A 12 year old boy, with a known case of seizure disorder presented to the casualty with generalized maculopapular rash, fever and facial puffiness. The patient had been operated for gastric perforation when he was 3 days old following which the seizure episodes started. He was treated with phenobarbitone till the age of five. The drug was gradually withdrawn on the advice of a physician. Six months ago he suffered an episode of convulsion. He was started on sodium valproate and levetiracetam. One month back levetiracetam was stopped and lamotrigine was initiated. The patient was taking 1 tablet of 25mg daily for the first 15 days; later the dose was doubled. On the 18th day, patient presented with generalized maculopapular rash, itching and fever and on 20th day he developed colicky abdominal pain and vomiting followed by facial puffiness.\nOn examination, he was alert and oriented, with a temperature of 100°F and pulse was 114/ minutes. There was significant angioedema of his face with edematous lips and swollen ears, and an extensive maculo-papular rash on his entire trunk [], buttocks, extremities and face. The whole body had a red tinge. Examination showed yellow sclera, conjunctival congestion and crusting over his lips. He experienced no joint pain or neurological symptoms, and no abdominal or respiratory complaints. There was no history of any allergies. The birth history and immunization were uneventful. He had 3 siblings: one of them also had history of epileptic disorders for which she was on treatment.\nInvestigations revealed leukocytosis (15,960cells/cmm) with eosinophilia (19%) AEC=2600/cmm. Hemoglobin was 10.8gm%, platelet count 2.53lakhs/cmm; ESR and PCV were normal. Liver enzymes were raised with SGOT=905U/l, SGPT=495U/l, ALP=855U/l and GGT= 155U/l. Total bilirubin was 1.5mg% (direct 0.51mg% and indirect 0.99mg%). Blood urea and creatinine were normal. Routine examination of the urine was normal. Provisional diagnosis of DRESS was made based on the above findings.\nParental cortisteroids and systemic antihistamines were initiated. Lamotrigine was stopped. Within 5 days the rash subsided. Patient's general condition improved. On discharge patient was given oral steroids and antihistaminic's.
A 16-year-old boy, known case of juvenile myoclonic epilepsy, presented to the neurology OPD with generalized maculopapular rash [], fever, and facial puffiness. He was on LTG 250 mg TDS, clobazam 10 mg OD, and valproate 200 mg (morning) 400 mg (evening) for 1 year. Subsequently, he developed erythematous rashes over the face, neck, trunk, arms, and legs followed by painful ulcers in the oral cavity and encrustations on the lips. History revealed that no such lesions occurred earlier. His neurological examinations, CT head and MRI, were found to be within normal limits. EEG was abnormal and suggestive of generalized epilepsy. Physical examination showed multiple round erythematous plaques on the face, neck, trunk, and extremities, some of which were tender. On oral examination, there was limited mouth opening and erythematous crusted areas on both the lips due to inflammation and ulceration. Based on the history and clinical presentation, a diagnosis of SJS was made. As the Pre-assumption was LTG, the drug was withdrawn immediately and the dose of clobazam was increased. The patient was admitted to dermatology ward and treated with cyclosporine and methylprednisolone and intravenous fluids. His condition improved during the next 2 weeks; he eventually recovered and continued follow-up on an outpatient basis.