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Mucous Membrane Pemphigoid Causing Central Airway Obstruction
Mucous membrane pemphigoid (MMP) is a rare variant of the skin disease pemphigoid, which predominantly involves the mucous membranes. This rare autoimmune disease that infrequently affects the respiratory tract is characterized by subepithelial blister formation that may result in scarring. Immunopa...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Wolters Kluwer Health/Lippincott Williams & Wilkins
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5639999/ https://www.ncbi.nlm.nih.gov/pubmed/28248822 http://dx.doi.org/10.1097/LBR.0000000000000374 |
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author | Jalil, Bilal A. Abdou, Yara G. Rosen, Stephanie A. Dammad, Tarek |
author_facet | Jalil, Bilal A. Abdou, Yara G. Rosen, Stephanie A. Dammad, Tarek |
author_sort | Jalil, Bilal A. |
collection | PubMed |
description | Mucous membrane pemphigoid (MMP) is a rare variant of the skin disease pemphigoid, which predominantly involves the mucous membranes. This rare autoimmune disease that infrequently affects the respiratory tract is characterized by subepithelial blister formation that may result in scarring. Immunopathologic examination of mucous membranes reveals the deposition of immunoglobulins and complement within the subepithelial basement membrane. We describe a patient with undiagnosed MMP, with a near-fatal presentation of central airway obstruction causing acute respiratory distress. The patient was successfully treated with emergent rigid bronchoscopic resection of a ball valve-like endotracheal mass, and diagnosed with a rare variant of pemphigoid disease, MMP. The patient was treated with mycophenolate and was clinically in remission, with bronchoscopically stable lesions at 1 year of follow-up. |
format | Online Article Text |
id | pubmed-5639999 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | Wolters Kluwer Health/Lippincott Williams & Wilkins |
record_format | MEDLINE/PubMed |
spelling | pubmed-56399992017-10-24 Mucous Membrane Pemphigoid Causing Central Airway Obstruction Jalil, Bilal A. Abdou, Yara G. Rosen, Stephanie A. Dammad, Tarek J Bronchology Interv Pulmonol Case Reports Mucous membrane pemphigoid (MMP) is a rare variant of the skin disease pemphigoid, which predominantly involves the mucous membranes. This rare autoimmune disease that infrequently affects the respiratory tract is characterized by subepithelial blister formation that may result in scarring. Immunopathologic examination of mucous membranes reveals the deposition of immunoglobulins and complement within the subepithelial basement membrane. We describe a patient with undiagnosed MMP, with a near-fatal presentation of central airway obstruction causing acute respiratory distress. The patient was successfully treated with emergent rigid bronchoscopic resection of a ball valve-like endotracheal mass, and diagnosed with a rare variant of pemphigoid disease, MMP. The patient was treated with mycophenolate and was clinically in remission, with bronchoscopically stable lesions at 1 year of follow-up. Wolters Kluwer Health/Lippincott Williams & Wilkins 2017-10 2017-02-28 /pmc/articles/PMC5639999/ /pubmed/28248822 http://dx.doi.org/10.1097/LBR.0000000000000374 Text en Copyright © 2017 The Author(s). Published by Wolters Kluwer Health, Inc. This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (http://creativecommons.org/licenses/by-nc-nd/4.0/) (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc-nd/4.0/ |
spellingShingle | Case Reports Jalil, Bilal A. Abdou, Yara G. Rosen, Stephanie A. Dammad, Tarek Mucous Membrane Pemphigoid Causing Central Airway Obstruction |
title | Mucous Membrane Pemphigoid Causing Central Airway Obstruction |
title_full | Mucous Membrane Pemphigoid Causing Central Airway Obstruction |
title_fullStr | Mucous Membrane Pemphigoid Causing Central Airway Obstruction |
title_full_unstemmed | Mucous Membrane Pemphigoid Causing Central Airway Obstruction |
title_short | Mucous Membrane Pemphigoid Causing Central Airway Obstruction |
title_sort | mucous membrane pemphigoid causing central airway obstruction |
topic | Case Reports |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5639999/ https://www.ncbi.nlm.nih.gov/pubmed/28248822 http://dx.doi.org/10.1097/LBR.0000000000000374 |
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