² The illness also can manifest with hepatosplenomegaly, lymphadenopathy, uveitis, sensorineural hearing loss, cerebral atrophy, and other neurologic manifestations.³ A diagnosis of chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome was less likely given that our patient remained afebrile and did not show signs of lipodystrophy and persistent violaceous eyelid swelling. Both FCAS and MWS are less severe forms of CAPS when compared to NOMID. Familial cold autoinflammatory syndrome was less likely given the absence of the typical periodic fever pattern associated with the condition and severity of our patient’s symptoms. Muckle-Wells syndrome typically presents in adolescence with symptoms of FCAS, painful urticarial plaques, and progressive sensorinueral hearing loss. Tumor necrosis factor receptor–associated periodic fever (TRAPS) usually is associated with episodic fevers, abdominal pain, periorbital edema, migratory erythema, and arthralgia.^1,3,4

Diagnostic criteria for CAPS include elevated inflammatory markers and serum amyloid, plus at least 2 of the typical CAPS symptoms: urticarial rash, cold-triggered episodes, sensorineural hearing loss, musculoskeletal symptoms, chronic aseptic meningitis, and skeletal abnormalities.⁴ The sensitivity and specificity of these diagnostic criteria are 84% and 91%, respectively. Additional findings that can be seen but are not part of the diagnostic criteria include intermittent fever, transient joint swelling, bony overgrowths, uveitis, optic disc edema, impaired growth, and hepatosplenomegaly.⁵ Laboratory findings may reveal leukocytosis, eosinophilia, anemia, and/or thrombocytopenia.^3,5

Genetic testing, skin biopsies, ophthalmic examinations, neuroimaging, joint radiography, cerebrospinal fluid tests, and hearing examinations can be performed for confirmation of diagnosis and evaluation of systemic complications.⁴ A skin biopsy may reveal a neutrophilic infiltrate. Ophthalmic examination can demonstrate uveitis and optic disk edema. Neuroimaging may reveal cerebral atrophy or ventricular dilation. Lastly, joint radiography can be used to evaluate for the presence of premature long bone ossification or osseous overgrowth.⁴

In summary, NOMID is a multisystemic disorder with cutaneous manifestations. Early recognition of this entity is important given the severe sequelae and available efficacious therapy. Dermatologists should be aware of these manifestations, as dermatologic consultation and a skin biopsy may aid in diagnosis.

The Diagnosis: Neonatal-Onset Multisystem Inflammatory Disorder (NOMID)

The punch biopsy demonstrated a predominantly deep but somewhat superficial, periadnexal, neutrophilic and eosinophilic infiltrate (Figure). The eruption resolved 3 days later with supportive treatment, including appropriate wound care. Genetic analysis revealed an autosomal-dominant NLR family pyrin domain containing 3 gene, NLRP3, de novo variant associated with neonatal-onset multisystem inflammatory disorder (NOMID). Additional workup to characterize our patient’s inflammatory profile revealed elevated IL-18, CD3, CD4, S100A12, and S100A8/A9 levels. On day 48 of life, she was started on anakinra, an IL-1 inhibitor, at a dose of 1 mg/kg subcutaneously, which eventually was titrated to 10 mg/kg at hospital discharge. Hearing screenings were within normal limits.

Cryopyrin-associated periodic syndromes (CAPS) consist of 3 rare, IL-1–associated, autoinflammatory disorders, including familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), and NOMID (also known as chronic infantile neurologic cutaneous and articular syndrome). These conditions result from a sporadic or autosomal-dominant gain-of-function mutations in a single gene, NLRP3, on chromosome 1q44. NLRP3 encodes for cryopyrin, an important component of an IL-1 and IL-18 activating inflammasome.¹ The most severe manifestation of CAPS is NOMID, which typically presents at birth as a migratory urticarial eruption, growth failure, myalgia, fever, and abnormal facial features, including frontal bossing, saddle-shaped nose, and protruding eyes.² The illness also can manifest with hepatosplenomegaly, lymphadenopathy, uveitis, sensorineural hearing loss, cerebral atrophy, and other neurologic manifestations.³ A diagnosis of chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome was less likely given that our patient remained afebrile and did not show signs of lipodystrophy and persistent violaceous eyelid swelling. Both FCAS and MWS are less severe forms of CAPS when compared to NOMID. Familial cold autoinflammatory syndrome was less likely given the absence of the typical periodic fever pattern associated with the condition and severity of our patient’s symptoms. Muckle-Wells syndrome typically presents in adolescence with symptoms of FCAS, painful urticarial plaques, and progressive sensorinueral hearing loss. Tumor necrosis factor receptor–associated periodic fever (TRAPS) usually is associated with episodic fevers, abdominal pain, periorbital edema, migratory erythema, and arthralgia.^1,3,4

Diagnostic criteria for CAPS include elevated inflammatory markers and serum amyloid, plus at least 2 of the typical CAPS symptoms: urticarial rash, cold-triggered episodes, sensorineural hearing loss, musculoskeletal symptoms, chronic aseptic meningitis, and skeletal abnormalities.⁴ The sensitivity and specificity of these diagnostic criteria are 84% and 91%, respectively. Additional findings that can be seen but are not part of the diagnostic criteria include intermittent fever, transient joint swelling, bony overgrowths, uveitis, optic disc edema, impaired growth, and hepatosplenomegaly.⁵ Laboratory findings may reveal leukocytosis, eosinophilia, anemia, and/or thrombocytopenia.^3,5

Genetic testing, skin biopsies, ophthalmic examinations, neuroimaging, joint radiography, cerebrospinal fluid tests, and hearing examinations can be performed for confirmation of diagnosis and evaluation of systemic complications.⁴ A skin biopsy may reveal a neutrophilic infiltrate. Ophthalmic examination can demonstrate uveitis and optic disk edema. Neuroimaging may reveal cerebral atrophy or ventricular dilation. Lastly, joint radiography can be used to evaluate for the presence of premature long bone ossification or osseous overgrowth.⁴

In summary, NOMID is a multisystemic disorder with cutaneous manifestations. Early recognition of this entity is important given the severe sequelae and available efficacious therapy. Dermatologists should be aware of these manifestations, as dermatologic consultation and a skin biopsy may aid in diagnosis.

