Febrile Ulceronecrotic Mucha-Habermann Disease with Central Nervous System Vasculitis
Article first published online: 26 JAN 2012
© 2012 Wiley Periodicals, Inc.
Volume 30, Issue 1, pages 90–93, January/February 2013
How to Cite
Rosman, I. S., Liang, L.-C., Patil, S., Bayliss, S. J. and White, A. J. (2013), Febrile Ulceronecrotic Mucha-Habermann Disease with Central Nervous System Vasculitis. Pediatric Dermatology, 30: 90–93. doi: 10.1111/j.1525-1470.2011.01669.x
- Issue published online: 15 JAN 2013
- Article first published online: 26 JAN 2012
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.