Systemic allergic reaction to sulfasalazine treatment – DRESS syndrome in the course of juvenile idiopathic arthritis

DRESS syndrome (drug rash with eosinophilia and systemic symptoms) is a type of severe drug hypersensitivity reaction. It is characterized by maculopapular rash, haematological disorders (eosinophilia or atypical lymphocytes) and the involvement of internal organs (usually liver, kidney, lung, heart) (Table I, II, Fig. 1–3). There are often fever, lymphadenopathy, and facial oedema. Mortality is 10–38%. The pathogenesis, not fully understood, is multifactorial (viral infections, HHV-6, EBV, immunomodulatory effects of certain drugs, metabolic disorders, autoimmune diseases). The medical history remains the most important factor for the diagnosis. The treatment consists of discontinuation of the drug that caused an allergic reaction. In severe cases, corticosteroids and immunoglobulins are useful.

This article describes the case of 5-year-old girl, with a seven-week history of treatment with sulphasalazine due to isolated pain of the knee, and then symptoms of DRESS syndrome with a predominant clinical picture of liver dysfunction. The authors present diagnostic and therapeutic difficulties in the diagnosis of this syndrome, and the clinical course that required a wide differential diagnosis.