The Diagnosis: Neonatal-Onset Multisystem Inflammatory Disorder (NOMID)

The punch biopsy demonstrated a predominantly deep but somewhat superficial, periadnexal, neutrophilic and eosinophilic infiltrate (Figure). The eruption resolved 3 days later with supportive treatment, including appropriate wound care. Genetic analysis revealed an autosomal-dominant NLR family pyrin domain containing 3 gene, NLRP3, de novo variant associated with neonatal-onset multisystem inflammatory disorder (NOMID). Additional workup to characterize our patient’s inflammatory profile revealed elevated IL-18, CD3, CD4, S100A12, and S100A8/A9 levels. On day 48 of life, she was started on anakinra, an IL-1 inhibitor, at a dose of 1 mg/kg subcutaneously, which eventually was titrated to 10 mg/kg at hospital discharge. Hearing screenings were within normal limits.

Cryopyrin-associated periodic syndromes (CAPS) consist of 3 rare, IL-1–associated, autoinflammatory disorders, including familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), and NOMID (also known as chronic infantile neurologic cutaneous and articular syndrome). These conditions result from a sporadic or autosomal-dominant gain-of-function mutations in a single gene, NLRP3, on chromosome 1q44. NLRP3 encodes for cryopyrin, an important component of an IL-1 and IL-18 activating inflammasome.¹ The most severe manifestation of CAPS is NOMID, which typically presents at birth as a migratory urticarial eruption, growth failure, myalgia, fever, and abnormal facial features, including frontal bossing, saddle-shaped nose, and protruding eyes.² The illness also can manifest with hepatosplenomegaly, lymphadenopathy, uveitis, sensorineural hearing loss, cerebral atrophy, and other neurologic manifestations.³ A diagnosis of chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome was less likely given that our patient remained afebrile and did not show signs of lipodystrophy and persistent violaceous eyelid swelling. Both FCAS and MWS are less severe forms of CAPS when compared to NOMID. Familial cold autoinflammatory syndrome was less likely given the absence of the typical periodic fever pattern associated with the condition and severity of our patient’s symptoms. Muckle-Wells syndrome typically presents in adolescence with symptoms of FCAS, painful urticarial plaques, and progressive sensorinueral hearing loss. Tumor necrosis factor receptor–associated periodic fever (TRAPS) usually is associated with episodic fevers, abdominal pain, periorbital edema, migratory erythema, and arthralgia.^1,3,4

Diagnostic criteria for CAPS include elevated inflammatory markers and serum amyloid, plus at least 2 of the typical CAPS symptoms: urticarial rash, cold-triggered episodes, sensorineural hearing loss, musculoskeletal symptoms, chronic aseptic meningitis, and skeletal abnormalities.⁴ The sensitivity and specificity of these diagnostic criteria are 84% and 91%, respectively. Additional findings that can be seen but are not part of the diagnostic criteria include intermittent fever, transient joint swelling, bony overgrowths, uveitis, optic disc edema, impaired growth, and hepatosplenomegaly.⁵ Laboratory findings may reveal leukocytosis, eosinophilia, anemia, and/or thrombocytopenia.^3,5

Genetic testing, skin biopsies, ophthalmic examinations, neuroimaging, joint radiography, cerebrospinal fluid tests, and hearing examinations can be performed for confirmation of diagnosis and evaluation of systemic complications.⁴ A skin biopsy may reveal a neutrophilic infiltrate. Ophthalmic examination can demonstrate uveitis and optic disk edema. Neuroimaging may reveal cerebral atrophy or ventricular dilation. Lastly, joint radiography can be used to evaluate for the presence of premature long bone ossification or osseous overgrowth.⁴

In summary, NOMID is a multisystemic disorder with cutaneous manifestations. Early recognition of this entity is important given the severe sequelae and available efficacious therapy. Dermatologists should be aware of these manifestations, as dermatologic consultation and a skin biopsy may aid in diagnosis.

A skin care trend, particularly in the Korean beauty product market and now worldwide, cleansing balms are a soft, yet solid variation of an oil-based cleanser. The solid oily component is combined with a surfactant or emulsifier. The cream balm texture melts into more of an oil texture once warmed with fingertips and applied to facial skin. The oils are effective at breaking down or attracting skin care products, oil, and grime on the skin surface. Once warm water is added, the oil emulsifies, and after it is wiped or rinsed off, what’s left behind is clean, hydrated skin.

Unlike surfactant-based liquid cleansers that typically produce foam, cleansing balms are thicker in consistency and do not foam. These products are often packaged in a jar as a thick creamy cleanser or a solid stick. They don’t tend to compromise the moisture barrier or disrupt skin pH, thus, resulting in less dry skin and have less potential to cause irritation. These products are particularly useful during drier, colder months, or in dry climates, and for those who have dry skin or eczema.

The popularity of cleansing balms has largely been based on their ability to remove makeup, similar to an oil cleanser, without the need to necessarily “double cleanse” with a regular cleanser afterward.

Alternatives to remove makeup besides cleansing balms, oil cleansers, and regular liquid water-based cleansers include micellar water (oil in water), chemical makeup removing cloths, and nonchemical makeup removing pads used with water. Micellar water is also gentle on the skin; it requires a cotton pad, tip, or cloth to remove makeup, without the need for water or washing. Both are effective, but it may be easier to remove makeup with cleansing balms, without the need for rubbing dry skin, than with micellar water. A study published in 2020 of 20 individuals reported that waterproof sunscreen was more effectively removed with a cleansing oil than a non–oil-based cleanser, with less irritation and dryness. Both were effective at removing non-waterproof sunscreen.

