A case of recalcitrant DRESS syndrome with multiple autoimmune sequelae treated with intravenous immunoglobulins

摘要

Report of a Case. A 21-year-old woman with bipolar disorder and a family history of maternal hypothyroidism started therapy with lamotrigine, and 2 weeks later she presented to an outside hospital with fever (39°C) nausea, vomiting, and a diffuse morbilliform eruption without mucosal involvement (Figure). Abnormal laboratory results included a white blood cell count of 35 700/uL (normal, 4500-10 800/muL) with 20% atypical lymphocytes and 10% eosinophils; alanine amino transaminase level, 71 U/L (normal, 11-54 U/L); and creatinine level, 1.4 mg/dL (normal, 0.6-1.1 mg/dL). (To convert white blood cells to number of cells X 109/L, multiply by 0.001; to convert creatinine to micromoles per liter, multiply by 88.4.) Computed tomography demonstrated numerous enlarged retroperitoneal and mesenteric lymph nodes. She was diagnosed as having drug reaction with eosino-philia and systemic symptoms (DRESS), with a calculated RegiSCAR score of 7 (>5 indicates definitive diagnosis1) , and treated with intravenous methylprednisolone, 40 mg every 6 hours. She showed clinical and laboratory improvement and was discharged with a prescription for prednisone, 80 mg/d, to be tapered to nothing over 15 days.