Febrile Ulceronecrotic Mucha-Habermann Disease

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD), first reported by Degos et al in 1966, is a rare subtype of pityriasis lichenoides et varioliformis acuta. It consists of an acute and severe generalized lesion of purpuric and ulceronecrotic papulonodules and/or plaques associated with systemic involvement. Cutaneous lesions always are present prior to the acute fulminating course. According to a search using PubMed for articles indexed for MEDLINE and the Chinese National Knowledge Infrastructure for content written in Chinese using the term febrile ulceronecrotic Mucha-Habermann disease, 62 cases, including 1 boy, have been reported, with 9 reported deaths in adults. The disease has no ethnic or geographic dispositions. Herein, a rare case of FUMHD recurring after initial therapy with methylprednisolone is presented; the patient was successfully treated with methylprednisolone combined with methotrexate sodium.