Mucha-Habermann disease and its febrile ulceronecrotic variant

Abstract

In 1916 Mucha and in 1925 Habermann reported an acute form of pityriasis lichenoides characterized by the abrupt onset of papulovesicular eruptions and gave the name, pityriasis lichenoides et varioliformis acuta (PLEVA) or Mucha-Habermann disease (MH). In 1966, Degos reported a rare febrile ulceronecrotic variant of MH. MH occurs mainly in young adults, while febrile ulceronecrotic Mucha-Habermann's disease (FUMHD) occurs more frequently in children. The etiology of MH remains obscure, but it may be the result of a hypersensitivity reaction to an infectious agent. Although clinical and histologic features of the disease in children are similar to those of adults, more diseases need to be differentiated in pediatric patients. In addition, a number of effective therapeutic options in adults with MH are unsuitable for use in pediatric patients, to whom beginning with oral antibiotics, usually erythromycin, is recommended. A summary of previously reported fifteen cases with FUMHD, including our case, is listed.