Abstracts

Rationale: Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is a rare, acute and potentially life-threatening systemic disease. DRESS syndrome is characterized by fever, lymphadenopathy, rash, hypereosinophilia and involvement of systemic organs. Aromatic antiepileptic drugs are some of the most commonly implicated agents with Carbamazepine being the most common. Ethosuximide is the first line treatment for absence epilepsy. We report unusual case of Ethosuximide causing DRESS Syndrome in patient with childhood absence epilepsy. Methods: A 10-year-old girl presented with brief starring spells and unresponsiveness without automatism. Based on video EEG, diagnosis of childhood absence epilepsy was made. Patient did not have any drug allergy or coexisting medical problems. Patient was started on Ethosuximide 250 mg twice a day. Two and a half weeks later, she started experiencing fever with temperature of 102 Fahrenheit. It was quickly followed by rash. Rash first erupted on cheeks and periaural area and later progressed to involve trunk and extremities over 48-72 hours. It was accompanied by abdominal pain and vomiting. Patient presented to emergency room for evaluation. Results: On exam, patient was noted to have numerous diffuse dark red macules, papules ranging from pinpoint to up to 4 mm in diameter with occasional confluent plaques [figure 1 and 2]. Mucous membranes were spared. No lymphadenopathy or hepatomegaly was noted. Lab work revealed eosinophilia 5.8% (normal 0-4) and elevated liver enzymes: AST 1019 (normal 10-40), ALT 1441 (normal 10-30), bilirubin 3.2 (normal 0-1) and GGT 126 (normal 14-23). ANA, hepatitis viral panel, anti-smooth muscle antibody and EBV panel were negative. Echocardiography was unremarkable. Given appearance and timeline of rash was consistent with drug eruption, skin biopsy was not performed based on dermatology recommendation. Patient was diagnosed with DRESS Syndrome based on RegiSCAR score. Ethosuximide was discontinued. Patient was given intravenous solu-medrol for 3 days followed by oral taper in a time period of 6 weeks with topical steroid ointment. Patient gradually improved with disappearance of rash and normalization of liver enzymes over next six weeks. Conclusions: Ethosuximide is the first line treatment for childhood absence epilepsy. To date, there is only one reported case of DRESS Syndrome associated with Ethosuximide [Conilleau et al. 1999]. Our case illustrates importance of having high index of suspicion in cases with compatible clinical features even in patient being treated with non-aromatic anticonvulsant.