Bullous Pemphigoid in X-linked Alport Syndrome

被引：2

作者：

Yamawaki, Masahiro ^{[1
]}

Katayama, Kan ^{[1
]}

Fujimoto, Mika ^{[1
]}

Goto, Hiroyuki ^{[2
]}

Yuasa, Hiroto ^{[3
]}

Kozuka, Yuji ^{[3
]}

Mori, Mutsuki ^{[1
]}

Takahashi, Daisuke ^{[1
]}

Saiki, Ryosuke ^{[1
]}

Hirabayashi, Yosuke ^{[1
]}

Murata, Tomohiro ^{[1
]}

Yamanaka, Keiichi ^{[2
]}

Dohi, Kaoru ^{[1
]}

机构：

[1] Mie Univ, Dept Cardiol & Nephrol, Grad Sch Med, Tsu, Japan

[2] Mie Univ, Dept Dermatol, Grad Sch Med, Tsu, Japan

[3] Mie Univ Hosp, Dept Pathol, Tsu, Japan

来源：

INTERNAL MEDICINE | 2023年 / 62卷 / 16期

关键词：

basement membrane zone; bullous pemphigoid; X-linked Alport syndrome; SUBEPIDERMAL BLISTERS; COLLAGEN; IV; IDENTIFICATION; AUTOANTIBODIES; MUTATIONS; ALPHA-5;

D O I：

10.2169/internalmedicine.0972-22

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Skin lesions in X-linked Alport syndrome (XLAS) are rarely observed. Bullous pemphigoid (BP) is caused by autoantibodies against BP180, also called & alpha;1 (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. & alpha;1 (XVII) staining was positive on the epidermal side of & alpha;2/5 (IV) staining. Oral prednisolone improved his symptoms gradually. Abundant tense blisters on the palms and soles might suggest an important role of the & alpha;5 (IV) chain in the integrity of BMZ.

引用

页码：2375 / 2379

页数：5

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