Both cleansing balms and oil-based cleansers need to be kept at room temperature (not in the refrigerator), since they may separate or solidify at low temperatures.

Jose A. Bernat Bacete/Getty Images

Most cleansing balms can be applied to dry skin, massaged, and rinsed off with warm water, but they are sometimes easier to remove with a wet cloth (typically either cotton or muslin). Many are nonirritating to the eyes, which is important when used to remove eye makeup and mascara on delicate skin. While many cleansing balms are noncomedogenic, residue from balms that are too thick or not rinsed off properly can contribute to comedones or milia. If residue is present after use, then “double-cleansing” with a water-based cleanser is reasonable, but not necessary for most users.

Did the development of Ponds cold cream mark the beginning of this trend? Yes and no. The creation of the first cold cream prototype has been attributed to the Greek physician, Galen (who lived in Rome), a combination of rose water, beeswax, and olive oil in 150 CE. While Ponds also has manufactured a cleansing balm, the original cold cream is a 50% moisturizer in a cleanser. So while similar in containing an oil, water, emulsifier, and thickener, and effective, it is more of a moisturizer and less of a solid oil/balm in its consistency.

Dr. Wesley practices dermatology in Beverly Hills, Calif. Write to her at dermnews@mdedge.com. She had no relevant disclosures.

A skin care trend, particularly in the Korean beauty product market and now worldwide, cleansing balms are a soft, yet solid variation of an oil-based cleanser. The solid oily component is combined with a surfactant or emulsifier. The cream balm texture melts into more of an oil texture once warmed with fingertips and applied to facial skin. The oils are effective at breaking down or attracting skin care products, oil, and grime on the skin surface. Once warm water is added, the oil emulsifies, and after it is wiped or rinsed off, what’s left behind is clean, hydrated skin.

Unlike surfactant-based liquid cleansers that typically produce foam, cleansing balms are thicker in consistency and do not foam. These products are often packaged in a jar as a thick creamy cleanser or a solid stick. They don’t tend to compromise the moisture barrier or disrupt skin pH, thus, resulting in less dry skin and have less potential to cause irritation. These products are particularly useful during drier, colder months, or in dry climates, and for those who have dry skin or eczema.

The popularity of cleansing balms has largely been based on their ability to remove makeup, similar to an oil cleanser, without the need to necessarily “double cleanse” with a regular cleanser afterward.

Alternatives to remove makeup besides cleansing balms, oil cleansers, and regular liquid water-based cleansers include micellar water (oil in water), chemical makeup removing cloths, and nonchemical makeup removing pads used with water. Micellar water is also gentle on the skin; it requires a cotton pad, tip, or cloth to remove makeup, without the need for water or washing. Both are effective, but it may be easier to remove makeup with cleansing balms, without the need for rubbing dry skin, than with micellar water. A study published in 2020 of 20 individuals reported that waterproof sunscreen was more effectively removed with a cleansing oil than a non–oil-based cleanser, with less irritation and dryness. Both were effective at removing non-waterproof sunscreen.

Both cleansing balms and oil-based cleansers need to be kept at room temperature (not in the refrigerator), since they may separate or solidify at low temperatures.

Jose A. Bernat Bacete/Getty Images

Most cleansing balms can be applied to dry skin, massaged, and rinsed off with warm water, but they are sometimes easier to remove with a wet cloth (typically either cotton or muslin). Many are nonirritating to the eyes, which is important when used to remove eye makeup and mascara on delicate skin. While many cleansing balms are noncomedogenic, residue from balms that are too thick or not rinsed off properly can contribute to comedones or milia. If residue is present after use, then “double-cleansing” with a water-based cleanser is reasonable, but not necessary for most users.

Did the development of Ponds cold cream mark the beginning of this trend? Yes and no. The creation of the first cold cream prototype has been attributed to the Greek physician, Galen (who lived in Rome), a combination of rose water, beeswax, and olive oil in 150 CE. While Ponds also has manufactured a cleansing balm, the original cold cream is a 50% moisturizer in a cleanser. So while similar in containing an oil, water, emulsifier, and thickener, and effective, it is more of a moisturizer and less of a solid oil/balm in its consistency.

Dr. Wesley practices dermatology in Beverly Hills, Calif. Write to her at dermnews@mdedge.com. She had no relevant disclosures.

A skin care trend, particularly in the Korean beauty product market and now worldwide, cleansing balms are a soft, yet solid variation of an oil-based cleanser. The solid oily component is combined with a surfactant or emulsifier. The cream balm texture melts into more of an oil texture once warmed with fingertips and applied to facial skin. The oils are effective at breaking down or attracting skin care products, oil, and grime on the skin surface. Once warm water is added, the oil emulsifies, and after it is wiped or rinsed off, what’s left behind is clean, hydrated skin.

Unlike surfactant-based liquid cleansers that typically produce foam, cleansing balms are thicker in consistency and do not foam. These products are often packaged in a jar as a thick creamy cleanser or a solid stick. They don’t tend to compromise the moisture barrier or disrupt skin pH, thus, resulting in less dry skin and have less potential to cause irritation. These products are particularly useful during drier, colder months, or in dry climates, and for those who have dry skin or eczema.

The popularity of cleansing balms has largely been based on their ability to remove makeup, similar to an oil cleanser, without the need to necessarily “double cleanse” with a regular cleanser afterward.

