S66 Leukocytoclastic Vasculitis in a Crohn’s Disease patient with recent Sars-CoV2 infection

Abstract

Case: Prior to the COVID-19 pandemic, leukocytoclastic vasculitis (LCV) had been described in association with a variety of chronic infections, autoimmune disorders, and medications. In the inflammatory bowel diseases (IBD) population, LCV has been reported as a rare cutaneous, extraintestinal manifestation, which may be related to the disease itself or associated with the use of biologic medications, particularly those that target tumor necrosis factor. Several cases of LCV related to infection with SARS-CoV2, not related to IBD, have been reported. We present a case of LCV in a woman with Crohn’s Disease (CD) who was on long-term maintenance infliximab and presented with purpuric papules on bilateral lower extremities shortly after infection with SARS-CoV2. A 37-year-old female with a 26-year history of ileocolonic CD in remission for at least 2 years presents to our IBD clinic with concern of rash on both lower extremities, accompanied by significant ankle pain. She had been on infliximab for 10 years. The symptoms began 1 month prior, 2 months after being diagnosed with COVID-19. She denied any concurrent GI symptoms that were new or worsened from her baseline. Lab evaluation was notable for positive ANA, elevated to 1:160. She had normal ANCA, anti-myeloperoxidase antibody, anti-dsDNA antibody, total IgG and total IgM, and rheumatoid factor. Skin punch biopsy showed fibrinoid necrosis of capillary walls within the dermis with surrounding neutrophils and karyorrhectic debris, findings consistent with LCV. Colonoscopy showed complete endoscopic remission of CD. An oral steroid taper lead only to temporary improvement and symptoms recurred at a dose of 10 mg prednisone. Due to suspicion of infliximab-associated LCV, infliximab was discontinued, and ustekinumab therapy was initiated. She was able to taper off steroids, and the rash completely resolved. LCV falls within a general category of cutaneous small vessel vasculitis; it is limited specifically to the skin, with no systemic or renal involvement. Patients typically present with palpable purpura, at times associated with urticaria. When IBD patients present with these findings, providers should review recent infectious symptoms, medication changes or drug exposure. Direct immunofluorescence should be performed on skin biopsy for lesions lasting >= 4 weeks. For symptoms < 4 weeks, conservative therapy with oral anti-histamine, and compression stockings have been effective. Implicated drugs should be stopped. For severe cases, systemic therapy with prednisone is recommended; dapsone or colchicine have also been used. When LCV has been discovered in the setting of COVID infection, treatment approaches have been varied, including topical and oral steroids, low molecular weight heparin, and intravenous immunoglobulin (IVIG). Prior to this case, there has been one case describing successfully treatment of LCV in CD with IVIG. Because this is a rare entity, no randomized controlled trials have been performed to evaluate safety or efficacy of specific therapies. While rare, LCV is an important pathology that IBD providers should be comfortable diagnosing and may only increase as COVID-19 remains common. Importantly, we must be aware that management of LCV will likely involve change in therapy for IBD, as medications are often implicated.