Bullous Pemphigoid in X-linked Alport Syndrome

Masahiro Yamawaki; Kan Katayama; Mika Fujimoto; Hiroyuki Goto; Hiroto Yuasa; Yuji Kozuka; Mutsuki Mori; Daisuke Takahashi; Ryosuke Saiki; Yosuke Hirabayashi; Tomohiro Murata; Keiichi Yamanaka; Kaoru Dohi

doi:10.2169/internalmedicine.0972-22

CASE REPORTS

Bullous Pemphigoid in X-linked Alport Syndrome

Masahiro Yamawaki, Kan Katayama, Mika Fujimoto, Hiroyuki Goto, Hiroto Yuasa, Yuji Kozuka, Mutsuki Mori, Daisuke Takahashi, Ryosuke Saiki, Yosuke Hirabayashi, Tomohiro Murata, Keiichi Yamanaka, Kaoru Dohi

Author information

Masahiro Yamawaki
Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Japan
Kan Katayama
Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Japan
Mika Fujimoto
Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Japan
Hiroyuki Goto
Department of Dermatology, Mie University Graduate School of Medicine, Japan
Hiroto Yuasa
Department of Pathology, Mie University Hospital, Japan
Yuji Kozuka
Department of Pathology, Mie University Hospital, Japan
Mutsuki Mori
Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Japan
Daisuke Takahashi
Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Japan
Ryosuke Saiki
Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Japan
Yosuke Hirabayashi
Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Japan
Tomohiro Murata
Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Japan
Keiichi Yamanaka
Department of Dermatology, Mie University Graduate School of Medicine, Japan
Kaoru Dohi
Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Japan

Keywords: basement membrane zone, bullous pemphigoid, COL4A5, X-linked Alport syndrome

JOURNAL OPEN ACCESS

2023 Volume 62 Issue 16 Pages 2375-2379

DOI https://doi.org/10.2169/internalmedicine.0972-22

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Details

Abstract

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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