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

共 50 条

[21] Temporal Bone Histopathology of X-linked Inherited Alport Syndrome
Ungar, Omer J.
Nadol, Joseph B.
Santos, Felipe
LARYNGOSCOPE INVESTIGATIVE OTOLARYNGOLOGY, 2018, 3 (04): : 311 - 314
[22] Corneal endothelial neovascularization and glaucoma in X-linked Alport syndrome
Zhou, Lin
Zhang, Yao
Tian, Chaohua
Liao, Jinying
Yu, Houjue
Tang, Li
EUROPEAN JOURNAL OF OPHTHALMOLOGY, 2025,
[23] X-linked Alport Syndrome in Children: Clinical and Pathological Features
Yin, Xiaoling
Wang, Jia
Liu, Tonglin
Tang, Jinhui
Qiu, Liru
Chen, Yu
Yuan, Huiqing
JianhuaZhou
PEDIATRIC NEPHROLOGY, 2013, 28 (08) : 1547 - 1547
[24] NEPHROPROTECTION IN BOYS WITH X-LINKED ALPORT SYNDROME: THE SOONER THE BETTER
Aksenova, Marina
Piruzeeva, Oxana
Zaikova, Natalia
Lepaeva, Tatjana
Nikishina, Tatjana
Obuhova, Varvara
Konkova, Natalia
NEPHROLOGY DIALYSIS TRANSPLANTATION, 2023, 38 : I693 - I693
[25] GIRLS WITH X-LINKED ALPORT SYNDROME: RISK OF THE DISEASE PROGRESSION
Aksenova, Marina
Konkova, Natalia
Lepaeva, Tatiana
Nikishina, Tatiana
Morozov, Sergey
Piruzeeva, Oxana
Zaikova, Natalia
Obuhova, Varvara
Dlin, Vladimir
PEDIATRIC NEPHROLOGY, 2023, 38 : S238 - S239
[26] A family with X-linked Alport syndrome confirmed by skin biopsy
Komatsuda, A
Ohtani, H
Wakui, H
Tokuda, N
Nakamoto, Y
Sado, Y
Naitoh, I
Imai, H
NEPHROLOGY DIALYSIS TRANSPLANTATION, 2002, 17 (06) : 1145 - 1147
[27] X-LINKED ALPORT SYNDROME IN FEMALES: EXPIRIENCE OF ONE CENTER
Aksenova, Marina
Shagam, Lev
Konkova, Nataliya
Dlin, Vladimir
PEDIATRIC NEPHROLOGY, 2018, 33 (10) : 1913 - 1913
[28] Corneal endothelial cell abnormalities in X-linked Alport syndrome
Nicklason, Eleanor
Mack, Heather
Beltz, Jacqueline
Jacob, Julie
Farahani, Mina
Colville, Deb
Savige, Judy
OPHTHALMIC GENETICS, 2020, 41 (01) : 13 - 19
[29] Analyzing three pedigrees in X-linked Alport syndrome with the presentation of nephrotic syndrome
Zhang, Jian-Hui
Liu, Jie
Ruan, Dan-Dan
Chen, Qian
Yang, Jie
Wu, Min
Yu, Hong-Ping
Liao, Li-Sheng
Zheng, Xiao-Ling
Luo, Jie-Wei
Zhang, Li
FRONTIERS IN GENETICS, 2024, 15
[30] PREDICTORS OF KIDNEY DISEASE PROGRESSION IN MALES WITH X-LINKED ALPORT SYNDROME
Aksenova, Marina
Zaikova, Natalia
Piruzeeva, Oxana
Lepaeva, Tatjana
Obuhova, Varvara
Nikishina, Tatjana
Konkova, Natalia
NEPHROLOGY DIALYSIS TRANSPLANTATION, 2023, 38 : I476 - I476

← 1 2 3 4 5 →