关键词: Cutaneous Cutáneo Rosai-Dorfman Cutaneous Cutáneo Rosai-Dorfman

Mesh : Female GTP Phosphohydrolases / genetics Histiocytes / pathology Histiocytosis, Sinus / diagnosis genetics pathology Humans Membrane Proteins Middle Aged Mutation Proto-Oncogene Proteins B-raf / genetics Proto-Oncogene Proteins p21(ras) / genetics

来  源:   DOI:10.1016/j.patol.2019.03.007

Abstract:
Non-Langerhans cell histiocytosis, including Rosai-Dorfman disease (RDD) and xanthogranuloma are rare disorders with occasional overlapping in the histopathological and immunohistochemical (IHC) findings. We report the case of a 53-year-old woman with erythematous-violaceous plaques on the cheeks and edema in the auricular pavilions. A biopsy was performed and the histopathological examination revealed a histiocytic proliferation with emperipolesis characteristic of RDD and lymphoplasmocitic infiltrate. IHC analysis showed S100 and CD68 positivity in the histiocytes but was negative for CD1a, supporting the diagnosis of RDD. Molecular analysis failed to detect BRAF-V600, NRAS or KRAS mutation. We discuss the differential diagnosis of cutaneous non-Langerhans cell histiocytosis. Pathologist must be aware of unusual presentations of RDD and further treatment options must be explored for patients with unresectable lesions and/or resistance to the classical management of RDD.
摘要:
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