Quiz

ANSWER
The correct answer is nevus sebaceous (choice “b”), a rather common hamartomatous congenital tumor. The diagnosis was confirmed by pathologic examination of a tiny sample from the most papular portion of the lesion.

Given the lesion’s congenital nature and complete lack of response to treatment, wart (choice “a”) was quite unlikely. And although trichofolliculoma (choice “c”) and epidermal nevus (choice “d”) were possible differential items, the former usually appears much later in life and the latter is usually more dry and rough to the touch.

DISCUSSION
Nevus sebaceous (NS) of Jadassohn was first described in 1895 by a Swedish dermatologist who had seen a series of young patients with hairless plaques in the scalp or on surrounding neck or facial skin. Pathologic exam confirmed them to be organoid nevi representing an overgrowth of sebaceous glands.

Over time, it became clear that NS affects all genders and races equally. For most patients, the lesions are of cosmetic concern due to the lack of hair. But it has also been established that NS can develop in areas, including the face, ears, and neck, on which they may be cosmetically significant and difficult to remove.

Concern arose when reports began to surface that NS could undergo malignant transformation, especially in larger scalp lesions that are subject to years of excess UV exposure. This was the driving force behind the common practice of removing NS at puberty. We now know that although basal or squamous cell carcinoma, or even melanoma, can develop in longstanding NS, the frequency is probably far less than previously thought.

Most cases of NS in the scalp are easy to diagnose by their pink color, plaquish morphology, and mammillated hairless surface (coupled with congenital manifestation). But a few, such as this patient’s ear lesion, require biopsy for confirmation. As this patient ages, he may feel the need to have the rest of it surgically removed.

ANSWER
The correct answer is nevus sebaceous (choice “b”), a rather common hamartomatous congenital tumor. The diagnosis was confirmed by pathologic examination of a tiny sample from the most papular portion of the lesion.

Given the lesion’s congenital nature and complete lack of response to treatment, wart (choice “a”) was quite unlikely. And although trichofolliculoma (choice “c”) and epidermal nevus (choice “d”) were possible differential items, the former usually appears much later in life and the latter is usually more dry and rough to the touch.

DISCUSSION
Nevus sebaceous (NS) of Jadassohn was first described in 1895 by a Swedish dermatologist who had seen a series of young patients with hairless plaques in the scalp or on surrounding neck or facial skin. Pathologic exam confirmed them to be organoid nevi representing an overgrowth of sebaceous glands.

Over time, it became clear that NS affects all genders and races equally. For most patients, the lesions are of cosmetic concern due to the lack of hair. But it has also been established that NS can develop in areas, including the face, ears, and neck, on which they may be cosmetically significant and difficult to remove.

Concern arose when reports began to surface that NS could undergo malignant transformation, especially in larger scalp lesions that are subject to years of excess UV exposure. This was the driving force behind the common practice of removing NS at puberty. We now know that although basal or squamous cell carcinoma, or even melanoma, can develop in longstanding NS, the frequency is probably far less than previously thought.

Most cases of NS in the scalp are easy to diagnose by their pink color, plaquish morphology, and mammillated hairless surface (coupled with congenital manifestation). But a few, such as this patient’s ear lesion, require biopsy for confirmation. As this patient ages, he may feel the need to have the rest of it surgically removed.

ANSWER
The correct answer is nevus sebaceous (choice “b”), a rather common hamartomatous congenital tumor. The diagnosis was confirmed by pathologic examination of a tiny sample from the most papular portion of the lesion.

Given the lesion’s congenital nature and complete lack of response to treatment, wart (choice “a”) was quite unlikely. And although trichofolliculoma (choice “c”) and epidermal nevus (choice “d”) were possible differential items, the former usually appears much later in life and the latter is usually more dry and rough to the touch.

DISCUSSION
Nevus sebaceous (NS) of Jadassohn was first described in 1895 by a Swedish dermatologist who had seen a series of young patients with hairless plaques in the scalp or on surrounding neck or facial skin. Pathologic exam confirmed them to be organoid nevi representing an overgrowth of sebaceous glands.

Over time, it became clear that NS affects all genders and races equally. For most patients, the lesions are of cosmetic concern due to the lack of hair. But it has also been established that NS can develop in areas, including the face, ears, and neck, on which they may be cosmetically significant and difficult to remove.

Concern arose when reports began to surface that NS could undergo malignant transformation, especially in larger scalp lesions that are subject to years of excess UV exposure. This was the driving force behind the common practice of removing NS at puberty. We now know that although basal or squamous cell carcinoma, or even melanoma, can develop in longstanding NS, the frequency is probably far less than previously thought.

Most cases of NS in the scalp are easy to diagnose by their pink color, plaquish morphology, and mammillated hairless surface (coupled with congenital manifestation). But a few, such as this patient’s ear lesion, require biopsy for confirmation. As this patient ages, he may feel the need to have the rest of it surgically removed.

The Diagnosis: Primary Cutaneous Carcinosarcoma

A generous shave biopsy and debulking performed on the initial visit revealed an infiltrating tumor consisting of malignant epithelial and stromal components (Figure). The basaloid and squamoid epithelial cells were keratin positive. The stromal cells demonstrated positivity for CD10 but were keratin negative. The epithelial portion of the tumor was composed mostly of basaloid islands of cells with nuclear pleomorphism, scattered mitoses, and focal sebaceous differentiation. The mesenchymal portion of the tumor displayed florid pleomorphism and polymorphism, with many large atypical cells and proliferation. A diagnosis of primary cutaneous carcinosarcoma (PCC) was rendered. Head and neck computed tomography showed tumor penetration of less than 1 cm into scalp soft tissues with no involvement of the underlying bone. There was some evidence of swelling of the supragaleal soft tissues without indication of perineural spread. An 11-mm hyperlucent lower cervical lymph node on the left side that likely represented an incidental finding was noted. Surgical excision with margin evaluation was recommended, but the patient declined. He instead received radiation therapy to the left side of the posterior scalp with a total dose of 30 Gy at 6 Gy per fraction and 1 fraction daily. The patient was found to have a well-healed scar with no evidence of recurrence at 4-week follow-up and again at 5 months after radiation therapy.

A generous shave biopsy and debulking performed on the initial visit revealed an inflitrating tumor consisting on malignant epithelial and stromal components (A-C)(H&E; original magnifications ×10, ×20, and ×40, respectively).

Primary cutaneous carcinosarcoma is a rare biphasic neoplasm of unknown etiology that is characterized by the presence of both malignant epithelial and mesenchymal components.¹ Carcinosarcomas have been reported in both the male and female reproductive tracts, urinary tract, gastrointestinal tract, lungs, breasts, larynx, thymus, and thyroid but is uncommon as a primary neoplasm of the skin.² Epidermal PCC occurs with greater frequency in males than in females and typically presents in the eighth or ninth decades of life.³ These tumors tend to arise in sun-exposed regions, most commonly on the face and scalp.²

Morphologically, PCCs typically are exophytic growths that often feature surface ulceration and may or may not bleed upon palpation.⁴ Primary cutaneous carcinosarcomas may present as long-standing lesions that have undergone rapid transformation in the weeks preceding presentation.⁴ It is not uncommon for PCC lesions to carry the clinical diagnosis of squamous cell carcinoma, which suggests notable morphologic overlap between these entities. Histopathologically, PCC shows a basal cell carcinoma and/or a squamous cell carcinoma epithelial component intimately admixed with a sarcomatous component.⁵ The mesenchymal component of PCC typically resembles a superficial malignant fibrous histiocytoma characterized by pleomorphic nuclei and cytoplasm, necrosis, and an increased number of mitotic figures.² Immunohistochemistry can be beneficial in the diagnosis of PCC. A combination of p63 and AE1/AE3 stains can be used to confirm cells of epithelial origin. Staining with vimentin, CD10, or caldesmon can help to delineate the mesenchymal component of PCC.

Epidermal PCC most commonly affects elderly individuals with a history of extensive sun exposure. It has been suggested that p53 mutations due to UV damage are key in tumor formation for both epithelial and mesenchymal elements.⁵ Literature supports a monoclonal origin for the epithelial and mesenchymal components of this tumor; however, there is insufficient evidence.⁶ Surgical excision is the primary treatment modality for epidermal PCC, but adjuvant or substitutive radiotherapy has been used in some cases.⁴ The prognosis of PCC is notably better than its visceral counterpart due to early diagnosis and treatment of easily visible lesions. Epidermal PCC has a 70% 5-year disease-free survival rate, while adnexal PCC tends to occur in younger patients and has a 25% 5-year disease-free survival rate.³ Due to the rarity of reported cases and limited follow-up, the long-term prognosis for PCC remains unclear.

