DRESS: Return of the 3-Week Sulfasalazine Syndrome …

Abstract

Introduction: Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) is a medical emergency due to drug-induced hypersensitivity. If not promptly recognized, it carries a mortality rate of 5-10% due to hepatotoxoticity. We present a case of Sulfasalazine-induced DRESS presenting as the classic Three-week sulfasalazine syndrome. Case report: A 21-year-old female with a past medical history significant of ulcerative colitis presented with a one-week history of fever, sore throat and rash despite antibiotic therapy. On examination, she was afebrile with facial edema, cervical lymphadenopathy, and a diffuse maculopapular rash. Blood work demonstrated leukocytosis and elevated liver enzymes. She denied recent travel, excessive alcohol intake/IVDA or sexual activity. Further history revealed the initiation of sulfasalazine three weeks prior. Despite the discontinuation of sulfasalazine, the patient liver function tests worsened. Infectious and autoimmune causes of hepatobiliary pathology were excluded, peripheral smear and CBC with differential demonstrated atypical lymphocytes and eosinophilia. The patient was started on methylprednisolone with improvements of her liver functions and discharged on oral prednisone. Discussion: Three-week sulfasalazine syndrome is a rare drug-induced hypersensitivity reaction characterized by a pentad of fever, lymphadenopathy, dermatitis, hematological abnormalities, and hepatitis around the third week of treatment. The majority of cases can resolve with discontinuation of the drug, however, due to its sepsis-like presentation removal of the offending agent is often delayed. Awareness of this syndrome is prudent not only because it can be fatal, but also its ability to present as a great imitator.