Bourrat E, Faure C, Vignon-Pennamen M D, Rybojad M, Morel P, Navarro J
Service de Dermatologie, Hôpital St Louis, Paris.
Ann Dermatol Venereol. 1997;124(9):626-8.
We report a case of oro-genital and perianal Crohn's disease which progressed for 4 years with no digestive involvement in a 12-year-old girl.
At the age of 8 years, a young girl developed recurrent anal fissures. Voluminous vulvar edema developed at 12 years with fissurar macrocheilitis. There were no digestive signs and the diagnosis of Crohn's disease was obtained on the basis of granulomatous epithelioid infiltration of biopsy specimens (Bauhin valve, anus, vulva). Metronidazole given per os at the dose of 25 mg/kg/day for 6 months led to partial significant symptomatic remission.
Unilateral or bilateral vulvar edema is highly suggestive of Crohn's disease even if the classical digestive inflammatory signs are absent. Demonstration of perianal lesions (erythema, pseudocondylomatous formations) must not mislead the diagnosis (sexual abuse). Simultaneous granulomatous lesions in the genital and labial regions is exceptional in Crohn's disease. Ileocolonoscopy is indicated in such cases and alone can demonstrate latent digestive inflammatory processes. Different agents have been proposed for treatment of local Crohn's disease skin lesions. It is difficult to evaluate their efficacy due to the spontaneous variability of disease expression.
我们报告一例口生殖器及肛周克罗恩病病例,该病例发生在一名12岁女孩身上,病程长达4年,无消化系统受累情况。
该女童8岁时出现复发性肛裂。12岁时出现大量外阴水肿,并伴有裂隙性巨唇炎。无消化系统症状,根据活检标本(回盲瓣、肛门、外阴)的肉芽肿性上皮样浸润确诊为克罗恩病。口服甲硝唑,剂量为25mg/kg/天,持续6个月,症状部分显著缓解。
即使没有典型的消化系统炎症体征,单侧或双侧外阴水肿也高度提示克罗恩病。肛周病变(红斑、假性湿疣形成)的表现不能误诊为性虐待。生殖器和唇部区域同时出现肉芽肿性病变在克罗恩病中较为罕见。在此类病例中应进行回结肠镜检查,其本身就能显示潜在的消化系统炎症过程。已提出多种药物用于治疗局部克罗恩病皮肤病变。由于疾病表现的自然变异性,很难评估它们的疗效。