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Melkersson-Rosenthal Syndrome with Genitalia Involved in a 12-Year-Old Boy
Melkersson–Rosenthal syndrome (MRS) is an uncommon granulomatous disease characterized by the triad of relapsing facial paralysis, orofacial swelling, and fissured tongue. Genital swelling in MRS is rarely reported. We presented the first case of complete MRS with genital swelling in a child. Biopsy...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Korean Dermatological Association; The Korean Society for Investigative Dermatology
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4828388/ https://www.ncbi.nlm.nih.gov/pubmed/27081272 http://dx.doi.org/10.5021/ad.2016.28.2.232 |
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author | Chu, Zhaowei Liu, Yanting Zhang, Huan Zeng, Weihui Geng, Songmei |
author_facet | Chu, Zhaowei Liu, Yanting Zhang, Huan Zeng, Weihui Geng, Songmei |
author_sort | Chu, Zhaowei |
collection | PubMed |
description | Melkersson–Rosenthal syndrome (MRS) is an uncommon granulomatous disease characterized by the triad of relapsing facial paralysis, orofacial swelling, and fissured tongue. Genital swelling in MRS is rarely reported. We presented the first case of complete MRS with genital swelling in a child. Biopsy examinations of both the child's lower lip and penis showed noncaseating granuloma and intralymphatic granuloma infiltration. No symptoms or signs of other systemic disease (Crohn's disease or sarcoidosis) were observed after 2 years of follow-up. Genetic screening for CARD15/NOD2 in this patient showed negative, which further confirmed the diagnosis of MRS. Eleven other cases of suspected complete or incomplete MRS with genitalia involved were reviewed. Our case emphasizes the specific clinical feature of MRS with genitalia involved, which was genetically different from Crohn's disease and could be an independent entity. Lymphatic obstruction is responsible for localized edema in MRS. |
format | Online Article Text |
id | pubmed-4828388 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Korean Dermatological Association; The Korean Society for Investigative Dermatology |
record_format | MEDLINE/PubMed |
spelling | pubmed-48283882016-04-14 Melkersson-Rosenthal Syndrome with Genitalia Involved in a 12-Year-Old Boy Chu, Zhaowei Liu, Yanting Zhang, Huan Zeng, Weihui Geng, Songmei Ann Dermatol Case Report Melkersson–Rosenthal syndrome (MRS) is an uncommon granulomatous disease characterized by the triad of relapsing facial paralysis, orofacial swelling, and fissured tongue. Genital swelling in MRS is rarely reported. We presented the first case of complete MRS with genital swelling in a child. Biopsy examinations of both the child's lower lip and penis showed noncaseating granuloma and intralymphatic granuloma infiltration. No symptoms or signs of other systemic disease (Crohn's disease or sarcoidosis) were observed after 2 years of follow-up. Genetic screening for CARD15/NOD2 in this patient showed negative, which further confirmed the diagnosis of MRS. Eleven other cases of suspected complete or incomplete MRS with genitalia involved were reviewed. Our case emphasizes the specific clinical feature of MRS with genitalia involved, which was genetically different from Crohn's disease and could be an independent entity. Lymphatic obstruction is responsible for localized edema in MRS. Korean Dermatological Association; The Korean Society for Investigative Dermatology 2016-04 2016-03-31 /pmc/articles/PMC4828388/ /pubmed/27081272 http://dx.doi.org/10.5021/ad.2016.28.2.232 Text en Copyright © 2016 The Korean Dermatological Association and The Korean Society for Investigative Dermatology http://creativecommons.org/licenses/by-nc/4.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Chu, Zhaowei Liu, Yanting Zhang, Huan Zeng, Weihui Geng, Songmei Melkersson-Rosenthal Syndrome with Genitalia Involved in a 12-Year-Old Boy |
title | Melkersson-Rosenthal Syndrome with Genitalia Involved in a 12-Year-Old Boy |
title_full | Melkersson-Rosenthal Syndrome with Genitalia Involved in a 12-Year-Old Boy |
title_fullStr | Melkersson-Rosenthal Syndrome with Genitalia Involved in a 12-Year-Old Boy |
title_full_unstemmed | Melkersson-Rosenthal Syndrome with Genitalia Involved in a 12-Year-Old Boy |
title_short | Melkersson-Rosenthal Syndrome with Genitalia Involved in a 12-Year-Old Boy |
title_sort | melkersson-rosenthal syndrome with genitalia involved in a 12-year-old boy |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4828388/ https://www.ncbi.nlm.nih.gov/pubmed/27081272 http://dx.doi.org/10.5021/ad.2016.28.2.232 |
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