Crohn's disease of the vulva

Abstract

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.

Crohn’s disease presenting as vulvar edema in a 15-year-old girl

“…In our case, erythema and edema of the labium majus were present, but there was no ulcer or architectural destruction in the vulval region. [5][6][7][8][9]10 The lesion in the vulva of our patient was a "metastatic" CD. As in our patient, perianal lesions accompany 90% of cases.…”

“…[5][6][7][8][9] The recovery of skin lesions is variable and does not parallel that of intestinal lesions. In our patient, treatment of vulval CD was disappointing with corticosteroid and mesalamine.…”

“…[5][6][7][8][9] Patients with vulval CD typically present with erythema and edema of the labia majora, but soon progress to extensive ulcer formation. 6 The labia majora and clitoris are involved in most cases, and there may be gross architectural destruction of the vulval region.…”

Vulvar inflammation as a manifestation of Crohn's disease

“…[1,2] Genital complications, such as vulvar lesions, however, are very rare. [3] Fewer than 200 cases of vulvar Crohn's disease have been reported in the literature since it was first described by Parks et al [4,5] in 1965. The development of vulvar lesions may occur due to the contiguous spread of Crohn's disease (i.e.…”

“…[11] In most cases, the diagnosis of vulvar Crohn's disease is confirmed on histopathology by the presence of inflammatory infiltrates, epidermal ulceration, and noncaseating granulomas. [5] Treatment recommendations for vulvar Crohn's disease have not been well established, as the natural course of disease is unpredictable. While some lesions resolve spontaneously, others fail both medical management and surgical debridement.…”