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A pure Cutaneous Rosai-Dorfman disease: case report and a review of the literature
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Fatemeh Montazer    , Saeedeh Farahani , Zoha Shaka , Zeinab Aryanian , Azadeh Goodarzi |
| Department of Dermatology, Rasool Akram Medical Complex, Iran University of Medical Sciences, Tehran, Iran , azadeh_goodarzi1984@yahoo.com |
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Abstract: (2450 Views) |
Background: Cutaneous Rosai-Dorfman disease (CRDD) is a rare variant of benign histiocytic proliferative disorder limited to the skin. The underlying etiology is still unclear, but it had been claimed that infections, immunodeficiencies, and autoimmune disorders might have a role in the etiology of this disorder. The characteristic presentation of RDD is lymphadenopathy due to abnormal production and accumulation of histiocytes in lymph nodes; however, the extra-nodal areas could also be affected, such as cutaneous. Herein, we presented a 45-year-old Iranian woman presented with an atypical pure cutaneous Rosai Dorfman disease, in addition to a summarized list of atypical cases of RDD that are reported as pure cutaneous RDD with atypical presentation.
Case presentation: Herein, we presented a 45-year-old woman who referred to us with an ulcerative nodule with a size of 5×5 cm on her buttock, gradually growing over one year. After the primary evaluations, a biopsy specimen was obtained, and histologic studies revealed a dense cellular infiltrate involving the dermis and the subcutis, which was composed of abundant sheets of large histiocytes with admixtures of lymphocytes, plasma cells, neutrophils, and eosinophils within their cytoplasm -known as the emperipolesis phenomenon. The immunohistochemistry staining was positive for S100 and CD68 and negative for CD1a.
Conclusion: The diagnosis of CRDD was confirmed based on these histopathological findings.
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| Keywords: pure cutaneous Rosai Dorfman disease, histiocytosis, atypical cutaneous presentation, non-Langerhans cell histiocytosis, review, case report, histopathology |
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Full-Text [PDF 322 kb]
(597 Downloads)
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Type of Study: Review Article |
Subject:
Dermathology
Received: 2021/05/29 | Accepted: 2021/11/16 | Published: 2022/09/2
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