Febrile Ulceronecrotic Mucha-Habermann Disease in a Young Boy: A Case Report and Review of the Literature

Abstract

© 2014 The Authors. doi: 10.2340/00015555-1791 Journal Compilation © 2014 Acta Dermato-Venereologica. ISSN 0001-5555 Pityriasis lichenoides et varioriformis acuta (PLEVA), also known as Mucha-Habermann disease, is an uncommon, idiopathic, acquired dermatosis characterised by erythematous and scaly papules that are often accompanied by haemorrhagic and papulonecrotic lesions. The patient’s general health is usually not affected. Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare variant of PLEVA that was first described by Degos et al. in 1966 (1). FUMHD is more destructive and is associated with high fever, systemic symptoms and the development of large coalescent skin necroses which may lead to death. Here we report a young boy with FUMHD successfully treated with systemic corticosteroid.