Alternatives to remove makeup besides cleansing balms, oil cleansers, and regular liquid water-based cleansers include micellar water (oil in water), chemical makeup removing cloths, and nonchemical makeup removing pads used with water. Micellar water is also gentle on the skin; it requires a cotton pad, tip, or cloth to remove makeup, without the need for water or washing. Both are effective, but it may be easier to remove makeup with cleansing balms, without the need for rubbing dry skin, than with micellar water. A study published in 2020 of 20 individuals reported that waterproof sunscreen was more effectively removed with a cleansing oil than a non–oil-based cleanser, with less irritation and dryness. Both were effective at removing non-waterproof sunscreen.

Both cleansing balms and oil-based cleansers need to be kept at room temperature (not in the refrigerator), since they may separate or solidify at low temperatures.

Jose A. Bernat Bacete/Getty Images

Most cleansing balms can be applied to dry skin, massaged, and rinsed off with warm water, but they are sometimes easier to remove with a wet cloth (typically either cotton or muslin). Many are nonirritating to the eyes, which is important when used to remove eye makeup and mascara on delicate skin. While many cleansing balms are noncomedogenic, residue from balms that are too thick or not rinsed off properly can contribute to comedones or milia. If residue is present after use, then “double-cleansing” with a water-based cleanser is reasonable, but not necessary for most users.

Did the development of Ponds cold cream mark the beginning of this trend? Yes and no. The creation of the first cold cream prototype has been attributed to the Greek physician, Galen (who lived in Rome), a combination of rose water, beeswax, and olive oil in 150 CE. While Ponds also has manufactured a cleansing balm, the original cold cream is a 50% moisturizer in a cleanser. So while similar in containing an oil, water, emulsifier, and thickener, and effective, it is more of a moisturizer and less of a solid oil/balm in its consistency.

Dr. Wesley practices dermatology in Beverly Hills, Calif. Write to her at dermnews@mdedge.com. She had no relevant disclosures.

Although the past 30 years have stirred up a whirlwind of neurological research that has dramatically expanded therapeutic options for patients with epilepsy, historical pioneers in the field might be disappointed at the fact that treatment response has remained stubbornly stagnant. “Plus ça change, plus c’est la même chose,” they might say: The more things change, the more they stay the same. In fact, since 1993, despite an explosion of third-generation drugs, an abundance of new surgical approaches, and a whole new category of treatment in the form of neurostimulation devices, response rates in epilepsy have not budged, with roughly two-thirds of patients achieving seizure freedom and a third still struggling with treatment resistance.

But if you widen the lens and look towards the horizon, things are “on the cusp and going like a rocket,” said Jacqueline A. French, MD, professor of neurology in the Comprehensive Epilepsy Center at NYU Langone Health, New York. While treatment response rates may be stuck, adverse effects of those treatments have plummeted, and even treatment-resistant patients dealing with residual seizures live a much freer life with far fewer and less serious episodes.
 

Simpler times

In the late 1980s, just as Dr. French was finishing her second epilepsy fellowship at Yale, it was “almost laughable that things were so simple,” she recalls. “There were a few major centers that were doing epilepsy surgery … and in the world of medication, there were just five major drugs: phenobarbital, primidone, carbamazepine, phenytoin, and valproate.” That all changed as she was settling in to her first academic position at the University of Pennsylvania, with the “explosive” introduction of felbamate, a new antiseizure drug whose precipitous rise and fall from favor cast a sobering shadow which set the course for future drug development in the field.

“The felbamate story has a lot to do with what came after, but it was a drug that was much more advantageous in regards to a lot of the things that we didn’t like about antiseizure medicines or antiepileptic drugs as we called them at that time,” she said. The older drugs affected the cerebellum, making people sleepy and unable to concentrate. They also came with the risk of serious adverse effects such as hepatic enzyme induction and teratogenicity. Not only was felbamate nonsedating, “it actually was a little bit alerting,” said Dr. French. “People felt so different and so great on it, and it was effective for some seizure types that we didn’t really have good drugs for.” Very quickly, felbamate became a first-line therapy. Within its first year on the market, 150,000 newly diagnosed patients were started on it, “which is unthinkable now,” she said.

Sure enough, it all came crashing down a year later, on Aug. 1, 1994, when the drug was urgently withdrawn by the U.S. Food and Drug Administration after being linked to the development of aplastic anemia. “There was a day that anybody who was there at the time will remember when we all got the news, that everybody had to be taken off the drug,” Dr. French recalled. “We spent the weekend in the chart room, looking chart by chart by chart, for who was on felbamate.”

Until then, Dr. French had been straddling the line between her interests in pharmacologic versus surgical treatments for epilepsy. In fact, during her second epilepsy fellowship, which was dedicated to surgery, she published “Characteristics of medial temporal lobe epilepsy” in Annals of Neurology, one of the most-cited papers of her career. “Epilepsy from the temporal lobe is the biggest and best shot on goal when you’re talking about sending somebody to epilepsy surgery and rendering them completely seizure free,” she said. “Early in my career at the University of Pennsylvania, it was all about identifying those patients. And you know, there is nothing more gratifying than taking somebody whose life has been devastated by frequent seizures, who is injuring themselves and not able to be independent, and doing a surgery, which is very safe, and then all the seizures are gone – which is probably why I was so excited by surgery at the time.”

For a while, in the early 1990s, temporal lobectomy eclipsed many of the other avenues in epilepsy treatment, but it too has given way to a much wider variety of more complex techniques, which may be less curative but more palliative.
 

More drug options

Meanwhile, the felbamate story had ignited debate in the field about safer drug development – pushing Dr. French into establishing what was then known as the Antiepileptic Drug Trials conference, later renamed the Epilepsy Therapies & Diagnostics Development Symposium – a forum that encouraged safer, but also swifter movement of drugs through the pipeline and onto the market. “After felbamate, came gabapentin, and then came to topiramate and lamotrigine, and very quickly there were many, many, many choices,” she explained. “But once stung, twice shy. Felbamate really gave us a new perspective on which patients we put on the new drugs. Now we have a process of starting them in people with treatment-resistant epilepsy first. The risk-benefit equation is more reasonable because they have lots of risks. And then we work our way back to people with newly diagnosed epilepsy.”

