Vulvar Crohn's Disease in an Elderly Woman

A 72-year-old woman presented with a two-year history of intermittent burning, pain, and redness of the labia majora. She attributed these symptoms as a result of irritant dermatitis from her history of Clostridioides difficile diarrhea. She reported frequent gastrointestinal upset, anal fissures, and hemorrhoids, but denied a history of inflammatory bowel disease (IBD). Her colonoscopy 5 years prior was unremarkable. Prior treatment with triamcinolone acetonide 0.1% ointment led to partial improvement. Physical examination was notable for bilateral labia majora erythema and swelling, more prominent on the right (Figure 1A) alongside superficial perianal fissuring and large external hemorrhoids. A skin biopsy was recommended at initial evaluation, though the patient deferred and wanted to trial empiric treatment with mupirocin 2% ointment and betamethasone augmented dipropionate 0.05% ointment each twice a day, and oral fluconazole 200 mg weekly for 2 weeks, resulting in partial improvement. She was also urgently referred to a gastroenterologist for a repeat colonoscopy. Colonoscopy revealed active chronic colitis with granuloma and was negative for dysplasia. Subsequently, a vulvar skin biopsy of the right labia majora confirmed granulomatous dermatitis (Figure 2). These clinical and histopathological findings were consistent with a diagnosis of cutaneous Crohn's disease (CD). The patient was started on mesalamine suppositories for colitis and continued betamethasone augmented dipropionate 0.05% ointment twice a day. Oral mesalamine 500 mg was added with significant reduction in vulvar erythema, edema, and discomfort and stabilization of her skin disease within 3 months (Figure 1B). The patient infrequently uses topical betamethasone and mupirocin for vulvar skin symptoms as needed for burning and fissures. CD is characterized by granulomatous, transmural inflammation of the gastrointestinal mucosa, with up to 50% of patients experiencing extraintestinal manifestations; the skin is among the most common sites [1]. Cutaneous lesions may represent an associated condition, intestinal complication, reactive process, treatment effect, or metastatic disease [1]. Vulvar CD is a rare metastatic manifestation that can precede intestinal involvement in 20%–36% of cases [2]. Though CD typically presents in younger adults, 10%–15% of cases arise in older patients, potentially delaying recognition when extraintestinal manifestations predominate [2]. Its variable presentation, non-specific symptoms, and sensitive anatomical location likely contribute to underestimation of true prevalence. Manifestations include pruritus, erythema, pain, erosions, “knife-cut” ulcerations, asymmetrical labial swelling from impaired lymphatic drainage, and perianal tags; severe disease may involve fistulae [2, 3]. Untreated disease can result in lymphatic destruction, recurrent abscess and cellulitis, disfigurement, and squamous cell carcinoma [2]. As vulvar CD can mimic other conditions and present without gastrointestinal symptoms, tissue biopsy demonstrating non-caseating granulomas is essential for diagnosis. With limited data and no established guidelines, treatment of vulvar CD remains challenging and requires an individualized approach using systemic and topical therapies. Topical, intralesional, and oral corticosteroids are typically first-line [2]. Alternative or adjunct therapies, including metronidazole, azathioprine, methotrexate, mycophenolate mofetil, sulfasalazine, infliximab, certolizumab, and adalimumab, are efficacious in select cases [2]. Although mesalamine is rarely used in CD, few cases suggest potential benefit in vulvar CD. For instance, an 18 year-old woman showed improvement in vulvar lesions within 6 weeks of treatment with azathioprine 50 mg daily and mesalamine 1 g three times daily [4]. Similarly, a 15 year-old girl with painful vulvar edema, erosions, perianal skin tags experienced significant improvement in vulvar and intestinal disease after 3 months of topical clobetasol and oral mesalamine [5]. Treatments remained individualized, with close monitoring to optimize outcomes. Multidisciplinary efforts among dermatologists, gynecologists, and gastroenterologists may improve diagnostic delay and strengthen clinical outcomes in vulvar CD. The authors have nothing to report. The authors declare no conflicts of interest. The data that support the findings of this study are available from the corresponding author upon reasonable request.