Severe Febrile Mucha-Habermann''s Disease in Children: Case Report and Review of the Literature

Mucha-Habermann disease, or pityriasis lichenoides et varioliformis acuta, is usually a benign, papulosquamous, cutaneous disorder. It has also been reported in a severe form with fever and systemic symptoms both in children and adults. We report a 12-year-old boy with the febrile, ulceronecrotic type. A review of similar cases in the literature shows a 16% frequency of acute necrotic lesions, as well as rare complications such as fever, superinfected lesions, bacteremia (most often with Staphylococcus aureus), and rheumatologic manifestations such as arthritis and scleroderma. There is no definitive treatment, but tetracycline, erythromycin, methotrexate, and ultraviolet light are used most frequently. The most common histologic feature is mononuclear perivascular infiltrates. Mucha-Habermann disease can mimic other common entities such as varicella and insect bites.