Rare, but Serious Extra-intestinal Complication of Inflammatory Bowel Disease

Abstract

Bowel-associated dermatosis-arthritis syndrome (BADAS) has been linked to Inflammatory Bowel Disease. Classic prodrome includes fever, chills, myalgias, and polyarthralgias that later evolve into inflammatory papules similar to Sweet Syndrome. The papules consist of neutrophilic infiltrate without vessel wall destruction, differentiating it from other neutrophilic dermatoses. We present a case of a patient with Ulcerative Colitis who also underwent gastric sleeve surgery and presented with BADAS. A 38 year-old Caucasian female presented with diffuse rash for one-week duration. Patient reported fever, chills and myalgias for two days. The rash, consisting of blisters and pustules, erupted initially on her face and spread to her trunk and extremities. She denied pruritus, but did report a burning and stinging sensation. Arthralgias were also noted in bilateral shoulders and knees. She denied any changes in bowel habits or recent illness. The patient had previously been on infliximab, but was discontinued approximately six months earlier. The patient was febrile (101.8F), tachycardic (113 bpm), and hypertensive (150/78 mmHg) on admission. She was in slight distress secondary to symptom burden. On examination, her skin demonstrated multiple pustules with peripheral erythema and crusting. These pustules were noted on her head, face, upper and lower extremities sparing the palms and soles. The rash was non-tender. On admission, her white blood cell count was 16.1 (TH/mm3) hemoglobin 13.1 (g/dL), platelets 341 (TH/mm3), total protein 7 (g/dL), AST 50 (U/L), ALT 62 (U/L), CRP 11.13 (mg/dL), ESR 60 (mm/hr). Her pregnancy, HIV, Hepatitis panel, direct streptococcal antigen, mononucleosis antibody, and mycoplasma IgM testing were all negative. Skin biopsy demonstrated mature neutrophilic infiltrate. This involved the dermis and caused papillary dermal edema. There was no noted fibrinoid necrosis or infarction of the vessels. This was consistent with diagnosis of BADAS given the patient's history of UC. The patient was treated with valacyclovir, steroids, anti-histamine agents and responded well. Our case demonstrates extra intestinal manifestation of IBD that is often not considered in differential diagnosis. Our goal is to bring awareness due to its common recurrence and prompt early treatment initiation for resolution.2048_A Figure 1. Abdomen, punch biopsy. Suppurative folliculitis with a ruptured follicle.2048_B Figure 2. Abdomen, punch biopsy demonstrating neutrophilic infiltrate. Suppurative folliculitis with a ruptured follicle.2048_C Figure 3. Abdomen, punch biopsy. Suppurative folliculitis with a ruptured follicle.