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
收藏
页码：2375 / 2379
页数：5
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