Bullous Pemphigoid in X-linked Alport Syndrome

Skin lesions in X-linked Alport syndrome (XLAS) are rarely observed. Bullous pemphigoid (BP) is caused by autoantibodies against BP180, also called α1 (XVII) chain, in the basement membrane zone (BMZ). A 48-year-old man with XLAS developed tense blisters. A skin biopsy showed a cleft between the bas...

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Autores principales: Yamawaki, Masahiro, Katayama, Kan, Fujimoto, Mika, Goto, Hiroyuki, Yuasa, Hiroto, Kozuka, Yuji, Mori, Mutsuki, Takahashi, Daisuke, Saiki, Ryosuke, Hirabayashi, Yosuke, Murata, Tomohiro, Yamanaka, Keiichi, Dohi, Kaoru
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2022
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10484771/
https://www.ncbi.nlm.nih.gov/pubmed/36543213
http://dx.doi.org/10.2169/internalmedicine.0972-22
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author Yamawaki, Masahiro
Katayama, Kan
Fujimoto, Mika
Goto, Hiroyuki
Yuasa, Hiroto
Kozuka, Yuji
Mori, Mutsuki
Takahashi, Daisuke
Saiki, Ryosuke
Hirabayashi, Yosuke
Murata, Tomohiro
Yamanaka, Keiichi
Dohi, Kaoru
author_facet Yamawaki, Masahiro
Katayama, Kan
Fujimoto, Mika
Goto, Hiroyuki
Yuasa, Hiroto
Kozuka, Yuji
Mori, Mutsuki
Takahashi, Daisuke
Saiki, Ryosuke
Hirabayashi, Yosuke
Murata, Tomohiro
Yamanaka, Keiichi
Dohi, Kaoru
author_sort Yamawaki, Masahiro
collection PubMed
description Skin lesions in X-linked Alport syndrome (XLAS) are rarely observed. Bullous pemphigoid (BP) is caused by autoantibodies against BP180, also called α1 (XVII) chain, in the basement membrane zone (BMZ). A 48-year-old man with XLAS developed tense blisters. A skin biopsy showed a cleft between the basal cell layer and dermis, with the infiltration of neutrophils and eosinophils. α1 (XVII) staining was positive on the epidermal side of α2/5 (IV) staining. Oral prednisolone improved his symptoms gradually. Abundant tense blisters on the palms and soles might suggest an important role of the α5 (IV) chain in the integrity of BMZ.
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spelling pubmed-104847712023-09-09 Bullous Pemphigoid in X-linked Alport Syndrome Yamawaki, Masahiro Katayama, Kan Fujimoto, Mika Goto, Hiroyuki Yuasa, Hiroto Kozuka, Yuji Mori, Mutsuki Takahashi, Daisuke Saiki, Ryosuke Hirabayashi, Yosuke Murata, Tomohiro Yamanaka, Keiichi Dohi, Kaoru Intern Med Case Report Skin lesions in X-linked Alport syndrome (XLAS) are rarely observed. Bullous pemphigoid (BP) is caused by autoantibodies against BP180, also called α1 (XVII) chain, in the basement membrane zone (BMZ). A 48-year-old man with XLAS developed tense blisters. A skin biopsy showed a cleft between the basal cell layer and dermis, with the infiltration of neutrophils and eosinophils. α1 (XVII) staining was positive on the epidermal side of α2/5 (IV) staining. Oral prednisolone improved his symptoms gradually. Abundant tense blisters on the palms and soles might suggest an important role of the α5 (IV) chain in the integrity of BMZ. The Japanese Society of Internal Medicine 2022-12-21 2023-08-15 /pmc/articles/PMC10484771/ /pubmed/36543213 http://dx.doi.org/10.2169/internalmedicine.0972-22 Text en Copyright © 2023 by The Japanese Society of Internal Medicine https://creativecommons.org/licenses/by-nc-nd/4.0/The Internal Medicine is an Open Access journal distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit (https://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
Yamawaki, Masahiro
Katayama, Kan
Fujimoto, Mika
Goto, Hiroyuki
Yuasa, Hiroto
Kozuka, Yuji
Mori, Mutsuki
Takahashi, Daisuke
Saiki, Ryosuke
Hirabayashi, Yosuke
Murata, Tomohiro
Yamanaka, Keiichi
Dohi, Kaoru
Bullous Pemphigoid in X-linked Alport Syndrome
title Bullous Pemphigoid in X-linked Alport Syndrome
title_full Bullous Pemphigoid in X-linked Alport Syndrome
title_fullStr Bullous Pemphigoid in X-linked Alport Syndrome
title_full_unstemmed Bullous Pemphigoid in X-linked Alport Syndrome
title_short Bullous Pemphigoid in X-linked Alport Syndrome
title_sort bullous pemphigoid in x-linked alport syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10484771/
https://www.ncbi.nlm.nih.gov/pubmed/36543213
http://dx.doi.org/10.2169/internalmedicine.0972-22
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