We report an unusual case of PCC on the scalp that was successfully treated with radiation therapy alone. This modality should be considered in patients with large tumors who refuse surgery or are not good surgical candidates.

The Diagnosis: Primary Cutaneous Carcinosarcoma

A generous shave biopsy and debulking performed on the initial visit revealed an infiltrating tumor consisting of malignant epithelial and stromal components (Figure). The basaloid and squamoid epithelial cells were keratin positive. The stromal cells demonstrated positivity for CD10 but were keratin negative. The epithelial portion of the tumor was composed mostly of basaloid islands of cells with nuclear pleomorphism, scattered mitoses, and focal sebaceous differentiation. The mesenchymal portion of the tumor displayed florid pleomorphism and polymorphism, with many large atypical cells and proliferation. A diagnosis of primary cutaneous carcinosarcoma (PCC) was rendered. Head and neck computed tomography showed tumor penetration of less than 1 cm into scalp soft tissues with no involvement of the underlying bone. There was some evidence of swelling of the supragaleal soft tissues without indication of perineural spread. An 11-mm hyperlucent lower cervical lymph node on the left side that likely represented an incidental finding was noted. Surgical excision with margin evaluation was recommended, but the patient declined. He instead received radiation therapy to the left side of the posterior scalp with a total dose of 30 Gy at 6 Gy per fraction and 1 fraction daily. The patient was found to have a well-healed scar with no evidence of recurrence at 4-week follow-up and again at 5 months after radiation therapy.

A generous shave biopsy and debulking performed on the initial visit revealed an inflitrating tumor consisting on malignant epithelial and stromal components (A-C)(H&E; original magnifications ×10, ×20, and ×40, respectively).

Primary cutaneous carcinosarcoma is a rare biphasic neoplasm of unknown etiology that is characterized by the presence of both malignant epithelial and mesenchymal components.¹ Carcinosarcomas have been reported in both the male and female reproductive tracts, urinary tract, gastrointestinal tract, lungs, breasts, larynx, thymus, and thyroid but is uncommon as a primary neoplasm of the skin.² Epidermal PCC occurs with greater frequency in males than in females and typically presents in the eighth or ninth decades of life.³ These tumors tend to arise in sun-exposed regions, most commonly on the face and scalp.²

Morphologically, PCCs typically are exophytic growths that often feature surface ulceration and may or may not bleed upon palpation.⁴ Primary cutaneous carcinosarcomas may present as long-standing lesions that have undergone rapid transformation in the weeks preceding presentation.⁴ It is not uncommon for PCC lesions to carry the clinical diagnosis of squamous cell carcinoma, which suggests notable morphologic overlap between these entities. Histopathologically, PCC shows a basal cell carcinoma and/or a squamous cell carcinoma epithelial component intimately admixed with a sarcomatous component.⁵ The mesenchymal component of PCC typically resembles a superficial malignant fibrous histiocytoma characterized by pleomorphic nuclei and cytoplasm, necrosis, and an increased number of mitotic figures.² Immunohistochemistry can be beneficial in the diagnosis of PCC. A combination of p63 and AE1/AE3 stains can be used to confirm cells of epithelial origin. Staining with vimentin, CD10, or caldesmon can help to delineate the mesenchymal component of PCC.

Epidermal PCC most commonly affects elderly individuals with a history of extensive sun exposure. It has been suggested that p53 mutations due to UV damage are key in tumor formation for both epithelial and mesenchymal elements.⁵ Literature supports a monoclonal origin for the epithelial and mesenchymal components of this tumor; however, there is insufficient evidence.⁶ Surgical excision is the primary treatment modality for epidermal PCC, but adjuvant or substitutive radiotherapy has been used in some cases.⁴ The prognosis of PCC is notably better than its visceral counterpart due to early diagnosis and treatment of easily visible lesions. Epidermal PCC has a 70% 5-year disease-free survival rate, while adnexal PCC tends to occur in younger patients and has a 25% 5-year disease-free survival rate.³ Due to the rarity of reported cases and limited follow-up, the long-term prognosis for PCC remains unclear.

We report an unusual case of PCC on the scalp that was successfully treated with radiation therapy alone. This modality should be considered in patients with large tumors who refuse surgery or are not good surgical candidates.

The Diagnosis: Primary Cutaneous Carcinosarcoma

A generous shave biopsy and debulking performed on the initial visit revealed an infiltrating tumor consisting of malignant epithelial and stromal components (Figure). The basaloid and squamoid epithelial cells were keratin positive. The stromal cells demonstrated positivity for CD10 but were keratin negative. The epithelial portion of the tumor was composed mostly of basaloid islands of cells with nuclear pleomorphism, scattered mitoses, and focal sebaceous differentiation. The mesenchymal portion of the tumor displayed florid pleomorphism and polymorphism, with many large atypical cells and proliferation. A diagnosis of primary cutaneous carcinosarcoma (PCC) was rendered. Head and neck computed tomography showed tumor penetration of less than 1 cm into scalp soft tissues with no involvement of the underlying bone. There was some evidence of swelling of the supragaleal soft tissues without indication of perineural spread. An 11-mm hyperlucent lower cervical lymph node on the left side that likely represented an incidental finding was noted. Surgical excision with margin evaluation was recommended, but the patient declined. He instead received radiation therapy to the left side of the posterior scalp with a total dose of 30 Gy at 6 Gy per fraction and 1 fraction daily. The patient was found to have a well-healed scar with no evidence of recurrence at 4-week follow-up and again at 5 months after radiation therapy.

A generous shave biopsy and debulking performed on the initial visit revealed an inflitrating tumor consisting on malignant epithelial and stromal components (A-C)(H&E; original magnifications ×10, ×20, and ×40, respectively).

Primary cutaneous carcinosarcoma is a rare biphasic neoplasm of unknown etiology that is characterized by the presence of both malignant epithelial and mesenchymal components.¹ Carcinosarcomas have been reported in both the male and female reproductive tracts, urinary tract, gastrointestinal tract, lungs, breasts, larynx, thymus, and thyroid but is uncommon as a primary neoplasm of the skin.² Epidermal PCC occurs with greater frequency in males than in females and typically presents in the eighth or ninth decades of life.³ These tumors tend to arise in sun-exposed regions, most commonly on the face and scalp.²

Morphologically, PCCs typically are exophytic growths that often feature surface ulceration and may or may not bleed upon palpation.⁴ Primary cutaneous carcinosarcomas may present as long-standing lesions that have undergone rapid transformation in the weeks preceding presentation.⁴ It is not uncommon for PCC lesions to carry the clinical diagnosis of squamous cell carcinoma, which suggests notable morphologic overlap between these entities. Histopathologically, PCC shows a basal cell carcinoma and/or a squamous cell carcinoma epithelial component intimately admixed with a sarcomatous component.⁵ The mesenchymal component of PCC typically resembles a superficial malignant fibrous histiocytoma characterized by pleomorphic nuclei and cytoplasm, necrosis, and an increased number of mitotic figures.² Immunohistochemistry can be beneficial in the diagnosis of PCC. A combination of p63 and AE1/AE3 stains can be used to confirm cells of epithelial origin. Staining with vimentin, CD10, or caldesmon can help to delineate the mesenchymal component of PCC.

Epidermal PCC most commonly affects elderly individuals with a history of extensive sun exposure. It has been suggested that p53 mutations due to UV damage are key in tumor formation for both epithelial and mesenchymal elements.⁵ Literature supports a monoclonal origin for the epithelial and mesenchymal components of this tumor; however, there is insufficient evidence.⁶ Surgical excision is the primary treatment modality for epidermal PCC, but adjuvant or substitutive radiotherapy has been used in some cases.⁴ The prognosis of PCC is notably better than its visceral counterpart due to early diagnosis and treatment of easily visible lesions. Epidermal PCC has a 70% 5-year disease-free survival rate, while adnexal PCC tends to occur in younger patients and has a 25% 5-year disease-free survival rate.³ Due to the rarity of reported cases and limited follow-up, the long-term prognosis for PCC remains unclear.

