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Linear Immunoglobulin A (IgA) Bullous Dermatosis Mimicking Stevens-Johnson Syndrome

Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disease characterized by linear IgA deposition along the dermo-epidermal junction on direct immunofluorescence (DIF). LABD appears clinically as erythematous polycyclic lesions in younger patients but can show considerable phenotyp...

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Detalles Bibliográficos
Autores principales: Park, Jason S, Hamilton, Caitlin D, Patel, Shaan, Lee, Jason B, Hsu, Sylvia
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9650960/
https://www.ncbi.nlm.nih.gov/pubmed/36381872
http://dx.doi.org/10.7759/cureus.30309
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author Park, Jason S
Hamilton, Caitlin D
Patel, Shaan
Lee, Jason B
Hsu, Sylvia
author_facet Park, Jason S
Hamilton, Caitlin D
Patel, Shaan
Lee, Jason B
Hsu, Sylvia
author_sort Park, Jason S
collection PubMed
description Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disease characterized by linear IgA deposition along the dermo-epidermal junction on direct immunofluorescence (DIF). LABD appears clinically as erythematous polycyclic lesions in younger patients but can show considerable phenotypic heterogeneity in older patients, often leading to misdiagnoses such as bullous pemphigoid, pemphigus vulgaris, Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN), or other bullous conditions. Cases of LABD mimicking SJS/TEN require prompt skin biopsies for histopathology and DIF for disease differentiation and medical decision-making. In cases of suspected drug-induced LABD or SJS/TEN, identification and removal of the offending agent are paramount. The preferred treatment for LABD is oral dapsone, while SJS/TEN may respond better to cyclosporine or a combination of intravenous immunoglobulin and systemic corticosteroids. This case highlights the rare occurrence of LABD mimicking SJS/TEN and emphasizes the details that clinicians must know to guide patient management.
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spelling pubmed-96509602022-11-14 Linear Immunoglobulin A (IgA) Bullous Dermatosis Mimicking Stevens-Johnson Syndrome Park, Jason S Hamilton, Caitlin D Patel, Shaan Lee, Jason B Hsu, Sylvia Cureus Dermatology Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disease characterized by linear IgA deposition along the dermo-epidermal junction on direct immunofluorescence (DIF). LABD appears clinically as erythematous polycyclic lesions in younger patients but can show considerable phenotypic heterogeneity in older patients, often leading to misdiagnoses such as bullous pemphigoid, pemphigus vulgaris, Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN), or other bullous conditions. Cases of LABD mimicking SJS/TEN require prompt skin biopsies for histopathology and DIF for disease differentiation and medical decision-making. In cases of suspected drug-induced LABD or SJS/TEN, identification and removal of the offending agent are paramount. The preferred treatment for LABD is oral dapsone, while SJS/TEN may respond better to cyclosporine or a combination of intravenous immunoglobulin and systemic corticosteroids. This case highlights the rare occurrence of LABD mimicking SJS/TEN and emphasizes the details that clinicians must know to guide patient management. Cureus 2022-10-14 /pmc/articles/PMC9650960/ /pubmed/36381872 http://dx.doi.org/10.7759/cureus.30309 Text en Copyright © 2022, Park et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Dermatology
Park, Jason S
Hamilton, Caitlin D
Patel, Shaan
Lee, Jason B
Hsu, Sylvia
Linear Immunoglobulin A (IgA) Bullous Dermatosis Mimicking Stevens-Johnson Syndrome
title Linear Immunoglobulin A (IgA) Bullous Dermatosis Mimicking Stevens-Johnson Syndrome
title_full Linear Immunoglobulin A (IgA) Bullous Dermatosis Mimicking Stevens-Johnson Syndrome
title_fullStr Linear Immunoglobulin A (IgA) Bullous Dermatosis Mimicking Stevens-Johnson Syndrome
title_full_unstemmed Linear Immunoglobulin A (IgA) Bullous Dermatosis Mimicking Stevens-Johnson Syndrome
title_short Linear Immunoglobulin A (IgA) Bullous Dermatosis Mimicking Stevens-Johnson Syndrome
title_sort linear immunoglobulin a (iga) bullous dermatosis mimicking stevens-johnson syndrome
topic Dermatology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9650960/
https://www.ncbi.nlm.nih.gov/pubmed/36381872
http://dx.doi.org/10.7759/cureus.30309
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