Case 2: Where did you get that DRESS?

Abstract

A 14-year-old teenage girl with a known seizure disorder presented to the emergency department (ED) with fever, extensive maculopapular rash, facial edema and odynophagia. Twenty-four days earlier, she had been placed on phenytoin and cephalexin after cranial lesionectomy surgery for epilepsy. Other medications included therapy for the previous two years with oxcarbazepine and valproic acid. She had no known allergies. Her symptoms had begun one week earlier with a mild morbilliform rash and fever. She was seen in the ED at that time, and both the phenytoin and cephalexin were discontinued. In the following days, her rash extended and became pruritic with associated facial swelling. Due to worsening of her symptoms and continuing fever, she returned to the ED. On examination, she was alert and oriented, but appeared unwell with a fever of 39°C. There was significant angioedema of her face and ears (Figure 1), cervical lymphadenopathy, and an extensive rash (Figure 2) on her entire trunk, buttocks, extremities and face; her palms and soles were spared. A review of her systems was otherwise unremarkable; specifically, she experienced no joint pain or neurological symptoms, and no abdominal or respiratory complaints. She denied any allergies, sick contacts or recent travel, and her immunizations were up to date. Figure 1) Significant angioedema of the patient’s face and ears in Case 2 Figure 2) An extensive rash on the patient’s arm in Case 2 Investigations revealed an elevated C-reactive protein level (96 mg/L [normal 0 mg/L to 8 mg/L]), normal erythrocyte sedimentation rate and mild leukocytosis (12.7×109/L [normal 4×109/L to 10×109/L]), with elevated bands (2.29×109/L [normal 0.00×109/L to 0.01×109/L]) and eosinophilia (1.14×109/L [normal 0.02×109/L to 0.50×109/L]). Hemoglobin (137 g/L [normal 120 g/L to 153 g/L]) and platelets (166×109/L [normal 150×109/L to 400×109/L]) were normal. Liver enzymes were mildly elevated (alanine aminotransferase 64 U/L [normal 0 U/L to 40 U/L], aspartate aminotransferase 68 U/L [normal 0 U/L to 36 U/L], gamma-glutamyl transpeptidase 397 U/L [normal 0 U/L to 43 U/L]). The creatinine level and a urinalysis were normal. A throat swab culture for group A streptococcus and an antistreptolysin O titre were sent. Heterophile antibody, Epstein-Barr virus and cytomegalovirus titres, as well as blood and urine cultures were sent. A chest x-ray and electrocardiogram were unremarkable. A provisional diagnosis was made based on the findings described above.