Histological variability and the importance of clinicopathological correlation in cutaneous Rosai-Dorfman disease

• Author(s): Gameiro,Ana ; Gouveia,Miguel ; Cardoso,José Carlos ; Tellechea,Oscar
• Date: 2016
• Origin: Oasisbr
• Subject(s): Emperipolesis; Histiocytosis; Histiocytosis, sinus; Thalidomide
• Document Type: Report
• Language: English

Description

Abstract: Rosai-Dorfman disease is a benign histiocytic proliferative disorder of unknown etiology. The disease mainly affects lymph node tissue, although it is rarely confined to the skin. Here, we describe a 53-year-old woman with purely cutaneous Rosai-Dorfman disease. The patient presented with a large pigmented plaque on her left leg, and sparse erythematous papules on her face and arms. A complete clinical response was achieved with thalidomide, followed by recurrence at the initial site one year later. The histological examination displayed the typical features of Rosai-Dorfman disease in the recent lesions but not in the older lesions. In the setting of no lymphadenopathy, the histopathological features of Rosai-Dorfman disease are commonly misinterpreted. Therefore, awareness of the histological aspects present at different stages, not always featuring the hallmark microscopic signs of Rosai-Dorfman disease, is particularly important for a correct diagnosis of this rare disorder.