Crohn's disease of the vulva: a unique presentation with an unconventional treatment approach

Abstract

Vulva's Crohn's disease (CD) is a rare condition that can be easily misdiagnosed if not thoroughly investigated. The typical presentation of the disease is swelling, deep linear fissures, erythema and painful, and progressive ulceration of the labia. There are various treatment protocols, yet none of them have been established as gold standard. Surgery is not recommended, and medical treatment is the current standard of care. We aim to report an unusual presentation of vulvar CD and discuss the treatment option of surgery, a valuable alternative when medical therapy yields ineffective results. A 36-year-old patient, whose initial symptoms had begun 7 years ago, presented with labial fistulas and multiple tissue papillomas. During the disease course, the patient had previously received corticosteroid and antibiotic treatments of limited benefit. Vulvar hypertrophy was significant, and papilloma formation related to the extension of the knife-like ulcers had left the outer genitals dysmorphic. The patient underwent two consecutive partial vulvectomies with 1 year apart, along with oral moxifloxacin treatment. Partial vulvectomies reduced the amount of hypertrophic tissues and papillomas and allowed for asymptomatic urine output. The patient also had psychological relief and experienced less intervening intertriginous fungal infections. Due to the ongoing inflammatory process of CD, symptomatic and clinical relief by stabilizing the condition is of utmost importance. Although medical treatment is frequently sufficient to stabilize symptoms of vulvar CD, surgery certainly reserves a place in the treatment algorithm when the patient is unresponsive to other treatment modalities.