Histological variability and the importance of clinicopathological correlation in cutaneous Rosai-Dorfman disease.
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.
Coimbra University Hospital - Coimbra, Portugal.
Ana
A
Gameiro
Coimbra University Hospital - Coimbra, Portugal.
Miguel
M
Gouveia
Coimbra University Hospital - Coimbra, Portugal.
José Carlos
JC
Cardoso
Coimbra University Hospital - Coimbra, Portugal.
Oscar
O
Tellechea
D000328:Adult; D015703:Antigens, CD / Q000378:metabolism; D015214:Antigens, Differentiation, Myelomonocytic / Q000378:metabolism; D001132:Arm; D003937:Diagnosis, Differential; D005260:Female; D006644:Histiocytes / Q000473:pathology; D015618:Histiocytosis, Sinus / Q000473:pathology*; D006801:Humans; D007866:Leg; D009418:S100 Proteins / Q000378:metabolism; D012871:Skin Diseases / Q000473:pathology*; D000097382:CD68 Molecule
D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D009418:S100 Proteins; C067980:CD68 antigen, human; D000097382:CD68 Molecule