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An Underrecognized Histologic Clue to the Diagnosis of Mucous Membrane Pemphigoid: A Case Report and Review of Diagnostic Guidelines
Mucous membrane pemphigoid (MMP), also known as cicatricial pemphigoid (CP), is a heterogeneous group of subepidermal blistering diseases that affect the mucous membranes, most frequently in the eye and oral cavity. MMP is often unrecognized or misdiagnosed in its early stages due to its rarity and...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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MDPI
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9944081/ https://www.ncbi.nlm.nih.gov/pubmed/36810567 http://dx.doi.org/10.3390/dermatopathology10010009 |
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author | McFadden, Jason R. Chaudhari, Advaita S. Birenbaum, Debra Margesson, Lynette Gonzalez, Jorge Sriharan, Aravindhan |
author_facet | McFadden, Jason R. Chaudhari, Advaita S. Birenbaum, Debra Margesson, Lynette Gonzalez, Jorge Sriharan, Aravindhan |
author_sort | McFadden, Jason R. |
collection | PubMed |
description | Mucous membrane pemphigoid (MMP), also known as cicatricial pemphigoid (CP), is a heterogeneous group of subepidermal blistering diseases that affect the mucous membranes, most frequently in the eye and oral cavity. MMP is often unrecognized or misdiagnosed in its early stages due to its rarity and nonspecific presentation. We present the case of a 69-year-old female in which MMP of the vulva was not initially suspected. The first biopsy, from lesional tissue for routine histology, revealed fibrosis, late-stage granulation tissue, and nonspecific findings. A second biopsy, from perilesional tissue for direct immunofluorescence (DIF), revealed DIF findings typical of MMP. Scrutiny of both the first and second biopsies revealed a subtle but telling histologic feature: subepithelial clefts along adnexae in the context of a scarring process with neutrophils and eosinophils, which can be an important clue to MMP. This histologic clue has been previously described; reinforcing its importance may prove useful for future cases, especially those for which DIF is not feasible. Our case demonstrates the protean presentations of MMP, the need for persistence in sampling unusual cases, and the relevance of inconspicuous histologic features. The report highlights this underrecognized yet potentially decisive histologic clue to MMP, reviews current biopsy guidelines when MMP is suspected, and delineates the clinical and morphological features of vulvar MMP. |
format | Online Article Text |
id | pubmed-9944081 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-99440812023-02-23 An Underrecognized Histologic Clue to the Diagnosis of Mucous Membrane Pemphigoid: A Case Report and Review of Diagnostic Guidelines McFadden, Jason R. Chaudhari, Advaita S. Birenbaum, Debra Margesson, Lynette Gonzalez, Jorge Sriharan, Aravindhan Dermatopathology (Basel) Case Report Mucous membrane pemphigoid (MMP), also known as cicatricial pemphigoid (CP), is a heterogeneous group of subepidermal blistering diseases that affect the mucous membranes, most frequently in the eye and oral cavity. MMP is often unrecognized or misdiagnosed in its early stages due to its rarity and nonspecific presentation. We present the case of a 69-year-old female in which MMP of the vulva was not initially suspected. The first biopsy, from lesional tissue for routine histology, revealed fibrosis, late-stage granulation tissue, and nonspecific findings. A second biopsy, from perilesional tissue for direct immunofluorescence (DIF), revealed DIF findings typical of MMP. Scrutiny of both the first and second biopsies revealed a subtle but telling histologic feature: subepithelial clefts along adnexae in the context of a scarring process with neutrophils and eosinophils, which can be an important clue to MMP. This histologic clue has been previously described; reinforcing its importance may prove useful for future cases, especially those for which DIF is not feasible. Our case demonstrates the protean presentations of MMP, the need for persistence in sampling unusual cases, and the relevance of inconspicuous histologic features. The report highlights this underrecognized yet potentially decisive histologic clue to MMP, reviews current biopsy guidelines when MMP is suspected, and delineates the clinical and morphological features of vulvar MMP. MDPI 2023-02-02 /pmc/articles/PMC9944081/ /pubmed/36810567 http://dx.doi.org/10.3390/dermatopathology10010009 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Case Report McFadden, Jason R. Chaudhari, Advaita S. Birenbaum, Debra Margesson, Lynette Gonzalez, Jorge Sriharan, Aravindhan An Underrecognized Histologic Clue to the Diagnosis of Mucous Membrane Pemphigoid: A Case Report and Review of Diagnostic Guidelines |
title | An Underrecognized Histologic Clue to the Diagnosis of Mucous Membrane Pemphigoid: A Case Report and Review of Diagnostic Guidelines |
title_full | An Underrecognized Histologic Clue to the Diagnosis of Mucous Membrane Pemphigoid: A Case Report and Review of Diagnostic Guidelines |
title_fullStr | An Underrecognized Histologic Clue to the Diagnosis of Mucous Membrane Pemphigoid: A Case Report and Review of Diagnostic Guidelines |
title_full_unstemmed | An Underrecognized Histologic Clue to the Diagnosis of Mucous Membrane Pemphigoid: A Case Report and Review of Diagnostic Guidelines |
title_short | An Underrecognized Histologic Clue to the Diagnosis of Mucous Membrane Pemphigoid: A Case Report and Review of Diagnostic Guidelines |
title_sort | underrecognized histologic clue to the diagnosis of mucous membrane pemphigoid: a case report and review of diagnostic guidelines |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9944081/ https://www.ncbi.nlm.nih.gov/pubmed/36810567 http://dx.doi.org/10.3390/dermatopathology10010009 |
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