A case of febrile ulceronecrotic Mucha-Habermann disease with comorbidities

Febrile ulceronecrotic Mucha-Habermann disease is a very rare and severe variant of pityriasis lichenoides et varioliformis acuta. Adult cases are difficult to diagnose as in the early course they can mimic erythema multiforme or lymphomatoid papulosis. We report a case of a 38-year-old woman who presented with 90% body surface area involvement, fever, diarrhea, malaise and associated comorbidities. She was treated with systemic steroids and methotrexate but suffered a fatal outcome. So far, a total of 65 cases are reported in the literature.