A Case of Rosai-Dorfman Disease with Chronic Skin Ulceration as a Cutaneous Symptom

Abstract

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic disorder characterized by lymph node invasion and extranodal symptoms. The number of patients reported heretofore is less than 1,000, and it shows a predilection to certain groups: younger age, African race, and male sex. Herein, we report a middle-aged Asian female who presented to us with a single inflamed skin nodule on her left cheek, which finally concluded as an extranodal symptom of RDD. At first, the skin lesion was believed to be an inflamed epidermal cyst. Though excision was performed, it recurred as a chronic skin ulcer. As the lesion did not heal well despite 3 months of repetitive dressings, wide excision and curettage were performed. Immunohistochemistry study of the excised specimen diagnosed RDD. Six months after the last excision, there was no recurrence or complications. This report offers the possibility of RDD in patients showing chronic skin ulcers being accompanied by lymphadenopathy, and also suggests a surgical excision as a preferred option in skin manifestation of RDD.