P040 Vasculitis in a patient with refractory Crohn's disease

Abstract

BACKGROUND: Crohn's disease (CD) is associated with a variety of cutaneous manifestations, many of which parallel disease activity. Leukocytoclastic vasculitis (LV) is a rare and underdiagnosed cutaneous manifestation of CD. CASE: A twenty-year old woman presented to our unit with a rash affecting her lower extremities eleven days after ileocolic resection and ileostomy for refractory CD. Her past medical history included the diagnosis of penetrating CD for 3-years (anti-TNF refractory). Investigations revealed: normal full blood count and electrolytes, negative serologies (HIV, HBV, syphilis, HCV), normal C-reactive protein (CRP), negative rheumatoid factor, negative anti-nuclear antibodies (ANA), positive antineutrophil cytoplasmic autoantibody (ANCA). Skin biopsy diagnosed LV, characterized by angiocentric neutrophilic infiltrate with normal epidermis and no granulomata or fibrinoid necrosis. DISCUSSION: LV is an immune-complex syndrome characterized by palpable purpura commonly located in the lower limbs and ankles. It is a rare skin manifestation of CD. Lesions range from 1mm to several centimeters. The condition may also present as urticarial papules, nodules, vesicles, plaques, hemorrhagic bullae and necrotic ulcers. Fever, arthralgia, myalgia and ankle swelling may be present. It is often associated with active intestinal disease. It is believed that direct exposure of immune cells of inflamed intestinal mucosa to fecal antigens may lead to formation of immune complexes, deposited in vessel walls causing vasculitis. LV may also be triggered by medications, infections, surgery, chemicals and other autoimmune diseases. Our patient improved spontaneously after few weeks of surgery. During follow-up, she had no signs of skin lesions or intestinal flares. CONCLUSION: LV is rare a small-vessel vasculitis that may be observed in inflammatory bowel disease patients. Treatment of the predisposing condition is essential in patients with skin-limited disease.