We report an unusual case of PCC on the scalp that was successfully treated with radiation therapy alone. This modality should be considered in patients with large tumors who refuse surgery or are not good surgical candidates.

The Diagnosis: Herpetic Glossitis

Oral lesions of the tongue are common during primary herpetic gingivostomatitis, though most primary oral herpes simplex virus (HSV) infections occur during childhood or early adulthood. Reactivation of HSV type 1 most commonly manifests as herpes labialis.¹ When recurrent HSV involves intraoral lesions, they are typically confined to the gingiva and palate, sparing the tongue.

Clinical presentation of herpetic glossitis varies. Recurrent herpetic glossitis has been described in immunocompromised patients, particularly those with hematologic malignancies and organ transplants.² In addition, immunocompromised and human immunodeficiency virus–infected patients may present with deep and/or broad ulcers. A case of herpes infection presenting with nodules on the tongue has been reported in Hodgkin disease.³ Herpetic geometric glossitis also has been described, which is a linear, crosshatched, or sharply angled branching with painful fissuring of the tongue. Herpetic geometric glossitis has been reported to occur in both immunocompetent and immunocompromised individuals.⁴ Tongue involvement during oral reactivation of HSV is exceedingly rare and the pathogenesis remains elusive, though one hypothesis proposes a protective role of salivary-specific IgA and lysozyme.⁵ Here, we report a case in which a patient developed similar lingual HSV lesions following recent immunosuppression.

The Diagnosis: Herpetic Glossitis

Oral lesions of the tongue are common during primary herpetic gingivostomatitis, though most primary oral herpes simplex virus (HSV) infections occur during childhood or early adulthood. Reactivation of HSV type 1 most commonly manifests as herpes labialis.¹ When recurrent HSV involves intraoral lesions, they are typically confined to the gingiva and palate, sparing the tongue.

Clinical presentation of herpetic glossitis varies. Recurrent herpetic glossitis has been described in immunocompromised patients, particularly those with hematologic malignancies and organ transplants.² In addition, immunocompromised and human immunodeficiency virus–infected patients may present with deep and/or broad ulcers. A case of herpes infection presenting with nodules on the tongue has been reported in Hodgkin disease.³ Herpetic geometric glossitis also has been described, which is a linear, crosshatched, or sharply angled branching with painful fissuring of the tongue. Herpetic geometric glossitis has been reported to occur in both immunocompetent and immunocompromised individuals.⁴ Tongue involvement during oral reactivation of HSV is exceedingly rare and the pathogenesis remains elusive, though one hypothesis proposes a protective role of salivary-specific IgA and lysozyme.⁵ Here, we report a case in which a patient developed similar lingual HSV lesions following recent immunosuppression.

The Diagnosis: Herpetic Glossitis

Oral lesions of the tongue are common during primary herpetic gingivostomatitis, though most primary oral herpes simplex virus (HSV) infections occur during childhood or early adulthood. Reactivation of HSV type 1 most commonly manifests as herpes labialis.¹ When recurrent HSV involves intraoral lesions, they are typically confined to the gingiva and palate, sparing the tongue.

Clinical presentation of herpetic glossitis varies. Recurrent herpetic glossitis has been described in immunocompromised patients, particularly those with hematologic malignancies and organ transplants.² In addition, immunocompromised and human immunodeficiency virus–infected patients may present with deep and/or broad ulcers. A case of herpes infection presenting with nodules on the tongue has been reported in Hodgkin disease.³ Herpetic geometric glossitis also has been described, which is a linear, crosshatched, or sharply angled branching with painful fissuring of the tongue. Herpetic geometric glossitis has been reported to occur in both immunocompetent and immunocompromised individuals.⁴ Tongue involvement during oral reactivation of HSV is exceedingly rare and the pathogenesis remains elusive, though one hypothesis proposes a protective role of salivary-specific IgA and lysozyme.⁵ Here, we report a case in which a patient developed similar lingual HSV lesions following recent immunosuppression.

The Diagnosis: Superficial Acral Fibromyxoma

Superficial acral fibromyxoma (SAF) was first described in 2001 by Fetsch et al.¹ Subsequently, the term digital fibromyxoma was proposed in 2012 by Hollmann et al² to describe a distinctive, slow-growing, soft-tissue tumor with a predilection for the periungual or subungual regions of the fingers and toes. The benign growth typically presents as a painless or tender nodule in middle-aged adults with a slight male predominance (1.3:1 ratio).^1,2 In a case series (N=124) described by Hollmann et al,² 9 of 25 patients (36%) who had imaging studies showed bone involvement by an erosive or lytic lesion. Reports of SAF with bone involvement also have been described in the radiologic and orthopedic surgery literature.^3,4 Radiographically, the soft-tissue invasion of the bone is demonstrated by scalloping on plain radiographs (Figure 1).³

Histologically, SAFs are moderately cellular with spindled or stellate fibroblastlike cells within a myxoid or collagenous matrix (Figure 2).¹ The vasculature is mildly accentuated and an increase in mast cells usually is observed. The nuclei have a low degree of atypia with few mitotic figures, and the stellate cells exhibit positive immunohistochemical staining for CD34 (Figure 3), epithelial membrane antigen, and CD99.¹ Hollmann et al² found that 66 of 95 tumors (69.5%) infiltrated the dermal collagen, 26 (27.4%) infiltrated fat, and 3 (3.2%) invaded bone. Of the 47 cases that were evaluated on follow-up, 10 tumors (21.3%) recurred locally (all near the nail unit of the fingers or toes) after a mean interval of 27 months. Although invasion of underlying tissues and recurrence of the tumor has been demonstrated, this growth is considered benign. The histologic differential diagnosis includes neurofibroma, myxoma, fibroma, low-grade fibromyxoid sarcoma, dermatofibroma, superficial angiomyxoma, and dermatofibrosarcoma protuberans.²

The primary treatment of SAF is local excision. The incidence of local recurrence found in the case series by Hollmann et al² was directly linked to positive margins after the first excision (10/47 [21.3%] recurrent lesions had positive margins). To date, there are no known reports of metastatic disease in SAF.² Our case manifested with a late recurrence of the tumor and bone involvement requiring surgical excision, which illustrates the role of adjuvant imaging and close follow-up following excision of any soft-tissue tumors of the fingers and toes that have been histologically confirmed as SAF, particularly those of the periungual region.

The Diagnosis: Superficial Acral Fibromyxoma

Superficial acral fibromyxoma (SAF) was first described in 2001 by Fetsch et al.¹ Subsequently, the term digital fibromyxoma was proposed in 2012 by Hollmann et al² to describe a distinctive, slow-growing, soft-tissue tumor with a predilection for the periungual or subungual regions of the fingers and toes. The benign growth typically presents as a painless or tender nodule in middle-aged adults with a slight male predominance (1.3:1 ratio).^1,2 In a case series (N=124) described by Hollmann et al,² 9 of 25 patients (36%) who had imaging studies showed bone involvement by an erosive or lytic lesion. Reports of SAF with bone involvement also have been described in the radiologic and orthopedic surgery literature.^3,4 Radiographically, the soft-tissue invasion of the bone is demonstrated by scalloping on plain radiographs (Figure 1).³

Histologically, SAFs are moderately cellular with spindled or stellate fibroblastlike cells within a myxoid or collagenous matrix (Figure 2).¹ The vasculature is mildly accentuated and an increase in mast cells usually is observed. The nuclei have a low degree of atypia with few mitotic figures, and the stellate cells exhibit positive immunohistochemical staining for CD34 (Figure 3), epithelial membrane antigen, and CD99.¹ Hollmann et al² found that 66 of 95 tumors (69.5%) infiltrated the dermal collagen, 26 (27.4%) infiltrated fat, and 3 (3.2%) invaded bone. Of the 47 cases that were evaluated on follow-up, 10 tumors (21.3%) recurred locally (all near the nail unit of the fingers or toes) after a mean interval of 27 months. Although invasion of underlying tissues and recurrence of the tumor has been demonstrated, this growth is considered benign. The histologic differential diagnosis includes neurofibroma, myxoma, fibroma, low-grade fibromyxoid sarcoma, dermatofibroma, superficial angiomyxoma, and dermatofibrosarcoma protuberans.²

The primary treatment of SAF is local excision. The incidence of local recurrence found in the case series by Hollmann et al² was directly linked to positive margins after the first excision (10/47 [21.3%] recurrent lesions had positive margins). To date, there are no known reports of metastatic disease in SAF.² Our case manifested with a late recurrence of the tumor and bone involvement requiring surgical excision, which illustrates the role of adjuvant imaging and close follow-up following excision of any soft-tissue tumors of the fingers and toes that have been histologically confirmed as SAF, particularly those of the periungual region.