Disease-modifying therapies

Today, the medications used to treat epilepsy are referred to as antiseizure rather than antiepileptic drugs because they simply suppress seizure symptoms and do not address the cause. But the rocket that Dr. French is watching gain speed and momentum is the disease-modifying gene therapies – true antiepileptics that may significantly move the needle on the number and type of patients who can reach seizure freedom. “We spent the last 25 years not even thinking we would ever have antiepileptic therapies, and now in the last 5 years or so, we were pretty sure we will,” she said. “We have gene therapies that can intervene now – none yet that have actually reached approval, these are all currently in trials – but we certainly have high expectations that they will very soon be available.”

Improving patients’ lives

While gene therapy rockets ahead, new device developments are already improving life for patients, even despite ongoing seizures. A drug-delivering pump is still in trials, but could make a big difference to daily medication adherence, and wearable or implantable devices are being developed to track seizures. More accurate tracking has also revealed that many people’s seizures are actually quite predictable, with regular cycles allowing for the possibility of prophylactic medication when increased seizure activity is expected.

Despite 30 years of no change in the proportion of epilepsy patients experiencing treatment resistance, Dr. French said that drugs, devices, and surgeries have improved the lives of all patients – both treatment resistant and treatment sensitive. “The difference between almost seizure free and completely seizure free is a big one because it means you can’t drive, you may have difficulty with your employment, but being able to take a pill every day and feel otherwise completely normal? We’ve come a long way.”

Although the past 30 years have stirred up a whirlwind of neurological research that has dramatically expanded therapeutic options for patients with epilepsy, historical pioneers in the field might be disappointed at the fact that treatment response has remained stubbornly stagnant. “Plus ça change, plus c’est la même chose,” they might say: The more things change, the more they stay the same. In fact, since 1993, despite an explosion of third-generation drugs, an abundance of new surgical approaches, and a whole new category of treatment in the form of neurostimulation devices, response rates in epilepsy have not budged, with roughly two-thirds of patients achieving seizure freedom and a third still struggling with treatment resistance.

But if you widen the lens and look towards the horizon, things are “on the cusp and going like a rocket,” said Jacqueline A. French, MD, professor of neurology in the Comprehensive Epilepsy Center at NYU Langone Health, New York. While treatment response rates may be stuck, adverse effects of those treatments have plummeted, and even treatment-resistant patients dealing with residual seizures live a much freer life with far fewer and less serious episodes.
 

Simpler times

In the late 1980s, just as Dr. French was finishing her second epilepsy fellowship at Yale, it was “almost laughable that things were so simple,” she recalls. “There were a few major centers that were doing epilepsy surgery … and in the world of medication, there were just five major drugs: phenobarbital, primidone, carbamazepine, phenytoin, and valproate.” That all changed as she was settling in to her first academic position at the University of Pennsylvania, with the “explosive” introduction of felbamate, a new antiseizure drug whose precipitous rise and fall from favor cast a sobering shadow which set the course for future drug development in the field.

“The felbamate story has a lot to do with what came after, but it was a drug that was much more advantageous in regards to a lot of the things that we didn’t like about antiseizure medicines or antiepileptic drugs as we called them at that time,” she said. The older drugs affected the cerebellum, making people sleepy and unable to concentrate. They also came with the risk of serious adverse effects such as hepatic enzyme induction and teratogenicity. Not only was felbamate nonsedating, “it actually was a little bit alerting,” said Dr. French. “People felt so different and so great on it, and it was effective for some seizure types that we didn’t really have good drugs for.” Very quickly, felbamate became a first-line therapy. Within its first year on the market, 150,000 newly diagnosed patients were started on it, “which is unthinkable now,” she said.

Sure enough, it all came crashing down a year later, on Aug. 1, 1994, when the drug was urgently withdrawn by the U.S. Food and Drug Administration after being linked to the development of aplastic anemia. “There was a day that anybody who was there at the time will remember when we all got the news, that everybody had to be taken off the drug,” Dr. French recalled. “We spent the weekend in the chart room, looking chart by chart by chart, for who was on felbamate.”

Until then, Dr. French had been straddling the line between her interests in pharmacologic versus surgical treatments for epilepsy. In fact, during her second epilepsy fellowship, which was dedicated to surgery, she published “Characteristics of medial temporal lobe epilepsy” in Annals of Neurology, one of the most-cited papers of her career. “Epilepsy from the temporal lobe is the biggest and best shot on goal when you’re talking about sending somebody to epilepsy surgery and rendering them completely seizure free,” she said. “Early in my career at the University of Pennsylvania, it was all about identifying those patients. And you know, there is nothing more gratifying than taking somebody whose life has been devastated by frequent seizures, who is injuring themselves and not able to be independent, and doing a surgery, which is very safe, and then all the seizures are gone – which is probably why I was so excited by surgery at the time.”

For a while, in the early 1990s, temporal lobectomy eclipsed many of the other avenues in epilepsy treatment, but it too has given way to a much wider variety of more complex techniques, which may be less curative but more palliative.
 

More drug options

Meanwhile, the felbamate story had ignited debate in the field about safer drug development – pushing Dr. French into establishing what was then known as the Antiepileptic Drug Trials conference, later renamed the Epilepsy Therapies & Diagnostics Development Symposium – a forum that encouraged safer, but also swifter movement of drugs through the pipeline and onto the market. “After felbamate, came gabapentin, and then came to topiramate and lamotrigine, and very quickly there were many, many, many choices,” she explained. “But once stung, twice shy. Felbamate really gave us a new perspective on which patients we put on the new drugs. Now we have a process of starting them in people with treatment-resistant epilepsy first. The risk-benefit equation is more reasonable because they have lots of risks. And then we work our way back to people with newly diagnosed epilepsy.”

