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Scleritis and anterior uveitis may herald the development of an epibulbar tumor in patients with extranodal Rosai-Dorfman disease: a case report
BACKGROUND: Rosai-Dorfman disease is a rare non–Langerhans cell histiocytosis. Ocular involvement is even rarer, mostly involving the orbit and eyelids, although marginal corneal ulcers, uveitis, and epibulbar masses have also been reported, and is characterized by multiple recurrences. However, the...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6617840/ https://www.ncbi.nlm.nih.gov/pubmed/31291929 http://dx.doi.org/10.1186/s12886-019-1158-2 |
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author | Lee, Yu-Kuei Chao, Sheau-Chiou Lee, Chaw-Ning Hung, Jia-Horung |
author_facet | Lee, Yu-Kuei Chao, Sheau-Chiou Lee, Chaw-Ning Hung, Jia-Horung |
author_sort | Lee, Yu-Kuei |
collection | PubMed |
description | BACKGROUND: Rosai-Dorfman disease is a rare non–Langerhans cell histiocytosis. Ocular involvement is even rarer, mostly involving the orbit and eyelids, although marginal corneal ulcers, uveitis, and epibulbar masses have also been reported, and is characterized by multiple recurrences. However, the disease course and optimal treatment strategies remain undetermined, in light of the rarity of this disease. CASE PRESENTATION: We reported a 36-year-old male patient with the extranodal form of Rosai-Dorfman disease, presenting with scleritis and anterior uveitis in the left eye, who experienced subsequent development of an epibulbar tumor in the same eye. The patient was also complicated by a relapsing facial nodule on the right cheek. After the pathological diagnosis of Rosai-Dorfman disease was obtained, the patient underwent surgical excision of the epibulbar tumor and the facial nodule, accompanied by systemic immunosuppression therapy. At the last follow-up, the patient was asymptomatic without signs of recurrence. CONCLUSIONS: This report highlights the progression of ocular manifestations of Rosai-Dorfman disease and emphasizes the importance of systemic therapy. |
format | Online Article Text |
id | pubmed-6617840 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-66178402019-07-22 Scleritis and anterior uveitis may herald the development of an epibulbar tumor in patients with extranodal Rosai-Dorfman disease: a case report Lee, Yu-Kuei Chao, Sheau-Chiou Lee, Chaw-Ning Hung, Jia-Horung BMC Ophthalmol Case Report BACKGROUND: Rosai-Dorfman disease is a rare non–Langerhans cell histiocytosis. Ocular involvement is even rarer, mostly involving the orbit and eyelids, although marginal corneal ulcers, uveitis, and epibulbar masses have also been reported, and is characterized by multiple recurrences. However, the disease course and optimal treatment strategies remain undetermined, in light of the rarity of this disease. CASE PRESENTATION: We reported a 36-year-old male patient with the extranodal form of Rosai-Dorfman disease, presenting with scleritis and anterior uveitis in the left eye, who experienced subsequent development of an epibulbar tumor in the same eye. The patient was also complicated by a relapsing facial nodule on the right cheek. After the pathological diagnosis of Rosai-Dorfman disease was obtained, the patient underwent surgical excision of the epibulbar tumor and the facial nodule, accompanied by systemic immunosuppression therapy. At the last follow-up, the patient was asymptomatic without signs of recurrence. CONCLUSIONS: This report highlights the progression of ocular manifestations of Rosai-Dorfman disease and emphasizes the importance of systemic therapy. BioMed Central 2019-07-10 /pmc/articles/PMC6617840/ /pubmed/31291929 http://dx.doi.org/10.1186/s12886-019-1158-2 Text en © The Author(s). 2019 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Case Report Lee, Yu-Kuei Chao, Sheau-Chiou Lee, Chaw-Ning Hung, Jia-Horung Scleritis and anterior uveitis may herald the development of an epibulbar tumor in patients with extranodal Rosai-Dorfman disease: a case report |
title | Scleritis and anterior uveitis may herald the development of an epibulbar tumor in patients with extranodal Rosai-Dorfman disease: a case report |
title_full | Scleritis and anterior uveitis may herald the development of an epibulbar tumor in patients with extranodal Rosai-Dorfman disease: a case report |
title_fullStr | Scleritis and anterior uveitis may herald the development of an epibulbar tumor in patients with extranodal Rosai-Dorfman disease: a case report |
title_full_unstemmed | Scleritis and anterior uveitis may herald the development of an epibulbar tumor in patients with extranodal Rosai-Dorfman disease: a case report |
title_short | Scleritis and anterior uveitis may herald the development of an epibulbar tumor in patients with extranodal Rosai-Dorfman disease: a case report |
title_sort | scleritis and anterior uveitis may herald the development of an epibulbar tumor in patients with extranodal rosai-dorfman disease: a case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6617840/ https://www.ncbi.nlm.nih.gov/pubmed/31291929 http://dx.doi.org/10.1186/s12886-019-1158-2 |
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