General Medical Information

Vulvar Manifestations of Systemic Disease

Behçet’s disease is a chronic, recurrent, inflammatory disease of unknown etiology that involves multiple systems and mucous membranes. Diagnosis is based on the presence of oral and genital ulcers and at least two of the following clinical findings: uveitis, cutaneous vasculitis, arthritis, meningoencephalitis, or cutaneous hyperreactivity to minor trauma. Recurrences and exacerbations characterize the disease. Ulcerations usually are painful and often occur on the labia minora. The ulcers usually are less than 1 cm in diameter and have a yellow, necrotic center with peripheral edema. Genital ulcerations tend to be less painful than the oral ones. In Behçet’s disease with childhood onset, the average time interval between the onset of oral ulcerations and the appearance of genital ulcers is around 9 years. Perianal aphthosis appears to be a specific feature of childhood Behçet’s disease. Manifestations of Behçet’s disease are similar in adults and children, but the course of the condition seems to be less severe in children. Systemic corticosteroids often are needed. Ulcers generally resolve in 1 to 2 weeks but may heal with scar formation. Thalidomide 100 mg/day has been shown to be effective for treating the oral and genital ulcers as well as follicular lesions of Behçet’s disease in adults. An infant with features of Behçet’s disease who did not respond to corticosteroids and cytotoxic agents had marked improvement and resolution of symptoms when treated with thalidomide. Sucralfate suspension application to the oral and genital ulcers has been shown to significantly decrease frequency, healing time, and pain.

Crohn’s disease is a chronic granulomatous disease that primarily affects the terminal ileum and can involve the entire gastrointestinal tract. The skin is the most common site of extraintestinal manifestation. Common genital features of Crohn’s disease in women include abscesses, fistulas, ulcers, fissures, and infections of the internal pelvic organs as well as the vulva, vagina, and perineum. A vulvar lesion may be an extension of an anal lesion or can arise separately (metastatic). Children and adolescents may present vulvar lesions several months before the development of gastrointestinal symptoms. Initial lesions may present as labial hypertrophy or vulvar swelling. Metastatic lesions may present as vulvar swelling or ulcerations. Perianal manifestation of Crohn’s disease is associated with severe, mutilating tissue destruction and is refractory to treatment. When the manifestations are limited to the vulva, treatment is oral metronidazole with local symptomatic relieve measures. Topical or systemic corticosteroids may be needed.