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Published on: February 8, 2019
Febrile ulceronecrotic Mucha-Habermann disease with central nervous system vasculitis
Ilana S Rosman1, Ling-Chih Liang, Sushama Patil
1Division of Dermatology, Department of Internal Medicine, School of Medicine Washington University, and St. Louis Children's Hospital, St. Louis, Missouri 63110, USA.
Abstract:
Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a severe variant of pityriasis lichenoides et varioliformis acuta (PLEVA). We report a case of FUMHD in an 11-year-old boy who subsequently developed neurologic symptoms and was found to have necrotizing vasculitis on brain biopsy. Over 5 years of follow-up, he had one biopsy-proven recurrence of his skin lesions and continued rehabilitation and treatment for residual neurologic complications. This case provides histological evidence of central nervous system vasculitis associated with FUMHD. Our patient's disease was eventually controlled with cyclophosphamide.
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