Korean J Dermatol.  2012 Jun;50(6):565-568.

Febrile Ulceronecotic Pityriasis Lichenoides et Varioliformis Acuta Successfully Treated with Oral Cyclosporin

• Yeom KB
• Park YW
• Jin SP
• Cho HH
• Kim KH

Abstract

Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta (PLEVA), or febrile ulcerative Mucha-Haberman disease (FUMHD) is very rare, but potentially lethal variants of PLEVA. This subtype is characterized by rapidly progressive ulceronecrotic lesions and systemic manifestations, such as high fever, gastrointestinal, neurological, cardiologic and pulmonary involvement, and rheumatologic manifestations. Several treatments with variable response have been challenged, such as systemic steroid, antibiotics, methotrexate, dapsone, cyclosporine and ultraviolet therapy. But there is no standard therapy for FUMHD to date. We report a 59-year-old woman with FUMHD, who recurred after discontinuation of systemic steroid, but successfully treated with oral cyclosporine.

Keyword

Cyclosporine; Febrile ulceronecrotic; Mucha-Haberrman disease; Pityriasis lichenoides et varioliformis acuta

MeSH Terms

Anti-Bacterial Agents
Cyclosporine
Dapsone
Female
Fever
Humans
Methotrexate
Middle Aged
Pityriasis
Pityriasis Lichenoides
Ulcer
Ultraviolet Therapy
Anti-Bacterial Agents
Cyclosporine
Dapsone
Methotrexate