Febrile Ulceronecrotic Mucha-Habermann Disease: Treatment with Infliximab and Intravenous Immunoglobulins and Review of the Literature

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare subtype of pityria- sis lichenoides et varioliformis acuta, charac- terized by an acute onset of ulceronecrotic papules, rapidly coalescing into large ulcers with necrotic crusts, associated with high fe- ver and severe systemic symptoms. We re- port a case of a 65-year-old woman with a resistant form of FUMHD successfully treat- ed with a tumor necrosis factor-α (TNFα) in- hibitor (infliximab). After 1 year of treatment, because of the recurrence of lesions and occurrence of severe sepsis, we decided to change the therapeutic procedure by intro- ducing intravenous immunoglobulin witch induced a spectacular improvement. Only few cases of FUMHD treated with intrave- nous immunoglobulin have been reported to date. In our case, we describe the first uti- lization of TNFα inhibitors in the treatment of FUMHD: TNFα inhibitors may be useful, particularly in resistant cases. Further re- ports are required to confirm this potential therapeutic option.