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Combined Cutaneous Rosai-Dorfman Disease and Localized Cutaneous Langerhans Cell Histiocytosis Within a Single Subcutaneous Nodule
Rosai-Dorfman disease (RDD) is a reactive multisystem histiocytosis that typically presents with cervical lymphadenopathy and systemic symptoms. Cutaneous involvement occurs in approximately 10% of cases, and 3% of cases are limited to the skin without nodal or other extranodal involvement. Langerha...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The American Journal of Dermatopathology
2015
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4894786/ https://www.ncbi.nlm.nih.gov/pubmed/26588339 http://dx.doi.org/10.1097/DAD.0000000000000347 |
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author | Litzner, Brandon R. Subtil, Antonio Vidal, Claudia I. |
author_facet | Litzner, Brandon R. Subtil, Antonio Vidal, Claudia I. |
author_sort | Litzner, Brandon R. |
collection | PubMed |
description | Rosai-Dorfman disease (RDD) is a reactive multisystem histiocytosis that typically presents with cervical lymphadenopathy and systemic symptoms. Cutaneous involvement occurs in approximately 10% of cases, and 3% of cases are limited to the skin without nodal or other extranodal involvement. Langerhans cell histiocytosis (LCH) is a clonal histiocytosis with a wide spectrum of presentations ranging from isolated skin or bone disease to multisystem involvement. Rare case reports have identified concomitant presentation of RDD and LCH; however, most of these reports have involved LCH and RDD occurring concurrently but at separate sites. We present a rare case of concurrent RDD and LCH presenting within a single skin nodule. The patient did not have any evidence of systemic involvement and has remained stable without additional treatment. We also review the literature on this unusual co-presentation and suggest possible underlying mechanisms. Finally, we recommend baseline laboratory and imaging studies and discuss treatment options based on the available evidence. |
format | Online Article Text |
id | pubmed-4894786 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2015 |
publisher | The American Journal of Dermatopathology |
record_format | MEDLINE/PubMed |
spelling | pubmed-48947862016-06-21 Combined Cutaneous Rosai-Dorfman Disease and Localized Cutaneous Langerhans Cell Histiocytosis Within a Single Subcutaneous Nodule Litzner, Brandon R. Subtil, Antonio Vidal, Claudia I. Am J Dermatopathol Extraordinary Case Report Rosai-Dorfman disease (RDD) is a reactive multisystem histiocytosis that typically presents with cervical lymphadenopathy and systemic symptoms. Cutaneous involvement occurs in approximately 10% of cases, and 3% of cases are limited to the skin without nodal or other extranodal involvement. Langerhans cell histiocytosis (LCH) is a clonal histiocytosis with a wide spectrum of presentations ranging from isolated skin or bone disease to multisystem involvement. Rare case reports have identified concomitant presentation of RDD and LCH; however, most of these reports have involved LCH and RDD occurring concurrently but at separate sites. We present a rare case of concurrent RDD and LCH presenting within a single skin nodule. The patient did not have any evidence of systemic involvement and has remained stable without additional treatment. We also review the literature on this unusual co-presentation and suggest possible underlying mechanisms. Finally, we recommend baseline laboratory and imaging studies and discuss treatment options based on the available evidence. The American Journal of Dermatopathology 2015-12 2015-11-20 /pmc/articles/PMC4894786/ /pubmed/26588339 http://dx.doi.org/10.1097/DAD.0000000000000347 Text en Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. |
spellingShingle | Extraordinary Case Report Litzner, Brandon R. Subtil, Antonio Vidal, Claudia I. Combined Cutaneous Rosai-Dorfman Disease and Localized Cutaneous Langerhans Cell Histiocytosis Within a Single Subcutaneous Nodule |
title | Combined Cutaneous Rosai-Dorfman Disease and Localized Cutaneous Langerhans Cell Histiocytosis Within a Single Subcutaneous Nodule |
title_full | Combined Cutaneous Rosai-Dorfman Disease and Localized Cutaneous Langerhans Cell Histiocytosis Within a Single Subcutaneous Nodule |
title_fullStr | Combined Cutaneous Rosai-Dorfman Disease and Localized Cutaneous Langerhans Cell Histiocytosis Within a Single Subcutaneous Nodule |
title_full_unstemmed | Combined Cutaneous Rosai-Dorfman Disease and Localized Cutaneous Langerhans Cell Histiocytosis Within a Single Subcutaneous Nodule |
title_short | Combined Cutaneous Rosai-Dorfman Disease and Localized Cutaneous Langerhans Cell Histiocytosis Within a Single Subcutaneous Nodule |
title_sort | combined cutaneous rosai-dorfman disease and localized cutaneous langerhans cell histiocytosis within a single subcutaneous nodule |
topic | Extraordinary Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4894786/ https://www.ncbi.nlm.nih.gov/pubmed/26588339 http://dx.doi.org/10.1097/DAD.0000000000000347 |
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