Case 1
A 9-year-old girl attended to our hospital with a generalized itchy skin eruption starting from the face, trunk and extremities for four days. She was diagnosed with epilepsy and treated with Valporat-acid for 3 years. Lamotrigine (25mg/day) was added to her regimen 2 weeks before the eruptions due to uncontrolled seizures. On examination she was alert and oriented with a temperature of 39°C and pulse was 112 /minutes. There was a generalized erythematous maculopapular skin eruptions that were localized in the trunk, extremities and face, erythematous target and purpuric lesions in the lower limbs, edema of the face and swollen lips. [Figure 1a; 1b]The examination of the rest of the body was normal, especially the lymph node areas were free.
The laboratory investigation showed that C-reactive protein: 100mg/dl, WBC: 9750/mm3, Neutrophil: 81% dominant (7910/mm3), Lymphopenia at 740/mm3, Na: 128 mEq/L (Normal range: 135-145 mEq/L). other electrolytes, liver and kidney functions were within normal limit. The skin pathology of a target lesion was consistent with erythema multiforme. Declaration to pharmacovigilance confirmed the imputability of the combination of Lamotrigine with Valporat Acid in the occurrence of drug-induced skin reaction.
The final diagnosis was DRESS syndrome based on the presence of fever, face oedema, occurrence of the itchy polymorphic generalized skin eruption 2 weeks after the onset of the lamotrigine and the presence of lymphopenia.
Lamotrigine and Valporat Acid were immediately discontinued and the patient was treated with prednisone at a dose of 0,5mg/kg/day with good outcomes.