Fatal eosinophilic myocarditis and submassive hepatic necrosis in lamotrigine induced DRESS syndrome

Abstract

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) syndrome is a rare but severe and sometimes fatal adverse drug reaction that is known to occur with a number of antiepileptic drugs. It often follows a prolonged clinical course, which can worsen even after discontinuing the causative drug and administering steroid treatment. Failure to promptly identify the delayed involvement of vital organs, such as the heart and liver, may result in irreversible organ failure and death. We report a case of a presumed sudden death of a young woman who had a documented history of a protracted intermittent hypersensitivity reaction to lamotrigine. Postmortem examination revealed the presence of eosinophilic myocarditis and submassive hepatic necrosis diagnostic of fatal DRESS syndrome that progressed despite early discontinuation of the medication and improvement of dermatologic and hematologic symptoms following steroid therapy.

Keywords: Autopsy; Drug hypersensitivity syndrome; Lamotrigine; Massive hepatic necrosis; Myocarditis; Thyroiditis.

Fig. 1

A, B. Myocardium. Intermediate and high-power sections from the heart showed an eosinophil-rich inflammatory infiltrate separating the myofibers with areas of complete loss of myocardial cells. (H&E, magnification 200X and 400X) C. Liver. The sections show extensive centrilobular coagulative necrosis. (H&E, magnification 100X) D. Thyroid. The thyroid follicles showed sloughing of follicular cells and infiltration by mononuclear cells with numerous multinucleated giant cells. (H&E, magnification 400X)