Sulfasalazine Induced DRESS (drug reaction with eosinophilia and systemic symptoms) syndrome with severe acute hepatitis: Case report

Abstract

Introduction: Drug Reaction with Eosinophilia and Systemic Symptoms Syndrome is a rare and severe form of drug induced delayed-type hypersensitivity reaction with mortality rate up to 10%. It usually manifests with skin rash, fever, lymphadenopathy, hematological abnormalities and involvement of one or more internal organs. Establishing the diagnosis is sometimes late due to variable clinical presentation. Current recommendations for therapy rely mainly on expert opinions, retrospective studies, case reports and series. Sulfasalazine was firstly synthesized in 1930 and is currently being prescribed for various inflammatory and rheumatic diseases. Case report: We present a 45-year-old patient who was prescribed sulfasalazine tablets by a rheumatologist due to reactive arthritis. In the fourth week of therapy, he developed skin rash and fever up to 39.5°C. On admission, generalized maculopapular exanthema covering over 60% of the body surface area, edema of the lower eyelids and bilateral cervical and inguinal lymphadenomegaly were registered. Laboratory findings showed leukocytosis with significant eosinophilia, lymphocytosis, elevated bilirubin values, ALT <100 U/L, while ultrasonography of the upper abdomen confirmed hepatosplenomegaly. The patient was diagnosed with Drug Reaction with Eosinophilia and Systemic Symptoms Syndrome using RegiSCAR and J-SCAR diagnostic criteria and systemic therapy with methylprednisolone at a dose of 1.5 mg/kg and other supportive therapy was applied, which resulted in complete regression of the skin changes and normalization of laboratory values. Conclusion: The authors would like to recall the occurrence of Drug Reaction with Eosinophilia and Systemic Symptoms Syndrome as a rare and potentially fatal drug reaction in which awareness of this disease is of key importance for early recognition. Identification of the offending drug followed by a prompt withdrawal and subsequent treatment is essential for decreasing disease related morbidity and mortality and thus we wish to familiarize the sulfasalazine prescribers with this syndrome.

Keywords

• drug induced hypersensitivity syndrome
• severe cutaneous adverse drug reaction
• skin rash
• hypereosinophilia
• regiscar scoring system
• j-scar scoring systeme