Anitis, vulvar edema and macrocheilitis disclosing Crohn disease in a child: value of metronidazole

BACKGROUND: 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. CASE REPORT: 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. DISCUSSION: 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.