P592 : Pemphigus vegetans showing complete remission to dapsone therapy

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발행기관 : 대한피부과학회 수록지정보 : 프로그램북(구 초록집) / 67권 / 1호
저자명 : ( Ji Young Choi ) , ( Soo Chan Kim )

영어 초록

Pemphigus vegetans is a rare form of pemphigus characterized by flaccid bullae and pustules that erode to form hypertrophic plaques. Systemic corticosteroids are used as a first-line therapy but they do not always induce good clinical response. Here, we report a case of pemphigus vegetans who was refractory to systemic corticosteroids, successfully treated with dapsone. A 46-year-old male patient presented with a 3-year history of hyperkeratotic, scaly plaques with erosion on the face and superficial bullae on the whole body. The skin biopsy result (done at the previous clinic) revealed irregular acanthosis, intraepithelial acantholysis and intraepidermal eosinophilic pustule. ELISA showed positive for anti-desmoglein 1 (190.4), but negative foranti-desmoglein 3 (4.1). The patient was treated with combination of systemic corticosteroid and cyclosporin, but the result was not remarkable. Subsequently, treatment with dapsone 50mg daily and oral methylprednisolone 6mg daily was started. Partial improvement was observed after 2 weeks of treatment, and the lesions disappeared completely after 4 weeks of treatment leaving only postinflammatory hyperpigmentation. The complete remission has been maintained with low dose of dapsone (25mg/d) and methylprednisolone (2mg/d). From this case, we propose that dapsone can be a useful treatment option in patients with pemphigus vegetans, who are refractory to systemic corticosteroids therapy alone.

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P592 : Pemphigus vegetans showing complete remission to dapsone therapy