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KMID : 0363220120500060565
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Korean Journal of Dermatology
2012 Volume.50 No. 6 p.565 ~ p.568
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Febrile Ulceronecotic Pityriasis Lichenoides et Varioliformis Acuta Successfully Treated with Oral Cyclosporin
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Yeom Kkot-Bora
Park Young-Woon
Jin Seon-Pil
Cho Hyun-Hee
Kim Kyu-Han
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Abstract
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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.
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KEYWORD
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Cyclosporine, Febrile ulceronecrotic, Mucha-Haberrman disease, Pityriasis lichenoides et varioliformis acuta
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