A child recovering from carbamazepine-induced SJS-TEN overlap — long case

Candidate instructions (10-minute long-case discussion)

You are the paediatric registrar on the ward. A 9-year-old girl was admitted three weeks ago with Stevens-Johnson syndrome-toxic epidermal necrolysis overlap, six weeks after starting carbamazepine for new-onset focal epilepsy. She presented with fever, painful red eyes, crusted bleeding lips, dusky target lesions and sheet-like detachment of about 12 percent of body surface area. She is now recovering — her skin is re-epithelialising and her oral intake is improving, but she still has ocular discomfort and photophobia. The examiner asks you to discuss her acute management, the severity assessment, the long-term sequelae and follow-up, and the family pharmacogenetic and drug-avoidance plan. [1]

Your tasks are: [1]

1. Outline the immediate acute management she should have received, including the single most important action. [10]
2. Explain the SCORTEN severity assessment and how it informed her disposition. [1]
3. Discuss the long-term sequelae she is at risk of and the follow-up she needs. [7]
4. Outline her lifelong drug-avoidance plan and the family HLA pharmacogenetic screening. [4]

You are not expected to manage her ongoing ophthalmology care independently — the ophthalmology team is involved, but you should explain its importance. [10]