Kath. Krankenhaus Hagen, Hagen, Germany.
Klinikum Bogenhausen, München, Germany.
J Crohns Colitis. 2018 Jan 24;12(2):197-203. doi: 10.1093/ecco-jcc/jjx131.
Genital granulomatosis [GG] is a metastatic form of Crohn's disease [CD], characterised by granulomatous inflammation of the genital skin without contact with the gastrointestinal tract. Little is known about GG, as most publications are case reports or small series, and only sporadic in male cases.
Cases of GG were retrospectively collected through the Collaborative Network For Exceptionally Rare case reports project of the European Crohn's and Colitis Organisation.
A total of 43 patients [9 males, 34 females] were diagnosed as having GG, mostly as oedema and/or ulcers. Histological confirmation of granulomas was obtained in 70% of the cases. CD location was colonic or ileocolonic in 97% and perianal disease was documented in 57%. There was no significant difference between males and females in CD phenotype or genital lesions. GG was the first manifestation of inflammatory bowel disease [IBD] in one-third of the patients; these patients were younger at the time of GG occurrence and they all were non-smokers. GG occurred in the absence of gastrointestinal disease activity in 30% of the cases. Ten out of 11 patients [91%] responded to systemic corticosteroid treatment, 5/9 patients responded to immunomodulators, and 9/11 patients responded to anti-tumour necrosis factor alpha [TNF-α] agents.
GG is a rare extraintestinal manifestation of CD. It mainly occurs among women, in the setting of colonic involvement of CD, and perianal disease is often associated. Most cases are successfully managed with systemic corticosteroids or anti-TNF agents.
生殖器肉芽肿病（GG）是克罗恩病（CD）的一种转移性形式，其特征为生殖器皮肤的肉芽肿性炎症，与胃肠道无接触。由于大多数出版物为病例报告或小系列，且男性病例罕见，因此对 GG 的了解甚少。
通过欧洲克罗恩病和结肠炎组织的协作网络对罕见病例报告项目回顾性收集 GG 病例。
共诊断出 43 例 GG 患者[9 例男性，34 例女性]，主要表现为水肿和/或溃疡。70%的病例获得了肉芽肿的组织学证实。97%的 CD 部位为结肠或回结肠，57%有肛周疾病。男性和女性在 CD 表型或生殖器病变方面无显著差异。三分之一的患者 GG 是炎症性肠病（IBD）的首发表现；这些患者在 GG 发生时年龄较小，且均不吸烟。30%的病例 GG 发生在无胃肠道疾病活动的情况下。11 例患者中有 10 例（91%）对全身皮质类固醇治疗有反应，9 例患者中的 5 例（56%）对免疫调节剂有反应，11 例患者中的 9 例（82%）对肿瘤坏死因子-α（TNF-α）拮抗剂有反应。
GG 是 CD 的一种罕见的肠外表现。它主要发生在女性中，在 CD 的结肠受累的情况下发生，并且常伴有肛周疾病。大多数病例可通过全身皮质类固醇或抗 TNF 药物成功治疗。
