Cutaneous Rosai-Dorfman Disease (RDD) Mimicking Primary Cutaneous Marginal Zone B-cell Lymphoma / 대한피부과학회지

ABSTRACT

Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is an idiopathic, benign, histiocytic proliferative disease involving lymph nodes and extranodal sites. A 52-year-old woman presented with a 2-year history of multiple non-tender, erythematous nodules on her face. The initial punch biopsy showed lymphoid follicle-like aggregation of CD20 positive lymphocytes in the papillary dermis consisting of primary cutaneous marginal zone B-cell lymphoma. She underwent a 3-month course of radiotherapy, but the lesions progressed. The re-biopsy specimen revealed marked histiocytes with positive CD68 and S-100 proteins. The patient was treated with prednisolone and isotretinoin to which she responded slightly. Repeated histopathologic examinations enable us to make a definite diagnosis. Differentiating these two disorders as early as possible is crucial for appropriate treatment.