The Diagnosis: Superficial Acral Fibromyxoma

Superficial acral fibromyxoma (SAF) was first described in 2001 by Fetsch et al.¹ Subsequently, the term digital fibromyxoma was proposed in 2012 by Hollmann et al² to describe a distinctive, slow-growing, soft-tissue tumor with a predilection for the periungual or subungual regions of the fingers and toes. The benign growth typically presents as a painless or tender nodule in middle-aged adults with a slight male predominance (1.3:1 ratio).^1,2 In a case series (N=124) described by Hollmann et al,² 9 of 25 patients (36%) who had imaging studies showed bone involvement by an erosive or lytic lesion. Reports of SAF with bone involvement also have been described in the radiologic and orthopedic surgery literature.^3,4 Radiographically, the soft-tissue invasion of the bone is demonstrated by scalloping on plain radiographs (Figure 1).³

Histologically, SAFs are moderately cellular with spindled or stellate fibroblastlike cells within a myxoid or collagenous matrix (Figure 2).¹ The vasculature is mildly accentuated and an increase in mast cells usually is observed. The nuclei have a low degree of atypia with few mitotic figures, and the stellate cells exhibit positive immunohistochemical staining for CD34 (Figure 3), epithelial membrane antigen, and CD99.¹ Hollmann et al² found that 66 of 95 tumors (69.5%) infiltrated the dermal collagen, 26 (27.4%) infiltrated fat, and 3 (3.2%) invaded bone. Of the 47 cases that were evaluated on follow-up, 10 tumors (21.3%) recurred locally (all near the nail unit of the fingers or toes) after a mean interval of 27 months. Although invasion of underlying tissues and recurrence of the tumor has been demonstrated, this growth is considered benign. The histologic differential diagnosis includes neurofibroma, myxoma, fibroma, low-grade fibromyxoid sarcoma, dermatofibroma, superficial angiomyxoma, and dermatofibrosarcoma protuberans.²

The primary treatment of SAF is local excision. The incidence of local recurrence found in the case series by Hollmann et al² was directly linked to positive margins after the first excision (10/47 [21.3%] recurrent lesions had positive margins). To date, there are no known reports of metastatic disease in SAF.² Our case manifested with a late recurrence of the tumor and bone involvement requiring surgical excision, which illustrates the role of adjuvant imaging and close follow-up following excision of any soft-tissue tumors of the fingers and toes that have been histologically confirmed as SAF, particularly those of the periungual region.

The Diagnosis: Congenital Folliculosebaceous Cystic Hamartoma

Folliculosebaceous cystic hamartoma (FSCH) is a rare skin condition that is either congenital or acquired. It presents as a slow-growing and flesh-colored papulonodular lesion¹ that mainly occurs on the head and neck. Involvement of the nipples, perineum, back, forearms, genital areas, and subcutaneous tissue also has been reported but usually indicates a larger lesion.^1,2

Histologically, FSCH is considered a hamartoma composed of both ectodermal and mesodermal elements.¹ Folliculosebaceous cystic hamartoma is a more complex lesion composed of infundibulocystic structures connected to maloriented folliculosebaceous units surrounded by whorls of highly vascularized fibrous stroma and adipocytes. Clefts between fibroepithelial units and surrounding stroma usually are present.¹

Epithelial components contribute to the adnexal and folliculosebaceous cystic proliferations, and mesenchymal elements include vascular tissue, adipose tissue, and fibroblast-rich stroma.^1,2 Acquired lesions arising in adults have been described,^1-5 but the congenital presentation of FSCH in infancy is rare.

Histopathologically, some variations of FSCH are mainly composed of epithelial components while others are composed of nonepithelial components. Nonepithelial components include neural proliferation, muscle components, vascular proliferation, and mucin deposition.^1-4 In some cases, FSCH may coexist with other diseases, such as nevus lipomatosus cutaneous superficialis and neurofibromatosis type I.^4,5

In our case, histopathology showed several dermal infundibulocystic structures that were lined by stratified squamous epithelium and contained horny material (Figure 1). Numerous immature sebaceous lobules and rudimentary hair follicles emanated from some of the cyst walls. Mesenchymal changes around the fibroepithelial units included fibrillary bundles of collagen, clusters of adipocytes, and an increased number of small venules (Figure 2). In addition, the stroma adjacent to the malformed perifollicle contained some amount of mucin. Prominent clefts formed between fibroepithelial units and the surrounding altered stroma.
 

The differential diagnosis mainly includes sebaceous trichofolliculoma, molluscum contagiosum, dermoid cysts, pilomatrixoma, Spitz nevus, and nevus lipomatosus superficialis. The differential diagnosis between FSCH and sebaceous trichofolliculoma is challenging. Both lesions show an infundibular cyst and surrounding sebaceous nodules. According to Plewig,⁶ trichofolliculoma has a wide spectrum ranging from low to high differentiation represented by trichofolliculoma, sebaceous trichofolliculoma, and FSCH, respectively. It is not difficult to distinguish FSCH from other diseases according to its peculiar histopathologic features.

The clinicopathologic features of our case were similar to those of reported FSCH cases, except for the following unique characteristics: congenital lesion, lack of terminal hair, and no sebaceous material extrusion. These features of hair and sebaceous material may be correlated with the patient’s age and hormonal level.¹ Androgen may play a key role in sebaceous gland development at puberty, which leads to sebaceous gland hyperplasia and hypertrophy. Therefore, slight pressure from the lesions can make ivory-white sebaceous material discharge. Hence, the dermatologist and pediatrician must be poised and sensitive while making an initial diagnosis of FSCH.

The Diagnosis: Congenital Folliculosebaceous Cystic Hamartoma

Folliculosebaceous cystic hamartoma (FSCH) is a rare skin condition that is either congenital or acquired. It presents as a slow-growing and flesh-colored papulonodular lesion¹ that mainly occurs on the head and neck. Involvement of the nipples, perineum, back, forearms, genital areas, and subcutaneous tissue also has been reported but usually indicates a larger lesion.^1,2

Histologically, FSCH is considered a hamartoma composed of both ectodermal and mesodermal elements.¹ Folliculosebaceous cystic hamartoma is a more complex lesion composed of infundibulocystic structures connected to maloriented folliculosebaceous units surrounded by whorls of highly vascularized fibrous stroma and adipocytes. Clefts between fibroepithelial units and surrounding stroma usually are present.¹

Epithelial components contribute to the adnexal and folliculosebaceous cystic proliferations, and mesenchymal elements include vascular tissue, adipose tissue, and fibroblast-rich stroma.^1,2 Acquired lesions arising in adults have been described,^1-5 but the congenital presentation of FSCH in infancy is rare.

Histopathologically, some variations of FSCH are mainly composed of epithelial components while others are composed of nonepithelial components. Nonepithelial components include neural proliferation, muscle components, vascular proliferation, and mucin deposition.^1-4 In some cases, FSCH may coexist with other diseases, such as nevus lipomatosus cutaneous superficialis and neurofibromatosis type I.^4,5

In our case, histopathology showed several dermal infundibulocystic structures that were lined by stratified squamous epithelium and contained horny material (Figure 1). Numerous immature sebaceous lobules and rudimentary hair follicles emanated from some of the cyst walls. Mesenchymal changes around the fibroepithelial units included fibrillary bundles of collagen, clusters of adipocytes, and an increased number of small venules (Figure 2). In addition, the stroma adjacent to the malformed perifollicle contained some amount of mucin. Prominent clefts formed between fibroepithelial units and the surrounding altered stroma.
 

The differential diagnosis mainly includes sebaceous trichofolliculoma, molluscum contagiosum, dermoid cysts, pilomatrixoma, Spitz nevus, and nevus lipomatosus superficialis. The differential diagnosis between FSCH and sebaceous trichofolliculoma is challenging. Both lesions show an infundibular cyst and surrounding sebaceous nodules. According to Plewig,⁶ trichofolliculoma has a wide spectrum ranging from low to high differentiation represented by trichofolliculoma, sebaceous trichofolliculoma, and FSCH, respectively. It is not difficult to distinguish FSCH from other diseases according to its peculiar histopathologic features.

