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Febrile Ulceronecrotic Mucha‐Habermann Disease with Central Nervous System Vasculitis
Author(s) -
Rosman Ilana S.,
Liang LingChih,
Patil Sushama,
Bayliss Susan J.,
White Andrew J.
Publication year - 2012
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.2011.01669.x
Subject(s) - medicine , vasculitis , brain biopsy , cyclophosphamide , necrotizing vasculitis , fibrinoid necrosis , disease , dermatology , biopsy , pathology , surgery , chemotherapy
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.