Febrile Ulceronecrotic Mucha‐habermann Disease: a Rare, Severe Variant

A 56 year old Hispanic man presented with extensive ulcerative skin lesions, involving his lower trunk, groin and upper legs, severe pain and a temperature of 38.7 degrees Celsius. He was admitted to the Medical Intensive Care Unit for empiric intravenous antibiotics. Several biopsies were performed. In the following days his condition worsened and ulcerative lesions involved nearly all of his skin. Previous biopsies were consistent with pityriasis lichenoides et varioliformis acuta (PLEVA), however, neither this, nor others in the histological differential diagnosis, fit his severe and worsening clinical picture. Histology revealed vacuolar alteration with dyskeratotic keratinocytes and a superficial perivascular mixed infiltrate of lymphocytes and eosinophils. There was confluent parakeratosis containing neutrophils, and a diminished granular layer with pallor in the upper portion of the spinous layer. Immunofluorescence studies were negative. These findings were consistent with PLEVA. A clinical diagnosis of febrile ulceronecrotic Mucha‐Habermann disease was made. Febrile ulceronecrotic Mucha‐Habermann disease is a rare and severe variant of PLEVA characterised by high fever and papulonecrotic skin lesions. Twenty‐five cases have been previously reported. We present the clinical and histological findings in this unusual clinical presentation.