The clinicopathologic features of our case were similar to those of reported FSCH cases, except for the following unique characteristics: congenital lesion, lack of terminal hair, and no sebaceous material extrusion. These features of hair and sebaceous material may be correlated with the patient’s age and hormonal level.¹ Androgen may play a key role in sebaceous gland development at puberty, which leads to sebaceous gland hyperplasia and hypertrophy. Therefore, slight pressure from the lesions can make ivory-white sebaceous material discharge. Hence, the dermatologist and pediatrician must be poised and sensitive while making an initial diagnosis of FSCH.

The Diagnosis: Congenital Folliculosebaceous Cystic Hamartoma

Folliculosebaceous cystic hamartoma (FSCH) is a rare skin condition that is either congenital or acquired. It presents as a slow-growing and flesh-colored papulonodular lesion¹ that mainly occurs on the head and neck. Involvement of the nipples, perineum, back, forearms, genital areas, and subcutaneous tissue also has been reported but usually indicates a larger lesion.^1,2

Histologically, FSCH is considered a hamartoma composed of both ectodermal and mesodermal elements.¹ Folliculosebaceous cystic hamartoma is a more complex lesion composed of infundibulocystic structures connected to maloriented folliculosebaceous units surrounded by whorls of highly vascularized fibrous stroma and adipocytes. Clefts between fibroepithelial units and surrounding stroma usually are present.¹

Epithelial components contribute to the adnexal and folliculosebaceous cystic proliferations, and mesenchymal elements include vascular tissue, adipose tissue, and fibroblast-rich stroma.^1,2 Acquired lesions arising in adults have been described,^1-5 but the congenital presentation of FSCH in infancy is rare.

Histopathologically, some variations of FSCH are mainly composed of epithelial components while others are composed of nonepithelial components. Nonepithelial components include neural proliferation, muscle components, vascular proliferation, and mucin deposition.^1-4 In some cases, FSCH may coexist with other diseases, such as nevus lipomatosus cutaneous superficialis and neurofibromatosis type I.^4,5

In our case, histopathology showed several dermal infundibulocystic structures that were lined by stratified squamous epithelium and contained horny material (Figure 1). Numerous immature sebaceous lobules and rudimentary hair follicles emanated from some of the cyst walls. Mesenchymal changes around the fibroepithelial units included fibrillary bundles of collagen, clusters of adipocytes, and an increased number of small venules (Figure 2). In addition, the stroma adjacent to the malformed perifollicle contained some amount of mucin. Prominent clefts formed between fibroepithelial units and the surrounding altered stroma.
 

The differential diagnosis mainly includes sebaceous trichofolliculoma, molluscum contagiosum, dermoid cysts, pilomatrixoma, Spitz nevus, and nevus lipomatosus superficialis. The differential diagnosis between FSCH and sebaceous trichofolliculoma is challenging. Both lesions show an infundibular cyst and surrounding sebaceous nodules. According to Plewig,⁶ trichofolliculoma has a wide spectrum ranging from low to high differentiation represented by trichofolliculoma, sebaceous trichofolliculoma, and FSCH, respectively. It is not difficult to distinguish FSCH from other diseases according to its peculiar histopathologic features.

The clinicopathologic features of our case were similar to those of reported FSCH cases, except for the following unique characteristics: congenital lesion, lack of terminal hair, and no sebaceous material extrusion. These features of hair and sebaceous material may be correlated with the patient’s age and hormonal level.¹ Androgen may play a key role in sebaceous gland development at puberty, which leads to sebaceous gland hyperplasia and hypertrophy. Therefore, slight pressure from the lesions can make ivory-white sebaceous material discharge. Hence, the dermatologist and pediatrician must be poised and sensitive while making an initial diagnosis of FSCH.

ANSWER
This ECG demonstrates marked sinus bradycardia with a second-degree atrioventricular (AV) block (Mobitz I). Other findings include left-axis deviation, an old inferior MI, and poor R-wave progression.

Second-degree Mobitz I block is present in a 3:1 pattern of progressive prolongation of the PR interval until the third beat, where there is block in the AV node preventing conduction of the P wave to the ventricles. This is typically caused by progressive fatigue within the AV node until block occurs, then the cycle repeats.

Left-axis deviation is evidenced by a QRS axis of –78°. An old inferior MI is signified by the significant Q waves in leads II, III, and aVF. Finally, poor R-wave progression is demonstrated by small R waves in all of the precordial leads.

This ECG represented a significant change from one obtained three months earlier, during a routine outpatient visit. Careful review of the records at the Alzheimer facility revealed that the patient had received twice his usual dose of propranolol on three consecutive days. His rhythm returned to a baseline sinus rhythm (at 68 beats/min) after his ß-blocker was withheld for two days, and no further intervention was needed.

ANSWER
This ECG demonstrates marked sinus bradycardia with a second-degree atrioventricular (AV) block (Mobitz I). Other findings include left-axis deviation, an old inferior MI, and poor R-wave progression.

Second-degree Mobitz I block is present in a 3:1 pattern of progressive prolongation of the PR interval until the third beat, where there is block in the AV node preventing conduction of the P wave to the ventricles. This is typically caused by progressive fatigue within the AV node until block occurs, then the cycle repeats.

Left-axis deviation is evidenced by a QRS axis of –78°. An old inferior MI is signified by the significant Q waves in leads II, III, and aVF. Finally, poor R-wave progression is demonstrated by small R waves in all of the precordial leads.

This ECG represented a significant change from one obtained three months earlier, during a routine outpatient visit. Careful review of the records at the Alzheimer facility revealed that the patient had received twice his usual dose of propranolol on three consecutive days. His rhythm returned to a baseline sinus rhythm (at 68 beats/min) after his ß-blocker was withheld for two days, and no further intervention was needed.

ANSWER
This ECG demonstrates marked sinus bradycardia with a second-degree atrioventricular (AV) block (Mobitz I). Other findings include left-axis deviation, an old inferior MI, and poor R-wave progression.

Second-degree Mobitz I block is present in a 3:1 pattern of progressive prolongation of the PR interval until the third beat, where there is block in the AV node preventing conduction of the P wave to the ventricles. This is typically caused by progressive fatigue within the AV node until block occurs, then the cycle repeats.

Left-axis deviation is evidenced by a QRS axis of –78°. An old inferior MI is signified by the significant Q waves in leads II, III, and aVF. Finally, poor R-wave progression is demonstrated by small R waves in all of the precordial leads.

This ECG represented a significant change from one obtained three months earlier, during a routine outpatient visit. Careful review of the records at the Alzheimer facility revealed that the patient had received twice his usual dose of propranolol on three consecutive days. His rhythm returned to a baseline sinus rhythm (at 68 beats/min) after his ß-blocker was withheld for two days, and no further intervention was needed.

Answer
The radiograph has several findings. First, there are multiple fractures along the left lateral ribs. Second, there is a small left apical pneumothorax. Most significant, though, is an elevated left hemidiaphragm. There appears to be stomach or possibly bowel protruding into it.

Although a large hiatal hernia could yield similar findings, in the setting of trauma, one must be concerned about a traumatic diaphragmatic hernia. Subsequent CT of the chest, abdomen, and pelvis confirmed a defect within the diaphragm, with a significant portion of the stomach herniating into the left chest.

Surgical consultation was obtained. The patient was admitted and ultimately underwent surgical intervention to repair the problem.

Answer
The radiograph has several findings. First, there are multiple fractures along the left lateral ribs. Second, there is a small left apical pneumothorax. Most significant, though, is an elevated left hemidiaphragm. There appears to be stomach or possibly bowel protruding into it.

Although a large hiatal hernia could yield similar findings, in the setting of trauma, one must be concerned about a traumatic diaphragmatic hernia. Subsequent CT of the chest, abdomen, and pelvis confirmed a defect within the diaphragm, with a significant portion of the stomach herniating into the left chest.

Surgical consultation was obtained. The patient was admitted and ultimately underwent surgical intervention to repair the problem.

