title: Febrile ulceronecrotic Mucha-Habermann disease with central nervous system vasculitis.
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.
authors.affiliation: Division of Dermatology, Department of Internal Medicine, School of Medicine Washington University, and St. Louis Children's Hospital, St. Louis, Missouri 63110, USA.
authors.forename: Ilana S
authors.initials: IS
authors.lastname: Rosman
authors.forename: Ling-Chih
authors.initials: LC
authors.lastname: Liang
authors.forename: Sushama
authors.initials: S
authors.lastname: Patil
authors.forename: Susan J
authors.initials: SJ
authors.lastname: Bayliss
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: White
mesh_terms: D001707:Biopsy, Needle; D002648:Child; D018450:Disease Progression; D004359:Drug Therapy, Combination; D006561:Herpes Simplex / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008775:Methylprednisolone / Q000627:therapeutic use; D008991:Monitoring, Physiologic; D017514:Pityriasis Lichenoides / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology*; D011379:Prognosis; D035583:Rare Diseases; D018570:Risk Assessment; D012720:Severity of Illness Index; D017211:Treatment Failure; D020293:Vasculitis, Central Nervous System / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology*
chemical_list: D008775:Methylprednisolone; D008727:Methotrexate