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Rosai-Dorfman Disease With Features of IgG4-Related Sclerosing Disease: A Case Report

Rosai-Dorfman disease (RDD) is a rare condition characterized by the proliferation of non-Langerhans cell histiocytes that are associated with phagocytosed lymphocytes (emperipolesis). Clinically, it is classified into nodal, extra-nodal, neoplasia-associated RDD, and immune-related. Here, we presen...

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Autores principales: AlMadan, Nasser M, Alwhabi, Mohammed K, Assem, Assem S, Khoja, Hatem
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9447937/
https://www.ncbi.nlm.nih.gov/pubmed/36106222
http://dx.doi.org/10.7759/cureus.27704
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author AlMadan, Nasser M
Alwhabi, Mohammed K
Assem, Assem S
Khoja, Hatem
author_facet AlMadan, Nasser M
Alwhabi, Mohammed K
Assem, Assem S
Khoja, Hatem
author_sort AlMadan, Nasser M
collection PubMed
description Rosai-Dorfman disease (RDD) is a rare condition characterized by the proliferation of non-Langerhans cell histiocytes that are associated with phagocytosed lymphocytes (emperipolesis). Clinically, it is classified into nodal, extra-nodal, neoplasia-associated RDD, and immune-related. Here, we present a case of a 65-year-old female who presented with facial pain following a dental procedure with no focal neurologic deficit. The MRI of the head and neck showed a well-defined lobulated soft tissue lesion with homogenous enhancement over the left cheek. Excision of the lesion was done, and the histopathological study reported extra-nodal RDD with features of IgG4-related sclerosing disease. The patient had no recurrence over the two years from the date of diagnosis.
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spelling pubmed-94479372022-09-13 Rosai-Dorfman Disease With Features of IgG4-Related Sclerosing Disease: A Case Report AlMadan, Nasser M Alwhabi, Mohammed K Assem, Assem S Khoja, Hatem Cureus Pathology Rosai-Dorfman disease (RDD) is a rare condition characterized by the proliferation of non-Langerhans cell histiocytes that are associated with phagocytosed lymphocytes (emperipolesis). Clinically, it is classified into nodal, extra-nodal, neoplasia-associated RDD, and immune-related. Here, we present a case of a 65-year-old female who presented with facial pain following a dental procedure with no focal neurologic deficit. The MRI of the head and neck showed a well-defined lobulated soft tissue lesion with homogenous enhancement over the left cheek. Excision of the lesion was done, and the histopathological study reported extra-nodal RDD with features of IgG4-related sclerosing disease. The patient had no recurrence over the two years from the date of diagnosis. Cureus 2022-08-05 /pmc/articles/PMC9447937/ /pubmed/36106222 http://dx.doi.org/10.7759/cureus.27704 Text en Copyright © 2022, AlMadan et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Pathology
AlMadan, Nasser M
Alwhabi, Mohammed K
Assem, Assem S
Khoja, Hatem
Rosai-Dorfman Disease With Features of IgG4-Related Sclerosing Disease: A Case Report
title Rosai-Dorfman Disease With Features of IgG4-Related Sclerosing Disease: A Case Report
title_full Rosai-Dorfman Disease With Features of IgG4-Related Sclerosing Disease: A Case Report
title_fullStr Rosai-Dorfman Disease With Features of IgG4-Related Sclerosing Disease: A Case Report
title_full_unstemmed Rosai-Dorfman Disease With Features of IgG4-Related Sclerosing Disease: A Case Report
title_short Rosai-Dorfman Disease With Features of IgG4-Related Sclerosing Disease: A Case Report
title_sort rosai-dorfman disease with features of igg4-related sclerosing disease: a case report
topic Pathology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9447937/
https://www.ncbi.nlm.nih.gov/pubmed/36106222
http://dx.doi.org/10.7759/cureus.27704
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