Answer
The radiograph has several findings. First, there are multiple fractures along the left lateral ribs. Second, there is a small left apical pneumothorax. Most significant, though, is an elevated left hemidiaphragm. There appears to be stomach or possibly bowel protruding into it.

Although a large hiatal hernia could yield similar findings, in the setting of trauma, one must be concerned about a traumatic diaphragmatic hernia. Subsequent CT of the chest, abdomen, and pelvis confirmed a defect within the diaphragm, with a significant portion of the stomach herniating into the left chest.

Surgical consultation was obtained. The patient was admitted and ultimately underwent surgical intervention to repair the problem.

ANSWER
The correct answer is palmoplantar hyperkeratosis (PPK; choice “c”). As a category, it includes many conditions that are characterized by a heterogeneous thickening of the palms and soles, although each has distinct features.

Psoriasis (choice “a”) can pre­sent in somewhat similar fashion. However, it rarely appears so early in life and almost never involves such uniform hyperkeratosis.

Xerosis (choice “b”) merely means dry skin—a common enough problem, but one that does not involve such uniform and deep hyperkeratosis. It is almost never congenital.

Occult cancers (eg, GI, breast) can occasionally trigger a hyperkeratotic “perineoplastic” reaction (choice “d”) in the palms, soles, and other areas. However, it is acquired relatively late in life.

DISCUSSION
PPK is a relatively common problem, especially in those of northern European ancestry. The incidence in Northern Ireland, for example, is 4.4/100,000. In its more severe forms, such as this case, it can be debilitating.

Categorization of this extraordinarily diverse group of disorders is a challenge, to say the least. For purposes of this discussion, let’s start with this patient’s condition and then clarify its place in the panoply of hyperkeratotic disorders.

Our patient has one of the more common forms of diffuse PPK, generically called nonepidermolytic PPK, which presents with a waxy, uniform yellowed hyperkeratosis confined to the palms and soles. The old eponymic term for it was Unna-Thost disorder, named for the Viennese and Norwegian dermatologists who first described it late in the 19th century. A more modern term is tylosis, but many variations exist. This particular form is inherited in autosomal dominant fashion, but other forms of PPK can be transmitted by autosomal recessive or even X-linked genes.

Other types include focal (thick calluses over points of friction, especially on the feet) versions that can present in linear configuration and punctate forms with deep calluses on palms and soles that can be discrete or widespread, with lesions that range in size from pinpoint (“speculated”) to pea-sized.

New-onset PPK should prompt a search for an occult malignancy. In terms of establishing a firm diagnosis, punch biopsy can be performed for clarification when necessary.

As might be expected, treatment is difficult at best. Urea or salicylic acid–based topical preparations can help to thin it out. This patient was started on acitretin, an oral retinoid (artificial form of vitamin A) that may help; it was one of the few treatments he hadn’t already tried. A one-month course of terbinafine had already been tried, without success, just in case there was any secondary fungal involvement—a fairly common complication of PPK.

ANSWER
The correct answer is palmoplantar hyperkeratosis (PPK; choice “c”). As a category, it includes many conditions that are characterized by a heterogeneous thickening of the palms and soles, although each has distinct features.

Psoriasis (choice “a”) can pre­sent in somewhat similar fashion. However, it rarely appears so early in life and almost never involves such uniform hyperkeratosis.

Xerosis (choice “b”) merely means dry skin—a common enough problem, but one that does not involve such uniform and deep hyperkeratosis. It is almost never congenital.

Occult cancers (eg, GI, breast) can occasionally trigger a hyperkeratotic “perineoplastic” reaction (choice “d”) in the palms, soles, and other areas. However, it is acquired relatively late in life.

DISCUSSION
PPK is a relatively common problem, especially in those of northern European ancestry. The incidence in Northern Ireland, for example, is 4.4/100,000. In its more severe forms, such as this case, it can be debilitating.

Categorization of this extraordinarily diverse group of disorders is a challenge, to say the least. For purposes of this discussion, let’s start with this patient’s condition and then clarify its place in the panoply of hyperkeratotic disorders.

Our patient has one of the more common forms of diffuse PPK, generically called nonepidermolytic PPK, which presents with a waxy, uniform yellowed hyperkeratosis confined to the palms and soles. The old eponymic term for it was Unna-Thost disorder, named for the Viennese and Norwegian dermatologists who first described it late in the 19th century. A more modern term is tylosis, but many variations exist. This particular form is inherited in autosomal dominant fashion, but other forms of PPK can be transmitted by autosomal recessive or even X-linked genes.

Other types include focal (thick calluses over points of friction, especially on the feet) versions that can present in linear configuration and punctate forms with deep calluses on palms and soles that can be discrete or widespread, with lesions that range in size from pinpoint (“speculated”) to pea-sized.

New-onset PPK should prompt a search for an occult malignancy. In terms of establishing a firm diagnosis, punch biopsy can be performed for clarification when necessary.

As might be expected, treatment is difficult at best. Urea or salicylic acid–based topical preparations can help to thin it out. This patient was started on acitretin, an oral retinoid (artificial form of vitamin A) that may help; it was one of the few treatments he hadn’t already tried. A one-month course of terbinafine had already been tried, without success, just in case there was any secondary fungal involvement—a fairly common complication of PPK.

ANSWER
The correct answer is palmoplantar hyperkeratosis (PPK; choice “c”). As a category, it includes many conditions that are characterized by a heterogeneous thickening of the palms and soles, although each has distinct features.

Psoriasis (choice “a”) can pre­sent in somewhat similar fashion. However, it rarely appears so early in life and almost never involves such uniform hyperkeratosis.

Xerosis (choice “b”) merely means dry skin—a common enough problem, but one that does not involve such uniform and deep hyperkeratosis. It is almost never congenital.

Occult cancers (eg, GI, breast) can occasionally trigger a hyperkeratotic “perineoplastic” reaction (choice “d”) in the palms, soles, and other areas. However, it is acquired relatively late in life.

DISCUSSION
PPK is a relatively common problem, especially in those of northern European ancestry. The incidence in Northern Ireland, for example, is 4.4/100,000. In its more severe forms, such as this case, it can be debilitating.

Categorization of this extraordinarily diverse group of disorders is a challenge, to say the least. For purposes of this discussion, let’s start with this patient’s condition and then clarify its place in the panoply of hyperkeratotic disorders.

Our patient has one of the more common forms of diffuse PPK, generically called nonepidermolytic PPK, which presents with a waxy, uniform yellowed hyperkeratosis confined to the palms and soles. The old eponymic term for it was Unna-Thost disorder, named for the Viennese and Norwegian dermatologists who first described it late in the 19th century. A more modern term is tylosis, but many variations exist. This particular form is inherited in autosomal dominant fashion, but other forms of PPK can be transmitted by autosomal recessive or even X-linked genes.

Other types include focal (thick calluses over points of friction, especially on the feet) versions that can present in linear configuration and punctate forms with deep calluses on palms and soles that can be discrete or widespread, with lesions that range in size from pinpoint (“speculated”) to pea-sized.

New-onset PPK should prompt a search for an occult malignancy. In terms of establishing a firm diagnosis, punch biopsy can be performed for clarification when necessary.

As might be expected, treatment is difficult at best. Urea or salicylic acid–based topical preparations can help to thin it out. This patient was started on acitretin, an oral retinoid (artificial form of vitamin A) that may help; it was one of the few treatments he hadn’t already tried. A one-month course of terbinafine had already been tried, without success, just in case there was any secondary fungal involvement—a fairly common complication of PPK.

The Diagnosis: Palmoplantar Lichen Planus

A skin biopsy from a lesion on the inner wrist showed an interface pattern with a dense bandlike infiltrate obscuring the dermoepidermal junction coupled with a superficial perivascular infiltrate (Figure). At higher magnification (×10), the histologic features included compact orthokeratosis, wedge-shaped hypergranulosis, vacuolar degeneration of the basal layer, basal dyskeratosis, a dense lymphohistiocytic infiltrate obscuring the basement membrane, and melanophages in the papillary dermis.

Lichen planus (LP) is a common inflammatory disease of the skin presenting with flat-topped, violaceous, polygonal papules with fine white lines (Wickham striae) on the surface. It is recognized and diagnosed clinically by its characteristic appearance. Common areas of LP presentation include the shins, inner thighs, genitalia, trunk, volar aspect of the wrists, and oral mucosa.

