Febrile Ulceronecrotic Mucha-Habermann Disease: A Rare Case Report and Review of Cases Treated With Oral Cyclosporine

Abstract

We present a case of febrile ulceronecrotic Mucha-Habermann disease who presented with widespread erythematous crusted papules, which rapidly progressed to ulceronecrotic lesions accompanied by fever. The serologies showed high titers of cytomegalovirus IgG (>1:3,200) and IgM (1:800). The histopathological study showed epidermal necrosis. The treatment was begun with systemic steroids and roxithromycin to which the patient did not respond. After switching the treatment to methotrexate, the patient was further complicated by getting hepatitis. Low dose cyclosporine resolved the situation within 2 weeks. In patients for whom methotrexate is contra-indicated or ineffective, cyclosporine can suppress T-lymphocyte hyperresponsiveness and resolve this disease.

Keywords

• cyclosporine
• cytomegalovirus
• febrile ulceronecrotic mucha-habermann disease
• pityriasis lichenoides with ulceronecrosis and hyperthermia