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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Detalles Bibliográficos
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
Descripción
Sumario: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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