Abstract
Febrile Ulceronecrotic Mucha-Habermann disease is a rare and potentially fatal variant of pityriasis lichenoides et varioliformis acuta and is characterized by high fever, constitutional symptoms, and acute oncet of ulceronecrotic lesions. We present an 11-year-old male with Febrile Ulceronecrotic Mucha-Habermann disease who was cured with methotrexate and review the use of methotrexate for this disorder in the pediatric age group with the relevant literature.
Highlights
Pityriasis lichenoides et varioliformis acuta (PLEVA) is a rare idiopathic dermatosis
Febrile Ulceronecrotic MuchaHabermann disease (FUMHD), first defined by Degos et al in 1966, is a severe variant of PLEVA that is characterized by destructive ulceronecrotic lesions and frequently accompanied by systemic findings [1]
PUBMED data reveal that only a limited number of pediatric FUMHD cases have been reported, and there is no clear treatment algorithm in this disorder
Summary
Pityriasis lichenoides et varioliformis acuta (PLEVA) is a rare idiopathic dermatosis. Febrile Ulceronecrotic MuchaHabermann disease (FUMHD), first defined by Degos et al in 1966, is a severe variant of PLEVA that is characterized by destructive ulceronecrotic lesions and frequently accompanied by systemic findings [1]. Oral antibiotics (erythromycin, tetracycline), phototherapy, and immunosuppressive agents are used for treatment but the results are mostly in the form of case reports. Methotrexate treatment has been reported as an effective option in a few cases in the literature. We report here an 11-year-old boy with FUMHD who was treated with methotrexate
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