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Spongiotic Pattern in Pemphigus: A Retrospective Observational Single-Center Study

Pemphigus is a chronic blistering disorder caused by autoantibodies that target desmosomal proteins in the epidermis. Acantholysis may be absent, and pemphigus may present only with spongiosis and vesiculation, thereby leading to a misdiagnosis of eczema. Herein, we conducted a retrospective, observ...

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Autores principales: Preclaro, Ivan Arni C., Wu, Yu-Hung
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9149856/
https://www.ncbi.nlm.nih.gov/pubmed/35645233
http://dx.doi.org/10.3390/dermatopathology9020022
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author Preclaro, Ivan Arni C.
Wu, Yu-Hung
author_facet Preclaro, Ivan Arni C.
Wu, Yu-Hung
author_sort Preclaro, Ivan Arni C.
collection PubMed
description Pemphigus is a chronic blistering disorder caused by autoantibodies that target desmosomal proteins in the epidermis. Acantholysis may be absent, and pemphigus may present only with spongiosis and vesiculation, thereby leading to a misdiagnosis of eczema. Herein, we conducted a retrospective, observational, single-center study to establish a pattern of spongiosis in cases of pemphigus confirmed by direct immunofluorescence. Immunopathologically diagnosed pemphigus specimens from 2001 to 2020 were retrieved, and specimens with spongiosis were analyzed for the following features: vesiculation, acantholysis, spongiosis, inflammatory cells in the epidermis, and inflammation in the dermis. Cases of spongiotic dermatitis were used as control. Out of 99 immunopathologically diagnosed pemphigus specimens, 41 samples with spongiosis were identified. About one quarter of the specimens did not have acantholysis. Spongiosis in the middle to lower thirds of the perilesional epidermis (p = 0.030), exocytosis with either neutrophils or eosinophils (p = 0.016), dermal infiltrates composed of lymphocytes, eosinophils, and neutrophils (p = 0.012), and absence of Langerhans cell microabscesses (p < 0.001) were more common in pemphigus than control. Spongiosis in pemphigus may mimic eczema in patients without acantholysis. The subtle histological findings in this study provide diagnostic clues and suggest that further immunofluorescence should be performed to confirm pemphigus diagnosis.
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spelling pubmed-91498562022-05-31 Spongiotic Pattern in Pemphigus: A Retrospective Observational Single-Center Study Preclaro, Ivan Arni C. Wu, Yu-Hung Dermatopathology (Basel) Article Pemphigus is a chronic blistering disorder caused by autoantibodies that target desmosomal proteins in the epidermis. Acantholysis may be absent, and pemphigus may present only with spongiosis and vesiculation, thereby leading to a misdiagnosis of eczema. Herein, we conducted a retrospective, observational, single-center study to establish a pattern of spongiosis in cases of pemphigus confirmed by direct immunofluorescence. Immunopathologically diagnosed pemphigus specimens from 2001 to 2020 were retrieved, and specimens with spongiosis were analyzed for the following features: vesiculation, acantholysis, spongiosis, inflammatory cells in the epidermis, and inflammation in the dermis. Cases of spongiotic dermatitis were used as control. Out of 99 immunopathologically diagnosed pemphigus specimens, 41 samples with spongiosis were identified. About one quarter of the specimens did not have acantholysis. Spongiosis in the middle to lower thirds of the perilesional epidermis (p = 0.030), exocytosis with either neutrophils or eosinophils (p = 0.016), dermal infiltrates composed of lymphocytes, eosinophils, and neutrophils (p = 0.012), and absence of Langerhans cell microabscesses (p < 0.001) were more common in pemphigus than control. Spongiosis in pemphigus may mimic eczema in patients without acantholysis. The subtle histological findings in this study provide diagnostic clues and suggest that further immunofluorescence should be performed to confirm pemphigus diagnosis. MDPI 2022-05-20 /pmc/articles/PMC9149856/ /pubmed/35645233 http://dx.doi.org/10.3390/dermatopathology9020022 Text en © 2022 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 Article
Preclaro, Ivan Arni C.
Wu, Yu-Hung
Spongiotic Pattern in Pemphigus: A Retrospective Observational Single-Center Study
title Spongiotic Pattern in Pemphigus: A Retrospective Observational Single-Center Study
title_full Spongiotic Pattern in Pemphigus: A Retrospective Observational Single-Center Study
title_fullStr Spongiotic Pattern in Pemphigus: A Retrospective Observational Single-Center Study
title_full_unstemmed Spongiotic Pattern in Pemphigus: A Retrospective Observational Single-Center Study
title_short Spongiotic Pattern in Pemphigus: A Retrospective Observational Single-Center Study
title_sort spongiotic pattern in pemphigus: a retrospective observational single-center study
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9149856/
https://www.ncbi.nlm.nih.gov/pubmed/35645233
http://dx.doi.org/10.3390/dermatopathology9020022
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