SCOTTSDALE, ARIZ. – Patients with acne fulminans should first begin corticosteroid monotherapy before adding isotretinoin, according to the first evidence-based consensus recommendations on this disease.
Antibiotics should not be used as first line treatment or monotherapy for acne fulminans, according to the experts who drafted the recommendations. Acne fulminans is an uncommon, understudied, and severe disorder that “typically manifests as an explosive worsening and ulceration of skin lesions, and can be associated with fever, bone pain, and other systemic symptoms,” noted panel co-chairs Dr. Andrea Zaenglein, professor of dermatology and pediatric dermatology, Pennsylvania State University, Hershey, and Dr. Sheila Friedlander, professor of medicine and pediatrics, University of California, San Diego, together with their associates.
The recommendations – based on a full literature review, a 5-hour audioconference, and two rounds of surveys to achieve consensus on these topics – were summarized in a poster presented at the annual meeting of the Society for Investigative Dermatology.
Until now, there have been no clear guidelines on the pathogenesis, treatment, and prevention of acne fulminans, according to the panelists.
“Acne fulminans is not just an extreme form of acne, but rather, a distinct, likely auto-inflammatory disorder,” the experts stated. Affected patients typically have an “abrupt, dramatic flare of inflammatory acne, with erosions, and with or without crusts, hemorrhagic nodules/plaques, and systemic findings.”
Systemic involvement is uncommon, but when present, includes fever, malaise, bone pain, arthralgias, erythema nodosum, and leukocytosis, they noted. Some patients also have anemia, an elevated erythrocyte sedimentation rate, and an increased C-reactive protein level. Radiography typically reveals osteolytic lesions of the sternum, clavicles, sacroiliac joints, and hips.
Acne fulminans is most often triggered by isotretinoin therapy, but can occur without it, the panel said. Isotretinoin-induced acne fulminans can have systemic involvement, but usually does not.
Patients should start corticosteroid monotherapy at a dose of 0.5 to 1.0 mg per kg per day, according to the recommendations. Patients with systemic involvement should receive steroids for at least 4 weeks, and other patients should continue steroids for at least 2 weeks and until all lesions have healed.
Oral corticosteroids should be tapered slowly over about 4 to 8 weeks, first by halving the dose to a physiologic dose each week, and then by dosing every other day for 2 weeks. Topical corticosteroids also can be used for eroded sites with granulation tissue, they noted.
Ironically, isotretinoin is both a treatment and a potential trigger of acne fulminans, and the recommendations included detailed guidance on its use.
Patients should wait at least 2 weeks after crusting resolves before starting isotretinoin, and should overlap isotretinoin with corticosteroids for at least 4 weeks, the experts emphasized. They recommended starting isotretinoin at 0.1 mg per kg per day, and waiting at least 2 months to increase this dose. Because patients clear at different rates, there is no universal optimal cumulative dose of isotretinoin, they noted.
If patients on isotretinoin develop flare, crusts, and erosions, they should halt treatment and either start corticosteroids, or increase the steroid dose to 1.0 mg per kg. If crusts and erosions persist, the panelists recommended considering cyclosporine, biologics, or dapsone.
If hemorrhagic crusts or erosions resolve after stopping isotretinoin, it can be restarted at the initial dose of 0.1 mg per kg and overlapped with steroids for 4 weeks.
If flares, crusts, and erosions begin when patients on isotretinoin are tapering corticosteroids, then steroids should be continued without tapering, isotretinoin should be stopped temporarily, and it should be restarted at 0.1 mg per kg after crusts and erosions have healed. This dose should be continued for 4 weeks, and then slowly increased as tolerated.
Antibiotics are not useful as monotherapy for first-line therapy for acne fulminans, according to the recommendations. But to avoid isotretinoin-induced acne fulminans, the experts often pretreat patients with oral antibiotics before starting isotretinoin, and may continue oral antibiotics during the initial phase of isotretinoin treatment. However, there have been no prospective studies supporting this approach, they noted.
Other treatment considerations
Acne fulminans lesions should not be debrided, the experts emphasized. Acne fulminans associated with SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis), PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne), and PAPASH (pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa) has responded successfully to tumor necrosis factor–alpha inhibitors and interleukin-1 receptor antagonists, they noted. Pulsed dye laser also has been used to successfully treat acne fulminans, particularly when there is associated granulation tissue, they stated.
Reducing the risk of pseudotumor cerebri syndrome
Several drugs used to treat acne fulminans have been linked to pseudotumor cerebri syndrome, the experts cautioned. “Among the tetracyclines, minocycline carries the highest risk,” they noted. Both isotretinoin and corticosteroids have been linked to pseudotumor cerebri syndrome, but clinicians can reduce this risk by avoiding an abrupt steroid taper, they emphasized.