Disease-modifying therapies

Today, the medications used to treat epilepsy are referred to as antiseizure rather than antiepileptic drugs because they simply suppress seizure symptoms and do not address the cause. But the rocket that Dr. French is watching gain speed and momentum is the disease-modifying gene therapies – true antiepileptics that may significantly move the needle on the number and type of patients who can reach seizure freedom. “We spent the last 25 years not even thinking we would ever have antiepileptic therapies, and now in the last 5 years or so, we were pretty sure we will,” she said. “We have gene therapies that can intervene now – none yet that have actually reached approval, these are all currently in trials – but we certainly have high expectations that they will very soon be available.”

Improving patients’ lives

While gene therapy rockets ahead, new device developments are already improving life for patients, even despite ongoing seizures. A drug-delivering pump is still in trials, but could make a big difference to daily medication adherence, and wearable or implantable devices are being developed to track seizures. More accurate tracking has also revealed that many people’s seizures are actually quite predictable, with regular cycles allowing for the possibility of prophylactic medication when increased seizure activity is expected.

Despite 30 years of no change in the proportion of epilepsy patients experiencing treatment resistance, Dr. French said that drugs, devices, and surgeries have improved the lives of all patients – both treatment resistant and treatment sensitive. “The difference between almost seizure free and completely seizure free is a big one because it means you can’t drive, you may have difficulty with your employment, but being able to take a pill every day and feel otherwise completely normal? We’ve come a long way.”

Although the past 30 years have stirred up a whirlwind of neurological research that has dramatically expanded therapeutic options for patients with epilepsy, historical pioneers in the field might be disappointed at the fact that treatment response has remained stubbornly stagnant. “Plus ça change, plus c’est la même chose,” they might say: The more things change, the more they stay the same. In fact, since 1993, despite an explosion of third-generation drugs, an abundance of new surgical approaches, and a whole new category of treatment in the form of neurostimulation devices, response rates in epilepsy have not budged, with roughly two-thirds of patients achieving seizure freedom and a third still struggling with treatment resistance.

But if you widen the lens and look towards the horizon, things are “on the cusp and going like a rocket,” said Jacqueline A. French, MD, professor of neurology in the Comprehensive Epilepsy Center at NYU Langone Health, New York. While treatment response rates may be stuck, adverse effects of those treatments have plummeted, and even treatment-resistant patients dealing with residual seizures live a much freer life with far fewer and less serious episodes.
 

Simpler times

In the late 1980s, just as Dr. French was finishing her second epilepsy fellowship at Yale, it was “almost laughable that things were so simple,” she recalls. “There were a few major centers that were doing epilepsy surgery … and in the world of medication, there were just five major drugs: phenobarbital, primidone, carbamazepine, phenytoin, and valproate.” That all changed as she was settling in to her first academic position at the University of Pennsylvania, with the “explosive” introduction of felbamate, a new antiseizure drug whose precipitous rise and fall from favor cast a sobering shadow which set the course for future drug development in the field.

“The felbamate story has a lot to do with what came after, but it was a drug that was much more advantageous in regards to a lot of the things that we didn’t like about antiseizure medicines or antiepileptic drugs as we called them at that time,” she said. The older drugs affected the cerebellum, making people sleepy and unable to concentrate. They also came with the risk of serious adverse effects such as hepatic enzyme induction and teratogenicity. Not only was felbamate nonsedating, “it actually was a little bit alerting,” said Dr. French. “People felt so different and so great on it, and it was effective for some seizure types that we didn’t really have good drugs for.” Very quickly, felbamate became a first-line therapy. Within its first year on the market, 150,000 newly diagnosed patients were started on it, “which is unthinkable now,” she said.

Sure enough, it all came crashing down a year later, on Aug. 1, 1994, when the drug was urgently withdrawn by the U.S. Food and Drug Administration after being linked to the development of aplastic anemia. “There was a day that anybody who was there at the time will remember when we all got the news, that everybody had to be taken off the drug,” Dr. French recalled. “We spent the weekend in the chart room, looking chart by chart by chart, for who was on felbamate.”

Until then, Dr. French had been straddling the line between her interests in pharmacologic versus surgical treatments for epilepsy. In fact, during her second epilepsy fellowship, which was dedicated to surgery, she published “Characteristics of medial temporal lobe epilepsy” in Annals of Neurology, one of the most-cited papers of her career. “Epilepsy from the temporal lobe is the biggest and best shot on goal when you’re talking about sending somebody to epilepsy surgery and rendering them completely seizure free,” she said. “Early in my career at the University of Pennsylvania, it was all about identifying those patients. And you know, there is nothing more gratifying than taking somebody whose life has been devastated by frequent seizures, who is injuring themselves and not able to be independent, and doing a surgery, which is very safe, and then all the seizures are gone – which is probably why I was so excited by surgery at the time.”

For a while, in the early 1990s, temporal lobectomy eclipsed many of the other avenues in epilepsy treatment, but it too has given way to a much wider variety of more complex techniques, which may be less curative but more palliative.
 

More drug options

Meanwhile, the felbamate story had ignited debate in the field about safer drug development – pushing Dr. French into establishing what was then known as the Antiepileptic Drug Trials conference, later renamed the Epilepsy Therapies & Diagnostics Development Symposium – a forum that encouraged safer, but also swifter movement of drugs through the pipeline and onto the market. “After felbamate, came gabapentin, and then came to topiramate and lamotrigine, and very quickly there were many, many, many choices,” she explained. “But once stung, twice shy. Felbamate really gave us a new perspective on which patients we put on the new drugs. Now we have a process of starting them in people with treatment-resistant epilepsy first. The risk-benefit equation is more reasonable because they have lots of risks. And then we work our way back to people with newly diagnosed epilepsy.”

