A 58 year-old Caucasian female presented with a history of painful lesions of the gluteal, perineal, and vulvar areas. These lesions had been progressive with worsening drainage and scar formation over the last decade. She was treated with multiple antibiotics and antifungals in the past without improvement. No lesions were seen in her axilla. She denied fevers, chills, weight loss, back pain, joint pain or fecal incontinence. Her past medical history was significant for Crohns disease complicated by rectal fistulas. She has been treated with systemic steroids, mesalamine, azathioprine, and infliximab. Physical exam revealed marked scar tissue and thickened skin with purulent drainage at the perineal, perianal, and inguinal areas (Figure 1A). Given the extent of her skin lesions, an interdisciplinary team was consulted including plastic, gynecology, and general surgery. She underwent wide local excision involving 20 cm of each buttock with complex closure and 15 by 8 cm of the right vulva including the mons pubis and 12 by 4 cm of the left vulva. The histopathology of the excised lesions showed chronic granulomatous inflammation with sinus formation (Figure 1B). Postoperatively, she treated with a week of vancomycin followed by a course of oral trimethoprim-sulfamethoxazole.
Discussion:
Cutaneous manifestations are present in 20-30% of patients with Crohns disease. Cutaneous manifestations of Crohns disease can be classified into two histologic groups: granulomatous inflammation and reactive lesions. The former or Crohns disease specific lesions include perianal fissures and fistulas, oral aphthous ulcers, granulomatous cheilitis and metastatic Crohn disease (MCD). Reactive lesions or those that arise from immunological reactivity to antigens include pyoderma gangrenosum, erythema nodosum, hidradenitis suppurativa, leucocytoclastic vasculitis and Sweet’s syndrome. The metastatic Crohns disease was first described in 1965 and area under her breast, metastatic from the gastrointestinal tract. MCD is rare and with has been associated with a variety of cutaneous manifestations. Diagnosis is made upon histology findings of noncaseating granulomas in the dermis not contiguous with the gastrointestinal tract in a patient with Crohns disease. Pathophysiology likely involves maladaptive immune response resulting in disruption of mucosal homeostasis and hosting intestinal microflora. The lesions manifest on the flexural areas including retroauricular, vulvar, submammary and groin. Given the rarity of MCD, there are no definitive guidelines for treatment. Topical with or without systemic steroids are used for localized disease. Refractory or severe disease, such as this patient will require high-dose steroids and immunomodulatory. Severe and refractory cases of both HS and MCD have been treated with anti-tumor necrosis factor α agent such as infliximab. Recurrence rates are reduced with radical excision versus incision and drainage. It is widely acceptable to opt for early surgical intervention among patients with refractory and severe disease given its significant association with physical and psychosocial morbidities.
Conclusions:
Perianal and peristomal granulomatous inflammatory lesions are frequently observed in patients with Crohns disease. When occurring at sites noncontiguous with the gastrointestinal tract, they are termed metastatic Crohns disease. In severe cases, a multimodality treatment approach is necessary.