S3333 An Unusual Case of Drug Reaction With Eosinophilia and Systemic Symptoms Presenting as Cholestasis

Introduction: Drug Reaction with Eosinophilia and Systemic Symptoms, or “DRESS” syndrome, is a rare and severe drug-induced reaction that can be difficult to diagnose due to the variability in presentation and organ involvement. DRESS often presents a morbilliform cutaneous eruption with fevers, lymphadenopathy, hematologic abnormalities, and multiorgan involvement. The pathophysiology of DRESS involves drug metabolic defects, viral re-activation, and eosinophilia. Our case highlights an unusual presentation of DRESS in a woman with abdominal pain and jaundice. Case Description/Methods: A 65-year-old woman with past medical history of type 1 diabetes presented to the emergency department for abdominal pain and fevers. She was tender around the umbilicus, had scleral icterus, and had a diffuse maculopapular rash. Labs showed a total bilirubin of 6.2, direct bilirubin of 4.8, AST 221, ALT 206, ALP 2175, eosinophilia of 26%. Computed tomography of the abdomen and pelvis showed diffuse lymphadenopathy. She was admitted for suspected post-hepatic obstructive jaundice. Endoscopic ultrasound revealed multiple pathologically enlarged lymph nodes up to 33 mm in size, without evidence of biliary obstruction. Core biopsy from a lymph node showed polymorphic lymphoproliferative disorder with cells positive for Epstein-Barr Virus (EBV). Liver biopsy showed portal inflammation with EBV hepatitis. Skin and bone marrow biopsies were consistent with drug reaction and eosinophilia. The constellation of these findings was suggestive of DRESS syndrome, and the patient was started on prednisone with clinical improvement. Discussion: DRESS syndrome is a potentially fatal condition caused by a drug hypersensitivity reaction involving multiple organs, especially the liver. Our patient presented with vague abdominal symptoms, a rash, and jaundice, which yielded a broad differential. This patient also had positivity for EBV, raising the concern for EBV hepatitis being the leading diagnosis. However, in the presence of non-obstructive cholestasis, diffuse lymphadenopathy, and positive EBV, DRESS syndrome had to be considered due to its high rate of mortality of up to 10%. The involvement of multiple consultants early and obtaining biopsies of the skin, liver, lymph node, and bone marrow helped diagnose this condition promptly and assisted in further management. This case emphasizes the complexity of diagnosing DRESS syndrome and the need to consider it when evaluating patients with overlapping features.