Palmoplantar LP can present as erythematous plaques, punctuate keratosis, diffuse keratoderma, or ulcerated lesions. The most common concern among patients with LP is pruritus. One-fourth of patients with LP may present with lesions on the palms and soles, but diffuse palmoplantar hyperkeratosis is rare.¹ Lesions typically heal in 1 to 8 months, with an average of 3 months. Palmoplantar LP recurs within 1 year after stopping treatment in one-third of patients.¹

The cause of LP is unknown, but the pathophysiology is beginning to be understood. Cytotoxic CD8+ T cells stimulate apoptosis of the keratinocytes. The induction of this mechanism may be due to a self-antigen in a genetically predisposed patient. The evidence for LP being an autoimmune disease is supported by the high female predominance and the association of LP with other autoimmune diseases.² Patients with LP have an increased chance of coexisting hepatitis C virus. In a cross-sectional study of 303 patients, Lodi et al³ found that approximately 20% of LP patients were hepatitis C virus seropositive.

Treatment options for LP include topical and systemic steroids, tazarotene, acitretin, and immunosuppressive agents.⁴ Our patient initially was treated with oral cyclosporine 100 mg every morning and oral methotrexate at a dose of 7.5 mg weekly. She also was treated with clobetasol ointment 0.05%. After 3 months, cyclosporine was discontinued. Methotrexate was maintained. At 5 months’ followup there was marked improvement of both clinical and symptomatic concerns with only residual palmoplantar erythema.

The differential diagnosis for pruritic palmoplantar hyperkeratosis is large. The most common differential diagnoses include hyperkeratotic eczema, psoriasis, secondary syphilis, and hereditary palmoplantar keratoderma. Lichen planus should be considered in the differential diagnosis of palmoplantar hyperkeratosis. A skin biopsy may be needed, as palmoplantar LP often has an atypical presentation.⁵

The Diagnosis: Palmoplantar Lichen Planus

A skin biopsy from a lesion on the inner wrist showed an interface pattern with a dense bandlike infiltrate obscuring the dermoepidermal junction coupled with a superficial perivascular infiltrate (Figure). At higher magnification (×10), the histologic features included compact orthokeratosis, wedge-shaped hypergranulosis, vacuolar degeneration of the basal layer, basal dyskeratosis, a dense lymphohistiocytic infiltrate obscuring the basement membrane, and melanophages in the papillary dermis.

Lichen planus (LP) is a common inflammatory disease of the skin presenting with flat-topped, violaceous, polygonal papules with fine white lines (Wickham striae) on the surface. It is recognized and diagnosed clinically by its characteristic appearance. Common areas of LP presentation include the shins, inner thighs, genitalia, trunk, volar aspect of the wrists, and oral mucosa.

Palmoplantar LP can present as erythematous plaques, punctuate keratosis, diffuse keratoderma, or ulcerated lesions. The most common concern among patients with LP is pruritus. One-fourth of patients with LP may present with lesions on the palms and soles, but diffuse palmoplantar hyperkeratosis is rare.¹ Lesions typically heal in 1 to 8 months, with an average of 3 months. Palmoplantar LP recurs within 1 year after stopping treatment in one-third of patients.¹

The cause of LP is unknown, but the pathophysiology is beginning to be understood. Cytotoxic CD8+ T cells stimulate apoptosis of the keratinocytes. The induction of this mechanism may be due to a self-antigen in a genetically predisposed patient. The evidence for LP being an autoimmune disease is supported by the high female predominance and the association of LP with other autoimmune diseases.² Patients with LP have an increased chance of coexisting hepatitis C virus. In a cross-sectional study of 303 patients, Lodi et al³ found that approximately 20% of LP patients were hepatitis C virus seropositive.

Treatment options for LP include topical and systemic steroids, tazarotene, acitretin, and immunosuppressive agents.⁴ Our patient initially was treated with oral cyclosporine 100 mg every morning and oral methotrexate at a dose of 7.5 mg weekly. She also was treated with clobetasol ointment 0.05%. After 3 months, cyclosporine was discontinued. Methotrexate was maintained. At 5 months’ followup there was marked improvement of both clinical and symptomatic concerns with only residual palmoplantar erythema.

The differential diagnosis for pruritic palmoplantar hyperkeratosis is large. The most common differential diagnoses include hyperkeratotic eczema, psoriasis, secondary syphilis, and hereditary palmoplantar keratoderma. Lichen planus should be considered in the differential diagnosis of palmoplantar hyperkeratosis. A skin biopsy may be needed, as palmoplantar LP often has an atypical presentation.⁵

The Diagnosis: Palmoplantar Lichen Planus

A skin biopsy from a lesion on the inner wrist showed an interface pattern with a dense bandlike infiltrate obscuring the dermoepidermal junction coupled with a superficial perivascular infiltrate (Figure). At higher magnification (×10), the histologic features included compact orthokeratosis, wedge-shaped hypergranulosis, vacuolar degeneration of the basal layer, basal dyskeratosis, a dense lymphohistiocytic infiltrate obscuring the basement membrane, and melanophages in the papillary dermis.

Lichen planus (LP) is a common inflammatory disease of the skin presenting with flat-topped, violaceous, polygonal papules with fine white lines (Wickham striae) on the surface. It is recognized and diagnosed clinically by its characteristic appearance. Common areas of LP presentation include the shins, inner thighs, genitalia, trunk, volar aspect of the wrists, and oral mucosa.

Palmoplantar LP can present as erythematous plaques, punctuate keratosis, diffuse keratoderma, or ulcerated lesions. The most common concern among patients with LP is pruritus. One-fourth of patients with LP may present with lesions on the palms and soles, but diffuse palmoplantar hyperkeratosis is rare.¹ Lesions typically heal in 1 to 8 months, with an average of 3 months. Palmoplantar LP recurs within 1 year after stopping treatment in one-third of patients.¹

The cause of LP is unknown, but the pathophysiology is beginning to be understood. Cytotoxic CD8+ T cells stimulate apoptosis of the keratinocytes. The induction of this mechanism may be due to a self-antigen in a genetically predisposed patient. The evidence for LP being an autoimmune disease is supported by the high female predominance and the association of LP with other autoimmune diseases.² Patients with LP have an increased chance of coexisting hepatitis C virus. In a cross-sectional study of 303 patients, Lodi et al³ found that approximately 20% of LP patients were hepatitis C virus seropositive.

Treatment options for LP include topical and systemic steroids, tazarotene, acitretin, and immunosuppressive agents.⁴ Our patient initially was treated with oral cyclosporine 100 mg every morning and oral methotrexate at a dose of 7.5 mg weekly. She also was treated with clobetasol ointment 0.05%. After 3 months, cyclosporine was discontinued. Methotrexate was maintained. At 5 months’ followup there was marked improvement of both clinical and symptomatic concerns with only residual palmoplantar erythema.

The differential diagnosis for pruritic palmoplantar hyperkeratosis is large. The most common differential diagnoses include hyperkeratotic eczema, psoriasis, secondary syphilis, and hereditary palmoplantar keratoderma. Lichen planus should be considered in the differential diagnosis of palmoplantar hyperkeratosis. A skin biopsy may be needed, as palmoplantar LP often has an atypical presentation.⁵

The Diagnosis: Bilateral Auricular Tophaceous Gout

Histopathologic evaluation with hematoxylin and eosin staining demonstrated clusters of abundant granular amorphous material within the subcutaneous tissue (Figure 1). The overlying epidermis and dermis were unremarkable. The granular amorphous material demonstrated numerous monosodium urate crystals under polarized light (Figure 2). At a return visit following the biopsy results, the patient reported a history of a single episode of monoarticular gouty arthritis involving the right hallux approximately 6 months after the onset of the skin lesions. With the added clinical history and the biopsy results, his serum uric acid level was obtained and was found to be elevated at 9.2 mg/dL (reference range, 3.5–8 mg/dL).

In our patient, the clinical differential diagnosis included calcium deposits, weathering nodules, and tophaceous gout. The differential diagnosis of auricular lesions is broad, and benign lesions may mimic cancerous entities such as basal cell carcinoma and squamous cell carcinoma.¹ Therefore a detailed history, thorough physical examination, and tissue sampling are key to establishing the correct diagnosis. Our patient’s history of monoarticular gouty arthritis was only elucidated after a diagnosis of bilateral auricular tophaceous gout was made based on the biopsy results.

