Febrile Ulceronecotic Pityriasis Lichenoides et Varioliformis Acuta Successfully Treated with Oral Cyclosporin.
- Author:
Kkot Bora YEOM
1
;
Young Woon PARK
;
Seon Pil JIN
;
Hyun Hee CHO
;
Kyu Han KIM
Author Information
1. Department of Dermatology, Seoul National University College of Medicine, Seoul, Korea. kyuhkim@snu.ac.kr
- Publication Type:Case Report
- Keywords:
Cyclosporine;
Febrile ulceronecrotic;
Mucha-Haberrman disease;
Pityriasis lichenoides et varioliformis acuta
- MeSH:
Anti-Bacterial Agents;
Cyclosporine;
Dapsone;
Female;
Fever;
Humans;
Methotrexate;
Middle Aged;
Pityriasis;
Pityriasis Lichenoides;
Ulcer;
Ultraviolet Therapy
- From:Korean Journal of Dermatology
2012;50(6):565-568
- CountryRepublic of Korea
- Language:English
-
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.
