Febrile ulceronecrotic Mucha-Habermann disease is a severe variant of pityriasis lichenoides et varioliformis acuta characterized by the sudden onset of ulceronecrotic skin lesions and associated with high fever and systemic symptoms. We report here a case of a 20-year-old woman in whom the disease started as pityriasis lichenoides et varioliformis acuta and evolved to febrile ulceronecrotic Mucha-Habermann disease. Almost 90% of the body surface was involved, together with high fever and malaise. Steroids alone proved to be an insufficient therapeutic procedure. The remission achieved was attributed to the use of methotrexate. To our knowledge, only 39 cases of febrile ulceronecrotic Mucha-Habermann disease have been reported in the literature to date.
"No recurrence has been shown with high doses of corticosteroids and antibiotics in the first weeks (complete resolution of the lesions) and maintenance treatment for over 2–6 months with half doses of antibiotics . Because of the severity of disease in the present study and in an effort to avoid recurrences, the patient was treated with tetracycline at a dose of 2 g/day until the end of the first month and 1 g/day for 2 more months. "
[Show abstract][Hide abstract] ABSTRACT: A male dark-skinned patient, 33 years old, complaining of a 1-month history of skin lesions in the abdominal region and high fever was admitted to the clinical ward. There were ulcerated and coalescent skin lesions all over the body but sparing the palmar region. The culture of material obtained from the penile lesions was positive for . He was treated with oxacillin and prednisone. The patient persisted with high fever and skin lesions. After histopathological diagnosis of Mucha Habermann's disease, treatment was started with tetracycline with rapid improvement of the lesions. He was reexamined by the dermatologic service with no recurrences during the 12 months of follow-up. At this time, there was a great number of keloid scars from the skin lesions.
Case Reports in Dermatology 01/2013; 5(1):4-10. DOI:10.1159/000346742
[Show abstract][Hide abstract] ABSTRACT: We report a case of febrile ulceronecrotic Mucha-Habermann disease (FUMHD) in a 12-year-old boy. After 1 week of typical cutaneous appearance of pityriasis lichenoides et varioliformis acuta (PLEVA), he was given a percutaneous injection of measles virus vaccine, and then extensive polymorphous, papular, and ulcerohemorrhagic skin lesions developed along with intermittent high temperature, hepatic dysfunction, and hypoalbuminemia. Thoracic CT scan showed parenchymal nodular infiltration at the tip of vascular structures. Skin biopsy showed a combination of the classic features of PLEVA and an allergic vasculitis. We treated him with prednisolone and methotrexate (MTX) successfully. The evolution in our patient suggests that FUMHD may have been triggered by virus vaccination. We also emphasize that FUMHD in children may be successfully treated with a combination of systemic MTX and high-dose corticosteroids.
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