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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Febrile ulceronecrotic Mucha-Habermann disease - a case and treatment review.
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Departamento de Medicina, Servico de Medicina Interna, Unidade Local de Saude de Matosinhos EPE, Portugal.
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A case report of febrile ulceronecrotic Mucha-Habermann disease.
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Department of Pediatric Critical Care Medicine, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
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- - FUMHD is a rare skin condition characterized by rapid onset of painful necrotic lesions, systemic symptoms like fever, and a complex diagnosis that requires ruling out other severe skin diseases.
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Department of Dermatology, Columbia University Irving Medical Center, New York, NY.
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Febrile Ulceronecrotic Mucha-Habermann Disease: A Case Report and Review of Literature in the Paediatric Population.
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Department of Dermatology, Affiliated Hangzhou First People's Hospital, Zhejiang University School of Medicine, Hangzhou, China.
Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare fulminant variant of pityriasis lichenoides et varioliformis acuta (PLEVA) that is characterized by a large ulceronecrotic appearance with high fever and a variety of systemic symptoms. We report here a case of FUMHD in a 17-year-old male Chinese patient who was treated successfully with a combination therapy of methotrexate, methylprednisolone, and intravenous immunoglobulin. In addition, a literature review was conducted to summarize the key characteristics of paediatric FUMHD cases.
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