2070 Sweet's Syndrome as the Initial Presentation of Crohn's Disease

Abstract

INTRODUCTION: The diagnostic criteria for Sweet's syndrome include fever, peripheral neutrophilia, erythematous skin lesions and diffuse neutrophilic dermal infiltrate. Classically, the lesions and symptoms promptly improve with the initiation of systemic corticosteroids. This is a rare case of Sweet’s syndrome as the initial presentation of Crohn’s disease. CASE DESCRIPTION/METHODS: A 23-year-old African-American male with no past medical history presented to our ED with fever and rash. He reported a sudden onset mildly sensitive rash on his bilateral lower extremities, preceded by two weeks of fever, myalgia and headaches. He denied arthralgia but endorsed recent weight loss and sporadic aphthous ulcers. He developed watery, non-bloody diarrhea in the ED. His abdomen was soft and non-tender. Examination of the rash revealed erythematous nodules, some studded with pustules and a few ulcerated nodules. He was febrile to 102.7 ºF with tachycardia. Labs were significant for leukocytosis at 22.5 (68% neutrophils), thrombocytosis at 515, microcytic anemia (Hb 9.6), elevated inflammatory markers (CRP 326 mg/L; ESR >130 mm) and AST:ALT 64:41 U/L. Broad spectrum antibiotics were started due to concerns for sepsis. Blood, wound, and stool cultures, including Clostridium difficile were all negative. Fecal calprotectin was 32. His immunological workup showed ANA, RF and anti-GBM negative; normal IgM, IgG, IgA, C3 and C4. HIV, Hepatitis B and C and QuantiFERON were also negative. ANCA was positive at 1:320, with positive PR3. CT scan of the abdomen showed proctitis and ascending colitis with no lymphadenopathy. Colonoscopy showed mild-moderate inflammation of the left sided colon with skip lesions in the transverse colon, without terminal ileum involvement, with biopsies showing focal acute cryptitis and rare crypt abscesses. Skin biopsy revealed neutrophilic dermatitis and panniculitis without vasculitis. Once infection was ruled out, prednisone 60 mg was started and the rash and fever rapidly improved. DISCUSSION: Sweet’s syndrome is considered part of the family of neutrophilic dermatoses, which also includes pyoderma gangrenosum, and is considered an unusual extra-intestinal manifestation of IBD. Sweet's syndrome is also associated with malignancies, infections, pregnancy and medications. The temporal relationship between Sweet's syndrome and IBD activity is variable, but the majority of cases present after the diagnosis of IBD, with few cases in the literature with Sweet’s syndrome as the presenting symptom for IBD.