Barret Maximilien, de Parades Vincent, Battistella Maxime, Sokol Harry, Lemarchand Nicolas, Marteau Philippe
Department of Gastroenterology and Endoscopy, Hôpital Européen Georges Pompidou, Assistance Publique-Hôpitaux de Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Faculté de médecine, Paris, France.
Department of Medical and Surgical Proctology, Groupe Hospitalier Saint-Joseph, Institut Léopold Bellan, Paris, France.
J Crohns Colitis. 2014 Jul;8(7):563-70. doi: 10.1016/j.crohns.2013.10.009. Epub 2013 Nov 16.
Crohn's disease (CD) of the vulva is a rare, yet under recognized condition. Fistulae arising from the digestive tract account for the greater part of genital lesions in CD. However, cutaneous so-called metastatic lesions of the vulva have been reported in the literature. They are clinically challenging for gastroenterologists as well as for gynecologists, with numerous differential diagnoses, especially among venereal diseases, and require a multidisciplinary approach. The most frequently observed features of the disease are labial swelling, vulvar ulcers, and hypertrophic lesions. Biopsy samples for histological study are mandatory, in order to establish the diagnosis of vulvar CD. Treatment options include oral prolonged courses of metronidazole and systemic immunosuppressive therapy such as corticosteroids and azathioprine, with promising data published on the efficacy of infliximab. Surgery remains restricted to medical treatment failures or resection of unsightly lesions. Prospective studies or case series with long follow-up data are still missing to guide the treatment of this condition.
外阴克罗恩病（CD）是一种罕见但未得到充分认识的疾病。消化道瘘是CD生殖器病变的主要原因。然而，文献中已报道了外阴皮肤所谓的转移性病变。它们对胃肠病学家和妇科医生来说在临床上都具有挑战性，存在众多鉴别诊断，尤其是在性传播疾病中，并且需要多学科方法。该疾病最常观察到的特征是阴唇肿胀、外阴溃疡和肥厚性病变。为了确诊外阴CD，进行组织学研究的活检样本是必不可少的。治疗选择包括长期口服甲硝唑以及全身性免疫抑制治疗，如皮质类固醇和硫唑嘌呤，关于英夫利昔单抗疗效的有前景的数据已发表。手术仍仅限于药物治疗失败或切除难看的病变。目前仍缺乏前瞻性研究或有长期随访数据的病例系列来指导这种疾病的治疗。
