Tanya Greywal, MD, Andrea L. Zaenglein, MD (co-chair), Hilary E

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Evidence-based recommendations for the management of acne fulminans and its variants Tanya Greywal, MD, Andrea L. Zaenglein, MD (co-chair), Hilary E. Baldwin, MD, Neal Bhatia, MD, Karen A. Chernoff, MD, James Q. Del Rosso, DO, Lawrence F. Eichenfield, MD, Marc H. Levin, MD, PhD, James J. Leyden, MD, Diane M. Thiboutot, MD, Guy F. Webster, MD, PhD, Sheila Fallon Friedlander, MD (co-chair) Journal of the American Academy of Dermatology Volume 77, Issue 1, Pages 109-117 (July 2017) DOI: 10.1016/j.jaad.2016.11.028 Copyright © 2016 American Academy of Dermatology, Inc. Terms and Conditions

Fig 1 Representative patients with acne fulminans and their management. A and B, 14-year-old boy with papules, pustules, hemorrhagic crust, and scars on the face (A) and back (B). He did not have fever but did present with bone pain and leukocytosis (white blood cell count 18 cells/μL), thrombocytosis (468 platelets/μL), and elevated C-reactive protein (5.7 mg/L). Therefore, his diagnosis was consistent with acne fulminans with systemic symptoms (AF-SS). He was treated with prednisone 1 mg/kg monotherapy for 4 weeks, and then isotretinoin 0.1 mg/kg was added to his regimen for the subsequent 4 weeks. The patient showed dramatic improvement, allowing prednisone to be tapered with slow, incremental increases in his isotretinoin dosage. C-E, 12-year-old boy with inflammatory papules, pustules, and hemorrhagic crusts on the face (C), chest, and back (D). The lesions developed 4 weeks after initiating isotretinoin 30 mg/day with no systemic symptoms present. This patient was diagnosed with isotretinoin-induced acne fulminans without systemic symptoms (IIAF-WOSS), and isotretinoin was promptly discontinued. He received 2 weeks of prednisone 1 mg/kg monotherapy, at which time the hemorrhagic crusts and erosions had resolved (E). Isotretinoin 0.1 mg/kg was started and continued along with the prednisone 1 mg/kg for the subsequent 4 weeks. The patient was slowly tapered off of prednisone as he continued isotretinoin 0.1 mg/kg dose for another 4 weeks and until he was able to tolerate slowly increasing the dose of isotretinoin. His full course of isotretinoin therapy lasted 26 months with a final cumulative dose of 130 mg/kg. F, 16-year-old boy with papules, pustules, and scarring on the face, which is consistent with severe inflammatory acne. To prevent IIAF, the patient was administered prednisone 0.5 mg/kg for 2 weeks before initiating isotretinoin. He was then started on low-dose isotretinoin and did not experience a dramatic flare of inflammatory acne with crusting, erosions, or systemic symptoms. © American Acne & Rosacea Society. Printed with permission. Journal of the American Academy of Dermatology 2017 77, 109-117DOI: (10.1016/j.jaad.2016.11.028) Copyright © 2016 American Academy of Dermatology, Inc. Terms and Conditions

Fig 2 Consensus classification and treatment algorithm of acne fulminans and its variants. © American Acne & Rosacea Society. Printed with permission. Journal of the American Academy of Dermatology 2017 77, 109-117DOI: (10.1016/j.jaad.2016.11.028) Copyright © 2016 American Academy of Dermatology, Inc. Terms and Conditions