Disease-modifying therapies

Today, the medications used to treat epilepsy are referred to as antiseizure rather than antiepileptic drugs because they simply suppress seizure symptoms and do not address the cause. But the rocket that Dr. French is watching gain speed and momentum is the disease-modifying gene therapies – true antiepileptics that may significantly move the needle on the number and type of patients who can reach seizure freedom. “We spent the last 25 years not even thinking we would ever have antiepileptic therapies, and now in the last 5 years or so, we were pretty sure we will,” she said. “We have gene therapies that can intervene now – none yet that have actually reached approval, these are all currently in trials – but we certainly have high expectations that they will very soon be available.”

Improving patients’ lives

While gene therapy rockets ahead, new device developments are already improving life for patients, even despite ongoing seizures. A drug-delivering pump is still in trials, but could make a big difference to daily medication adherence, and wearable or implantable devices are being developed to track seizures. More accurate tracking has also revealed that many people’s seizures are actually quite predictable, with regular cycles allowing for the possibility of prophylactic medication when increased seizure activity is expected.

Despite 30 years of no change in the proportion of epilepsy patients experiencing treatment resistance, Dr. French said that drugs, devices, and surgeries have improved the lives of all patients – both treatment resistant and treatment sensitive. “The difference between almost seizure free and completely seizure free is a big one because it means you can’t drive, you may have difficulty with your employment, but being able to take a pill every day and feel otherwise completely normal? We’ve come a long way.”

“Papa! Where donut?” asks my 2½ year-old sitting with her legs dangling and hands folded in a bustling Starbucks. We’ve been waiting for 8 minutes and we’ve reached her limit of tolerance. She’s unimpressed by the queued customers who compliment her curly blonde hair, many of whom have come and gone since we’ve been waiting. I agree – how long does it take to pour a kiddie milk and grab a donut? We can both see it in the case right there!

No one likes to wait. Truly, one of the great benefits of the modern world is that wait times are now incredibly short. Many Starbucks customers, unlike my daughter, ordered their drink ahead and waited exactly 0 minutes to get their drink. What about Amazon? I ordered a bird feeder this morning and it’s already hanging in the yard. It’s still daylight. Feel like Himalayan Momo Dumplings tonight? Your food could arrive in 37 minutes. The modern wait standard has been set impossibly high for us.

Yes, medicine is no doubt at the top of the list of “Worst Wait Times.” We make patients wait for appointments (sometimes months), wait to be seen, wait for biopsy results, wait for follow-up surgery, wait for those second results, even wait for PET scans and treatment plans for some. We created a whole room just for waiting. Airlines call theirs “The Platinum Executive Lounge.” Ours is “The waiting room.”

Excess waiting is a significant reason why health care gets beat up in reviews. We’re unable to keep up with the new expectations. Waiting is also a significant cause of distress. Many patients report the most difficult part of their cancer diagnosis was the waiting for results, not the treatment. It’s because when under stress, we are hardwired to take action. Binding patients into inaction while they wait is very uncomfortable.

Fortunately, the psychology of waiting is well understood and there are best practices that can help. First, anxiety makes waiting much worse. Conveying confidence and reassuring patients they are in the right place and that everything will be OK makes the wait time feel shorter for them. Uncertainty also compounds their apprehension. If you believe the diagnosis will be melanoma, tell them that at the time of the biopsy and tell them what you expect next. This is better than saying, “Well, that could be cancer. We’ll see.”

Knowing a wait time is also much better than not. Have your staff advise patients on how much longer they can expect before seeing you (telling them they’re next isn’t as effective). Advise that test results should be back by the end of next week. Of course, under promise and over deliver. When the results are back on Tuesday, you’ve got a pleased patient.

Explaining that you had to add in an urgent patient helps. Even if it’s not your fault, it’s still better to apologize. For example, the 78 highway, the left anterior descending artery to our office, has been closed because of a sinkhole this month (not kidding). I’ve been apologizing to a lot of patients saying that all our patients are arriving late, which is putting us behind. As they can envision the linear parking lot that used to be a highway, it helps.

Lastly, as any child can tell you, waiting has to not only be, but to also appear, fair. The only thing worse than waiting for an appointment, or donut, is seeing someone who came in after you get their donut before you do. If you’re pulling both Mohs and cosmetics patients from the same waiting area, then your surgery patients will see a lot of patients come and go while they are sitting. Demarcating one sitting area for Mohs and one for clinics might help. So does ordering ahead. I’d show my daughter how to use the app so we don’t have to wait so long next week, but she’s 2 and I’m quite sure she already knows.

Dr. Benabio is director of Healthcare Transformation and chief of dermatology at Kaiser Permanente San Diego. The opinions expressed in this column are his own and do not represent those of Kaiser Permanente. Dr. Benabio is @Dermdoc on Twitter. Write to him at dermnews@mdedge.com.

“Papa! Where donut?” asks my 2½ year-old sitting with her legs dangling and hands folded in a bustling Starbucks. We’ve been waiting for 8 minutes and we’ve reached her limit of tolerance. She’s unimpressed by the queued customers who compliment her curly blonde hair, many of whom have come and gone since we’ve been waiting. I agree – how long does it take to pour a kiddie milk and grab a donut? We can both see it in the case right there!

No one likes to wait. Truly, one of the great benefits of the modern world is that wait times are now incredibly short. Many Starbucks customers, unlike my daughter, ordered their drink ahead and waited exactly 0 minutes to get their drink. What about Amazon? I ordered a bird feeder this morning and it’s already hanging in the yard. It’s still daylight. Feel like Himalayan Momo Dumplings tonight? Your food could arrive in 37 minutes. The modern wait standard has been set impossibly high for us.

