Rosai-Dorfman Disease with Massive Cutaneous Nodule on the Shoulder and Back

Rosai-Dorfman Disease with Massive Cutaneous Nodule on the Shoulder and Back

Rosai-Dorfman disease is a rare, idiopathic, benign, and self-limiting histiocytic proliferative disorder. A 26-year-old man presented with a single massive cutaneous nodule (reaching 30 cm in diameter) on the left shoulder and back for 15 months. The routine hematological and biochemical tests were...

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Main Authors: Ma, Han, Zheng, Yue, Zhu, Guoxing, Wu, Jie, Lu, Chun, Lai, Wei
Format: Online
Language:English
Published: Korean Dermatological Association; The Korean Society for Investigative Dermatology 2015
Online Access:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4323606/
id pubmed-4323606
recordtype oai_dc
spelling pubmed-43236062015-02-11 Rosai-Dorfman Disease with Massive Cutaneous Nodule on the Shoulder and Back Ma, Han Zheng, Yue Zhu, Guoxing Wu, Jie Lu, Chun Lai, Wei Case Report Rosai-Dorfman disease is a rare, idiopathic, benign, and self-limiting histiocytic proliferative disorder. A 26-year-old man presented with a single massive cutaneous nodule (reaching 30 cm in diameter) on the left shoulder and back for 15 months. The routine hematological and biochemical tests were normal. Magnetic resonance scanning showed the lesion involved the skin, subcutaneous tissue, and subjacent muscle group, accompanied by obvious lymph node enlargement in the left part of the neck, supraclavicular fossa, and axillary fossa. The histopathology of the left cervical lymph node revealed diffuse effacement of the normal nodal architecture, with patchy chronic inflammatory cell infiltrates comprising lymphocytes and sheets of histiocytes. Some histiocytes contained lymphocytes within their pale cytoplasm. Many multinucleated giant cells were found; however, caseating granulomas were not seen. The skin and muscle biopsy specimen obtained from the back revealed infiltrating lymphocytes and histiocytes diffusely distributed in the dermis, subcutaneous tissue, and crevices of the muscle fibers. The phenomenon of emperipolesis and the presence of multinucleated giant cells were also seen. Immunohistochemical staining revealed that the histiocytes were positive for S-100 protein and CD68 but negative for CD1a. Immunophenotyping of the infiltrating lymphocytes indicated positive reactions to CD3, CD45RO, CD5, CD7, CD4, CD8 (partly), CD79a, CD20 (partly), and Ki-67 (<1%). The final diagnosis was Rosai-Dorfman disease. Owing to the extensive and deep involvement of the subcutaneous tissue and muscles, the patient did not undergo surgery to excise the massive skin nodule. The lesion showed no obvious change at the 12-month follow-up. Korean Dermatological Association; The Korean Society for Investigative Dermatology 2015-02 2015-02-03 /pmc/articles/PMC4323606/ /pubmed/25673935 http://dx.doi.org/10.5021/ad.2015.27.1.71 Text en Copyright © 2015 The Korean Dermatological Association and The Korean Society for Investigative Dermatology http://creativecommons.org/licenses/by-nc/3.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
repository_type Open Access Journal
institution_category Foreign Institution
institution US National Center for Biotechnology Information
building NCBI PubMed
collection Online Access
language English
format Online
author Ma, Han
Zheng, Yue
Zhu, Guoxing
Wu, Jie
Lu, Chun
Lai, Wei
spellingShingle Ma, Han
Zheng, Yue
Zhu, Guoxing
Wu, Jie
Lu, Chun
Lai, Wei
Rosai-Dorfman Disease with Massive Cutaneous Nodule on the Shoulder and Back
author_facet Ma, Han
Zheng, Yue
Zhu, Guoxing
Wu, Jie
Lu, Chun
Lai, Wei
author_sort Ma, Han
title Rosai-Dorfman Disease with Massive Cutaneous Nodule on the Shoulder and Back
title_short Rosai-Dorfman Disease with Massive Cutaneous Nodule on the Shoulder and Back
title_full Rosai-Dorfman Disease with Massive Cutaneous Nodule on the Shoulder and Back
title_fullStr Rosai-Dorfman Disease with Massive Cutaneous Nodule on the Shoulder and Back
title_full_unstemmed Rosai-Dorfman Disease with Massive Cutaneous Nodule on the Shoulder and Back
title_sort rosai-dorfman disease with massive cutaneous nodule on the shoulder and back
description Rosai-Dorfman disease is a rare, idiopathic, benign, and self-limiting histiocytic proliferative disorder. A 26-year-old man presented with a single massive cutaneous nodule (reaching 30 cm in diameter) on the left shoulder and back for 15 months. The routine hematological and biochemical tests were normal. Magnetic resonance scanning showed the lesion involved the skin, subcutaneous tissue, and subjacent muscle group, accompanied by obvious lymph node enlargement in the left part of the neck, supraclavicular fossa, and axillary fossa. The histopathology of the left cervical lymph node revealed diffuse effacement of the normal nodal architecture, with patchy chronic inflammatory cell infiltrates comprising lymphocytes and sheets of histiocytes. Some histiocytes contained lymphocytes within their pale cytoplasm. Many multinucleated giant cells were found; however, caseating granulomas were not seen. The skin and muscle biopsy specimen obtained from the back revealed infiltrating lymphocytes and histiocytes diffusely distributed in the dermis, subcutaneous tissue, and crevices of the muscle fibers. The phenomenon of emperipolesis and the presence of multinucleated giant cells were also seen. Immunohistochemical staining revealed that the histiocytes were positive for S-100 protein and CD68 but negative for CD1a. Immunophenotyping of the infiltrating lymphocytes indicated positive reactions to CD3, CD45RO, CD5, CD7, CD4, CD8 (partly), CD79a, CD20 (partly), and Ki-67 (<1%). The final diagnosis was Rosai-Dorfman disease. Owing to the extensive and deep involvement of the subcutaneous tissue and muscles, the patient did not undergo surgery to excise the massive skin nodule. The lesion showed no obvious change at the 12-month follow-up.
publisher Korean Dermatological Association; The Korean Society for Investigative Dermatology
publishDate 2015
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4323606/
_version_ 1613186508116721664

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