S5010 Sulfasalazine-Induced Drug Reaction With Eosinophilia and Systemic Symptoms (DRESS) Syndrome in Ulcerative Colitis

Introduction: Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome or drug-induced hypersensitivity syndrome (DIHS) is a rare and potentially life-threatening adverse drug reaction (ADR). It has a reported prevalence of 2 per 100,000 patients and a mortality rate of 10%, often secondary to fulminant hepatic failure. DRESS/DIHS typically presents 2 to 6 weeks after initiation of the culprit medication, although it can occur earlier with re-exposure. Case Description/Methods: A 34-year-old woman with pancolitis ulcerative colitis was prescribed sulfasalazine. Five weeks later, she presented with intermittent high-grade fevers, chills, bloody diarrhea, and a pruritic rash. The rash first appeared on her neck then spread to her upper torso and face. On admission, she was febrile (39.7°C), tachycardic, and appeared uncomfortable, but had no signs of altered mental status. Facial edema and cervical lymphadenopathy were present. Skin exam was notable for pruritic coalescing erythematous macules and papules on her chest, abdomen, back, arms, and thighs. Labs revealed leukocytosis with eosinophilia, elevated hepatic transaminases, and C-reactive protein 9.30 (0-0.5 mg/dL). Further acute liver injury workup ruled out viral, autoimmune, vascular, and biliary etiologies. Computed tomography abdomen/pelvis with contrast and magnetic resonance imaging with magnetic resonance cholangiopancreatography revealed diffuse colitis without biliary abnormalities. Stool studies showed fecal leukocytes, stool calprotectin 1070 (0-120 ug/g), and negative stool pathogen direct testing. Dermatology was consulted for co-management of DRESS/DIHS. Treatment involved escalating doses of steroids and cyclosporine until laboratory values stabilized. Discussion: This case report describes a typical presentation of a rare ADR following 5 weeks of sulfasalazine for treatment of ulcerative colitis. It is imperative to promptly identify the ADR, remove the culprit medication, and work as a multi-disciplinary team to treat the patient. Careful long-term monitoring for both infectious complications as well as delayed extra-cutaneous manifestations is warranted.