2279 DRESS Causing a Subacute Fulminant Hepatitis Secondary to Sulfasalazine

Abstract

INTRODUCTION: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare and life-threatening hypersensitivity reaction most often due to anti-epileptics. It is characterized by a maculopapular rash, eosinophilia, lymphadenopathy, and visceral organ involvement. Here we present a case of DRESS caused by sulfasalazine which resulted in subacute fulminant hepatitis. CASE DESCRIPTION/METHODS: A 24-year-old male with ulcerative colitis was started on sulfasalazine. After being on sulfasalazine for one month, he ran out of the medication. Ten days later, he developed a diffuse pruritic morbilliform rash. He initially presented with the following lab values: total bilirubin 0.7 mg/dL, AST 175 U/L, ALT 354 U/L, alkaline phosphatase 208 U/L, and INR of 1.1. Skin biopsy revealed focal vacuolar interface dermatitis and superficial perivascular lymphocytic infiltrate with neutrophils and eosinophils, consistent with drug eruption. He was started on prednisone 50mg daily and was discharged home. Ten days later, he returned to the hospital with jaundice and the following lab values suggestive of subacute fulminant hepatitis: total bilirubin 6.5 mg/dL (conjugated bilirubin 3.4 mg/dL), AST 3475 U/L, ALT 4171 U/L, alkaline phosphatase 308 U/L, and INR 2.6. Due to the degree of his hepatocellular injury, he was started on prednisone 2mg/kg. The rash, liver chemistries and INR improved over the next week. DISCUSSION: Sulfasalazine is a rare, but known cause of DRESS and idiosyncratic liver injury. This case is notable because he developed a subacute fulminant hepatitis 10 days after initial presentation and 20 days after discontinuation of sulfasalazine. This highlights the fact that liver injury can evolve into acute liver failure despite withdrawal of the culprit drug several days prior; this latency period is characteristic of sulfasalazine induced acute liver injury and thus supports our diagnosis. AST and bilirubin levels are the most important prognostic indicators for liver transplantation and/or death. DRESS due to sulfasalazine with significantly elevated transaminase levels ( >2000 U/L) has been reported, however, to our knowledge, this is the third reported case with this degree of hepatocellular injury that did not result in liver transplantation. Given the fact that acute liver failure is often the cause of mortality in patients with DRESS, this case illustrates that serial lab monitoring is essential in such patients as liver injury can persist long after the offending agent has been discontinued.