A case of idiopathic granulomatous cheilitis and vulvitis

A 36-year-old woman presented with chronic recurring dermatitis of the vulva, perineum, and lips. The genital lesions had a 3-year history and were associated with slight pruritus and occasional pain. The lesions of the upper lip had a history of 6 months. The patient had been treated with cycles of antimycotics and topical steroids which only partially controlled the symptoms during treatment. Dermatologic examination showed erythematous, infiltrative dermatitis with edema of the labia majora and persistent edema of the upper lip (Fig. 1a,b). Routine blood chemistry, urine analysis, and chest X-ray were normal. Microscopic examination and cultures of vaginal swabs did not reveal any pathogenic bacteria or fungi. Histologic examination of a biopsy of vulval lesional skin showed lichenoid lymphocytic infiltration of the papillary dermis and small, nonnecrotic epithelioid granulomas in the deep dermis (Fig. 1c,d). No microorganisms, including acid-fast bacilli or fungi, were identified. Culture was negative for fungi. Polymerase chain reaction was negative for the mycobacterial genome. Histologic examination of a biopsy from the upper lip showed similar results. The pathology reports of both regions were compatible with a diagnosis of granulomatous cheilitis and vulvitis. To investigate concomitant asymptomatic inflammatory bowel disease, the patient underwent colonoscopy with retrograde ileoscopy and gastroscopy, which were both negative. The patient refused radiographic examination of the small intestine with a barium meal. The patient was treated with systemic metronidazole (500 mg/day). After 6 months of therapy, the upper lip showed significant improvement and erythema and desquamation in the genital area showed slight improvement, but genital edema was unaffected.