Febrile ulceronecrotic Mucha-Habermann disease: a case report and a review of the literature

Abstract

We report a case of febrile ulceronecrotic Mucha-Habermann disease (FUMHD) in a 27-year-old woman. After 20 days of a mild eruption, extensive polymorphous, papular and ulcerohemorrhagic skin lesions gradually developed, associated with intermittent high temperature, and constitutional symptoms. The initial treatment with acyclovir was not successful, the skin lesions still progressed distally and individual lesions evolved from necrotic papules and bullae to erosions and ulcers. Skin biopsies showed the typical histopathological changes of PLEVA. The patient was treated with systemic prednisolone but dosage was limited in order to avoid sepsis. Despite corticosteroid therapy and supportive therapy, the fulminating course led to death. Including this present case, only 31 cases of FUMHD have been reported in English literature. Our case is the second report from Turkey.