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成功的多学科治疗外阴克罗恩病的罕见病理学。

Successful multidisciplinary treatment of the rare pathology of vulvar Crohn's disease.

机构信息

Department of Medicine, University of Florida, Gainesville, Florida, USA

Department of Medicine, University of Florida, Gainesville, Florida, USA.

出版信息

BMJ Case Rep. 2023 Oct 16;16(10):e256343. doi: 10.1136/bcr-2023-256343.

Abstract

A woman in her 50s with a medical history of rheumatoid arthritis (RA) and hypothyroidism presented with 3 months of vaginal pain and swelling. Physical examination showed vulvar oedema and erythema, ulcerations of the inferior vulva that extended to the buttocks and introitus with erythema and superficial erosions. MR pelvis showed a fibroid in the uterus but otherwise was unremarkable. Biopsies of the vagina showed extensive acute and chronic inflammation, necrosis and granulomas without concern for malignancy, which was consistent with a diagnosis of cutaneous Crohn's disease. MR enterography, upper endoscopy and colonoscopy revealed no gastrointestinal manifestations of Crohn's. She was treated initially with corticosteroids and metronidazole as well as vulvectomy and graft placement. Azathioprine was added to her regimen due to lack of response; however, despite 6 weeks of this therapy, the ulcerations did not heal thus infliximab was initiated. After 8 weeks the lesions had completely resolved.

摘要

一位 50 多岁的女性,有类风湿关节炎(RA)和甲状腺功能减退症的病史,出现阴道疼痛和肿胀 3 个月。体格检查显示外阴水肿和红斑,外阴下部溃疡延伸至臀部和阴道口,伴有红斑和浅表糜烂。盆腔 MRI 显示子宫内有肌瘤,但其他方面无异常。阴道活检显示广泛的急性和慢性炎症、坏死和肉芽肿,但无恶性肿瘤的迹象,这与皮肤克罗恩病的诊断一致。磁共振肠造影、上消化道内镜和结肠镜检查未发现克罗恩病的胃肠道表现。她最初接受了皮质类固醇和甲硝唑治疗,以及外阴切除术和植皮。由于缺乏反应,加用了硫唑嘌呤;然而,尽管接受了 6 周的治疗,溃疡仍未愈合,因此开始使用英夫利昔单抗。治疗 8 周后,病变完全消退。

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本文引用的文献

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Histopathology of Hidradenitis Suppurativa: A Systematic Review.化脓性汗腺炎的组织病理学:一项系统综述。
Dermatopathology (Basel). 2022 Jul 14;9(3):251-257. doi: 10.3390/dermatopathology9030029.
2
Vulvar Crohn's Disease: Clinical Features and Outcomes.外阴克罗恩病:临床特征和结局。
Am J Gastroenterol. 2021 Nov 1;116(11):2296-2299. doi: 10.14309/ajg.0000000000001502.
6
Clinical experience and diagnostic algorithm of vulval Crohn's disease.外阴克罗恩病的临床经验与诊断算法
Eur J Gastroenterol Hepatol. 2017 Jul;29(7):838-843. doi: 10.1097/MEG.0000000000000879.
8
Vulval Crohn's disease: a clinical study of 22 patients.外阴克罗恩病:22例患者的临床研究
J Crohns Colitis. 2015 Apr;9(4):318-25. doi: 10.1093/ecco-jcc/jjv037. Epub 2015 Feb 16.
9
Crohn's disease of the vulva.外阴克罗恩病
J Crohns Colitis. 2014 Jul;8(7):563-70. doi: 10.1016/j.crohns.2013.10.009. Epub 2013 Nov 16.

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