큰종기와 유사한 임상 소견을 보인 피부에 국한된 다발성 Rosai-Dorfman Disease 1예

Abstract

Rosai-Dorfman disease (RDD) is a rare and non-neoplastic histiocytic proliferative disorder, whose most common clinical presentation is massive but painless cervical lymphadenopathy. The cutaneous form of RDD is rare, and usually presents as erythematous to brown papules, plaques, or nodules. Most lesions are located on the face and trunk. We herein describe the case of a 52-year-old female patient with RDD clinically limited to the skin. The patient presented with asymptomatic multiple erythematous inflammatory nodules on the right flank and nasal area. Histopathologic findings showed multiple foamy histiocytic infiltrations in the dermis. On immunohistochemistry, the lesions were positive for CD68 and S-100, and negative for CD1a. The patient was treated with oral methylprednisolone, prednicarbate ointment and intralesional injection of triamcinolone acetonide. After 3 months, the lesions achieved almost complete remission, and the patient has been under observation without recurrence for the past 1 year