CASE REPORT Febrile Ulceronecrotic Mucha-Habermann Disease

Objective: Case report and review of the current literature about febrile ulceronecrotic Mucha-Habermann disease (FUMHD). Methods: Review of our patient's medical records and of the current literature. Results: The FUMHD is a rare and potentially lethal type of pityriasis lichenoides et varioliformis acuta. It is characterized by the sudden onset of ulceronecrotic skin lesions associated with high fever and systemic symptoms. Because of a high case-fatality rate it requires quick and decisive action. Only 40 cases of this severe form of the disease have been reported in the literature to date. We present the case of a 30-year-old male patient with severe FUMHD who was successfully treated in our burn intensive care unit after failed treatment at a dermatological hospital. The patient was treated with topical antiseptics, moisturizers, and artificial skin substitutes, as well as systemic immunosuppressive therapy (glucocorticoids) with which we were able to control the disease activity so that healing of the patient's skin lesions could be achieved. Conclusion: Patients with FUMHD should be treated in a specialized center for severely burned patients. Only such centers can provide the structural and logistical capacities necessary for the treatment of such extensive superficial wounds.