3157 Sweet's Syndrome: A Rare Cutaneous Manifestation of IBD

Abstract

INTRODUCTION: Sweet's syndrome (SS) is one of the rare cutaneous presentations associated with Crohn's disease. SS is characterized by a sudden onset of painful, edematous, and erythematous papules, plaques or nodules on the skin along with fever and neutrophilia. Here we present a case of SS in a patient with Crohn's disease. CASE DESCRIPTION/METHODS: A 29-year-old gentleman with a past medical history of Crohn's disease that was well controlled on mesalamine and infliximab infusions presented with a one week history of numerous erythematous papules on the chest and back and a few pustules around hair follicles on the upper torso. At the time of presentation, he was febrile and had elevated WBCs with a neutrophilic predominance. ESR was 63 and CRP was 11.2. Based on the presentation, he was diagnosed with Varicella infection and treated with Valacyclovir. However, he continued to be febrile with worsening new eruptions. Labs including VZV & HSV PCR, viral cultures, fungal cultures, hepatitis panel, HIV and RPR were negative so the patient underwent a skin biopsy to investigate the etiology of the rash. Biopsy showed diffuse infiltration of the epidermis and dermis by a neutrophil-rich inflammatory cell infiltrate without any signs of leukocytoclastic vasculitis. This was consistent with the histopathological findings seen in Sweet's Syndrome. His antivirals were discontinued and he was started on a high dose prednisone taper with a resultant dramatic improvement in signs and symptoms. Over the next 7 days his rash resolved with normalization of white count and inflammatory markers. DISCUSSION: SS can be clinically classified into malignancy associated SS, drug induced SS or classic (idiopathic) SS most commonly associated with infections, inflammatory diseases or pregnancy. Classic SS usually appears in the active phase of inflammatory bowel disease. Our patient had uncontrolled CD for two years, but he had not had any extraintestinal manifestations during this time. Moreover, Infliximab has been used to treat steroid non-responsive SS. Our patient developed the syndrome even though he was on infliximab. Sweet's Syndrome should be one of the differentials when a patient with IBD presents with painful pustular rash and fever.