FEBRILE ULCERONECROTIC MUCHA-HABERMANN DISEASE

Febrile ulceronecrotic Mucha-Habermann disease in an 18-year-old man i s reported. This disease is a severe form of pityriasis lichenoides et varioliformis acuta (PLEVA) and is characterized by the sudden onset of diffuse coalescent ulcerations associated with high fever and syste mic symptoms. In the present case the disease was preceded by typical PLEVA. Histologically, a leukocytoclastic vasculitis was seen in addit ion to the usual features of PLEVA. Findings of laboratory studies rev ealed an elevated erythrocyte sedimentation rate, a high white blood c ell count, and a mild increase in liver enzymes. No systemic involveme nt was detected. Findings of T cell receptor gene analysis in skin and peripheral blood showed no abnormality. The patient was treated with PUVA and methotrexate with a good response. We review the eight previo usly reported cases of febrile ulceronecrotic Mucha-Habermann disease.