Sulfasalazine Induced DRESS Syndrome: A Review of Case Reports

Introduction: Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) is designated as a lethal adverse drug effect with characteristic sign and symptoms such as skin rashes, fever, leukocytosis with eosinophilia or atypical lymphocytes, lymph node enlargement, and liver or renal dysfunction. Incidences of the DRESS range from 1/1000-1/10,000 drug exposures and are associated with a mortality rate of 10%. Pathogenesis of DRESS relates to an abnormal immune response in a genetically vulnerable individual, i.e. presence of human leukocyte antigen (HLA)*5801 and HLA-B* 5701 genotype and slow acetylation metabolic pathways.

Methods: 48 cases were associated with the “Sulfasalazine-induced DRESS syndrome” reported between January 1990- March 2015 in PubMed-MEDLINE and HighWire Press. The “RegiSCAR” scoring system was used to analyze the case reports. Using this system, cases were classified into 4 categories as “no”, “possible, “probable” and “definite”.

Results: The vast majority of cases were classified as “probable/definite” DRESS cases (83%). Hypereosinophilia, atypical lymphocytes and fever were significantly associated with “probable/ definite” DRESS cases. Liver involvement and skin rash was described in almost all of the cases, including “possible cases”. DRESS was found fatal in two cases.

Conclusion: Awareness of DRESS is essential for diagnosis with the presence of skin rash, liver involvement, fever, hyper eosinophilia and lymphadenopathy. Early identification, followed by a prompt withdrawal of the culprit drug is the most essential measure to avoid disease evolution and to restore wellness.