S2635 A Unique Case of Neutrophilic Dermatitis (Sweet Syndrome) Associated With Primary Sclerosing Cholangitis: Case Report and Literature Review

Abstract

INTRODUCTION: We present a unique case of Sweet Syndrome associated with primary sclerosing cholangitis. CASE DESCRIPTION/METHODS: A 39-year-old woman presented to the emergency department with diffuse necrotic skin lesions and recent uveitis. Her inflammatory markers were elevated. Serologic studies showed a positive ANA titer of 1:320 and p-ANCA titer of 1:80. A full-thickness skin biopsy revealed dense neutrophilic inflammation without evidence of vasculitis. Tissue cultures were negative. She was started on oral corticosteroids with complete resolution. She was found to have an elevated alkaline phosphatase of 677 U/L. Magnetic resonance cholangiopancreatography (MRCP) revealed intrahepatic ductal dilation in an irregular beaded pattern consistent with primary sclerosing cholangitis. She did not have any gastrointestinal symptoms and had a normal previous colonoscopy. DISCUSSION: Sweet Syndrome is an inflammatory condition characterized by the abrupt eruption of cutaneous lesions. Characteristic findings on histology include prominent edema in the dermis, a dense neutrophilic infiltrate, and endothelial swelling without vasculitis. The most common extra-cutaneous manifestation is uveitis. The pathogenesis of Sweet syndrome is unclear but has been postulated to be associated with immune and cytokine dysregulation. Sweet syndrome has been associated with multiple disorders including infections, inflammatory bowel disease (IBD), autoimmune, pregnancy, malignancies, and certain prescription medications. To our knowledge, this is the first described case of Sweet Syndrome associated with primary sclerosing cholangitis in a patient without IBD.Figure 1.: Skin Lesion.Figure 2.: Histology.Figure 3.: MRCP.