Yes, medicine is no doubt at the top of the list of “Worst Wait Times.” We make patients wait for appointments (sometimes months), wait to be seen, wait for biopsy results, wait for follow-up surgery, wait for those second results, even wait for PET scans and treatment plans for some. We created a whole room just for waiting. Airlines call theirs “The Platinum Executive Lounge.” Ours is “The waiting room.”

Excess waiting is a significant reason why health care gets beat up in reviews. We’re unable to keep up with the new expectations. Waiting is also a significant cause of distress. Many patients report the most difficult part of their cancer diagnosis was the waiting for results, not the treatment. It’s because when under stress, we are hardwired to take action. Binding patients into inaction while they wait is very uncomfortable.

Fortunately, the psychology of waiting is well understood and there are best practices that can help. First, anxiety makes waiting much worse. Conveying confidence and reassuring patients they are in the right place and that everything will be OK makes the wait time feel shorter for them. Uncertainty also compounds their apprehension. If you believe the diagnosis will be melanoma, tell them that at the time of the biopsy and tell them what you expect next. This is better than saying, “Well, that could be cancer. We’ll see.”

Knowing a wait time is also much better than not. Have your staff advise patients on how much longer they can expect before seeing you (telling them they’re next isn’t as effective). Advise that test results should be back by the end of next week. Of course, under promise and over deliver. When the results are back on Tuesday, you’ve got a pleased patient.

Explaining that you had to add in an urgent patient helps. Even if it’s not your fault, it’s still better to apologize. For example, the 78 highway, the left anterior descending artery to our office, has been closed because of a sinkhole this month (not kidding). I’ve been apologizing to a lot of patients saying that all our patients are arriving late, which is putting us behind. As they can envision the linear parking lot that used to be a highway, it helps.

Lastly, as any child can tell you, waiting has to not only be, but to also appear, fair. The only thing worse than waiting for an appointment, or donut, is seeing someone who came in after you get their donut before you do. If you’re pulling both Mohs and cosmetics patients from the same waiting area, then your surgery patients will see a lot of patients come and go while they are sitting. Demarcating one sitting area for Mohs and one for clinics might help. So does ordering ahead. I’d show my daughter how to use the app so we don’t have to wait so long next week, but she’s 2 and I’m quite sure she already knows.

Dr. Benabio is director of Healthcare Transformation and chief of dermatology at Kaiser Permanente San Diego. The opinions expressed in this column are his own and do not represent those of Kaiser Permanente. Dr. Benabio is @Dermdoc on Twitter. Write to him at dermnews@mdedge.com.

“Papa! Where donut?” asks my 2½ year-old sitting with her legs dangling and hands folded in a bustling Starbucks. We’ve been waiting for 8 minutes and we’ve reached her limit of tolerance. She’s unimpressed by the queued customers who compliment her curly blonde hair, many of whom have come and gone since we’ve been waiting. I agree – how long does it take to pour a kiddie milk and grab a donut? We can both see it in the case right there!

No one likes to wait. Truly, one of the great benefits of the modern world is that wait times are now incredibly short. Many Starbucks customers, unlike my daughter, ordered their drink ahead and waited exactly 0 minutes to get their drink. What about Amazon? I ordered a bird feeder this morning and it’s already hanging in the yard. It’s still daylight. Feel like Himalayan Momo Dumplings tonight? Your food could arrive in 37 minutes. The modern wait standard has been set impossibly high for us.

Yes, medicine is no doubt at the top of the list of “Worst Wait Times.” We make patients wait for appointments (sometimes months), wait to be seen, wait for biopsy results, wait for follow-up surgery, wait for those second results, even wait for PET scans and treatment plans for some. We created a whole room just for waiting. Airlines call theirs “The Platinum Executive Lounge.” Ours is “The waiting room.”

Excess waiting is a significant reason why health care gets beat up in reviews. We’re unable to keep up with the new expectations. Waiting is also a significant cause of distress. Many patients report the most difficult part of their cancer diagnosis was the waiting for results, not the treatment. It’s because when under stress, we are hardwired to take action. Binding patients into inaction while they wait is very uncomfortable.

Fortunately, the psychology of waiting is well understood and there are best practices that can help. First, anxiety makes waiting much worse. Conveying confidence and reassuring patients they are in the right place and that everything will be OK makes the wait time feel shorter for them. Uncertainty also compounds their apprehension. If you believe the diagnosis will be melanoma, tell them that at the time of the biopsy and tell them what you expect next. This is better than saying, “Well, that could be cancer. We’ll see.”

Knowing a wait time is also much better than not. Have your staff advise patients on how much longer they can expect before seeing you (telling them they’re next isn’t as effective). Advise that test results should be back by the end of next week. Of course, under promise and over deliver. When the results are back on Tuesday, you’ve got a pleased patient.

Explaining that you had to add in an urgent patient helps. Even if it’s not your fault, it’s still better to apologize. For example, the 78 highway, the left anterior descending artery to our office, has been closed because of a sinkhole this month (not kidding). I’ve been apologizing to a lot of patients saying that all our patients are arriving late, which is putting us behind. As they can envision the linear parking lot that used to be a highway, it helps.

Lastly, as any child can tell you, waiting has to not only be, but to also appear, fair. The only thing worse than waiting for an appointment, or donut, is seeing someone who came in after you get their donut before you do. If you’re pulling both Mohs and cosmetics patients from the same waiting area, then your surgery patients will see a lot of patients come and go while they are sitting. Demarcating one sitting area for Mohs and one for clinics might help. So does ordering ahead. I’d show my daughter how to use the app so we don’t have to wait so long next week, but she’s 2 and I’m quite sure she already knows.

Dr. Benabio is director of Healthcare Transformation and chief of dermatology at Kaiser Permanente San Diego. The opinions expressed in this column are his own and do not represent those of Kaiser Permanente. Dr. Benabio is @Dermdoc on Twitter. Write to him at dermnews@mdedge.com.