Subcutaneous tophi represent a chronic state of hyperuricemia and tend to manifest after long-standing polyarthritis and repeated acute gout attacks.^2-5 These lesions develop in approximately 50% of gout patients and usually occur an average of 11.6 years after the onset of disease.² There is a subset of individuals that are at higher risk for developing tophi, including elderly and female patients, diuretic and chronic nonsteroidal anti-inflammatory drug users, patients with a history of cyclosporine therapy, and patients with underlying chronic renal insufficiency.^2,6,7 The most commonly affected tissues are those with poor blood supply and lower temperatures, such as the ear helix and first metacarpal joint.⁴ The auricle is the most common site of tophi on the head and neck. Tophi of the helices are generally asymptomatic and nontender; however, tophi can become large, inflamed, and ulcerated, causing pressure and discomfort.² Combination treatment with dietary modification and antihyperuricemic therapy (eg, allopurinol) has been shown to reduce the size of lesions and prevent future tophi formation. However, these results may take months, warranting excision of large and symptomatic lesions.^4,8

Our case is unusual in that the onset of the auricular lesions predated the articular gout by 6 months. Gouty tophi as the initial presentation of hyperuricemia is rare; however, tophi formation without concomitant arthritis has been reported.^2,3,7,9 Wernick et al⁷ described 6 patients presenting with tophi before the onset of inflammatory arthritis that they attributed to changes in active inflammation by age (eg, elderly patients were more commonly immunosuppressed), chronic illnesses, and anti-inflammatory medications (eg, nonsteroidal anti-inflammatory drugs). Another possible explanation for this atypical presentation is misdiagnosis caused by other forms of arthritis (eg, rheumatoid arthritis, osteoarthritis) masking acute gout episodes. It also has been reported that monosodium urate crystals can be found in synovial fluid with no inflammation and therefore no symptoms.⁷

Tophi, although rare, may be the sole clinical manifestation of underlying gouty disease. It is important to be aware of this atypical presentation to prevent misdiagnosis and provide appropriate treatment.

The Diagnosis: Bilateral Auricular Tophaceous Gout

Histopathologic evaluation with hematoxylin and eosin staining demonstrated clusters of abundant granular amorphous material within the subcutaneous tissue (Figure 1). The overlying epidermis and dermis were unremarkable. The granular amorphous material demonstrated numerous monosodium urate crystals under polarized light (Figure 2). At a return visit following the biopsy results, the patient reported a history of a single episode of monoarticular gouty arthritis involving the right hallux approximately 6 months after the onset of the skin lesions. With the added clinical history and the biopsy results, his serum uric acid level was obtained and was found to be elevated at 9.2 mg/dL (reference range, 3.5–8 mg/dL).

In our patient, the clinical differential diagnosis included calcium deposits, weathering nodules, and tophaceous gout. The differential diagnosis of auricular lesions is broad, and benign lesions may mimic cancerous entities such as basal cell carcinoma and squamous cell carcinoma.¹ Therefore a detailed history, thorough physical examination, and tissue sampling are key to establishing the correct diagnosis. Our patient’s history of monoarticular gouty arthritis was only elucidated after a diagnosis of bilateral auricular tophaceous gout was made based on the biopsy results.

Subcutaneous tophi represent a chronic state of hyperuricemia and tend to manifest after long-standing polyarthritis and repeated acute gout attacks.^2-5 These lesions develop in approximately 50% of gout patients and usually occur an average of 11.6 years after the onset of disease.² There is a subset of individuals that are at higher risk for developing tophi, including elderly and female patients, diuretic and chronic nonsteroidal anti-inflammatory drug users, patients with a history of cyclosporine therapy, and patients with underlying chronic renal insufficiency.^2,6,7 The most commonly affected tissues are those with poor blood supply and lower temperatures, such as the ear helix and first metacarpal joint.⁴ The auricle is the most common site of tophi on the head and neck. Tophi of the helices are generally asymptomatic and nontender; however, tophi can become large, inflamed, and ulcerated, causing pressure and discomfort.² Combination treatment with dietary modification and antihyperuricemic therapy (eg, allopurinol) has been shown to reduce the size of lesions and prevent future tophi formation. However, these results may take months, warranting excision of large and symptomatic lesions.^4,8

Our case is unusual in that the onset of the auricular lesions predated the articular gout by 6 months. Gouty tophi as the initial presentation of hyperuricemia is rare; however, tophi formation without concomitant arthritis has been reported.^2,3,7,9 Wernick et al⁷ described 6 patients presenting with tophi before the onset of inflammatory arthritis that they attributed to changes in active inflammation by age (eg, elderly patients were more commonly immunosuppressed), chronic illnesses, and anti-inflammatory medications (eg, nonsteroidal anti-inflammatory drugs). Another possible explanation for this atypical presentation is misdiagnosis caused by other forms of arthritis (eg, rheumatoid arthritis, osteoarthritis) masking acute gout episodes. It also has been reported that monosodium urate crystals can be found in synovial fluid with no inflammation and therefore no symptoms.⁷

Tophi, although rare, may be the sole clinical manifestation of underlying gouty disease. It is important to be aware of this atypical presentation to prevent misdiagnosis and provide appropriate treatment.

The Diagnosis: Bilateral Auricular Tophaceous Gout

Histopathologic evaluation with hematoxylin and eosin staining demonstrated clusters of abundant granular amorphous material within the subcutaneous tissue (Figure 1). The overlying epidermis and dermis were unremarkable. The granular amorphous material demonstrated numerous monosodium urate crystals under polarized light (Figure 2). At a return visit following the biopsy results, the patient reported a history of a single episode of monoarticular gouty arthritis involving the right hallux approximately 6 months after the onset of the skin lesions. With the added clinical history and the biopsy results, his serum uric acid level was obtained and was found to be elevated at 9.2 mg/dL (reference range, 3.5–8 mg/dL).

In our patient, the clinical differential diagnosis included calcium deposits, weathering nodules, and tophaceous gout. The differential diagnosis of auricular lesions is broad, and benign lesions may mimic cancerous entities such as basal cell carcinoma and squamous cell carcinoma.¹ Therefore a detailed history, thorough physical examination, and tissue sampling are key to establishing the correct diagnosis. Our patient’s history of monoarticular gouty arthritis was only elucidated after a diagnosis of bilateral auricular tophaceous gout was made based on the biopsy results.

Subcutaneous tophi represent a chronic state of hyperuricemia and tend to manifest after long-standing polyarthritis and repeated acute gout attacks.^2-5 These lesions develop in approximately 50% of gout patients and usually occur an average of 11.6 years after the onset of disease.² There is a subset of individuals that are at higher risk for developing tophi, including elderly and female patients, diuretic and chronic nonsteroidal anti-inflammatory drug users, patients with a history of cyclosporine therapy, and patients with underlying chronic renal insufficiency.^2,6,7 The most commonly affected tissues are those with poor blood supply and lower temperatures, such as the ear helix and first metacarpal joint.⁴ The auricle is the most common site of tophi on the head and neck. Tophi of the helices are generally asymptomatic and nontender; however, tophi can become large, inflamed, and ulcerated, causing pressure and discomfort.² Combination treatment with dietary modification and antihyperuricemic therapy (eg, allopurinol) has been shown to reduce the size of lesions and prevent future tophi formation. However, these results may take months, warranting excision of large and symptomatic lesions.^4,8

Our case is unusual in that the onset of the auricular lesions predated the articular gout by 6 months. Gouty tophi as the initial presentation of hyperuricemia is rare; however, tophi formation without concomitant arthritis has been reported.^2,3,7,9 Wernick et al⁷ described 6 patients presenting with tophi before the onset of inflammatory arthritis that they attributed to changes in active inflammation by age (eg, elderly patients were more commonly immunosuppressed), chronic illnesses, and anti-inflammatory medications (eg, nonsteroidal anti-inflammatory drugs). Another possible explanation for this atypical presentation is misdiagnosis caused by other forms of arthritis (eg, rheumatoid arthritis, osteoarthritis) masking acute gout episodes. It also has been reported that monosodium urate crystals can be found in synovial fluid with no inflammation and therefore no symptoms.⁷

Tophi, although rare, may be the sole clinical manifestation of underlying gouty disease. It is important to be aware of this atypical presentation to prevent misdiagnosis and provide appropriate treatment.