A fatal case of febrile ulceronecrotic Mucha-Habermann disease in a child

Abstract

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare, potentially fatal, severe variant of pityriasis lichenoides et varioliformis acuta (PLEVA). Lesions of FUMHD usually start with erythematous papules and plaques that rapidly progress to form large, often coalescing necrotic ulcers with hemorrhagic crusts. The skin findings are typically accompanied by systemic symptoms including high fever, abdominal pain, diarrhea, arthritis, pulmonary involvement, central nervous system symptoms, and sepsis. These systemic manifestations can lead eventually to serious complications or even a fatal outcome.1, 2, 3 Fatal cases were confined to adults (9 deaths of about 70 reported cases). On the contrary, children with FUMHD tend to have a more favorable outcome than adults, and no deaths have so far been reported in children.2, 3 Here we report the first case, to our knowledge, of a child with FUMHD complicated by sepsis that resulted in multiple organ failure and death.