Anais Brasileiros de Dermatologia (Oct 2016)

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

  • Ana Gameiro,
  • Miguel Gouveia,
  • José Carlos Cardoso,
  • Oscar Tellechea

DOI
https://doi.org/10.1590/abd1806-4841.20164477
Journal volume & issue
Vol. 91, no. 5
pp. 634 – 637

Abstract

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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.

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