Table 6. Practical management of pediatric Stevens–Johnson syndrome – toxic epidermal necrolysis.
Admission | Determine cause based on drug history (ALDEN), infectious symptoms
Baseline: Investigations: confirm cause, rule out contraindications to treatment - routine bloodwork, including complete metabolic profile, liver function tests, urinalysis - infectious workup, including viral serologies/PCR (Epstein–Barr virus, cytomegalovirus, HSV, human herpes virus 6), nasopharyngeal swab for respiratory viruses and Mycoplasma pneumoniae PCR, oral mucosal swab for HSV PCR, chest x-ray to rule out pneumonia - screen for HLA risk alleles if not already known ( Table 4) - if patient severe and might need immunosuppression: consider interferon-gamma release assay for tuberculosis, hepatitis and HIV serology, Strongyloides serology Document severity: SCORTEN, BSA, photography Treatment: Discontinue potential causative medications Treat for infection if present with directed antibiotics Supportive care: sterile wound care, fluid replacement and nutritional supplementation as for burns, airway management, pain control Plan: Assess need for transfer to specialized experienced center for severe cases (SCORTEN >1, BSA >10%, comorbidities, requiring ventilation) Consult dermatology, ophthalmology, gynecology, urology, infectious disease, pharmacy/clinical pharmacology urgently Consider anti-inflammatory/immunosuppressive treatment: consider contraindications, risk-benefit |
Monitoring | Frequent vital signs, monitor for fever
Frequent swabs to identify infection early, prophylactic antibiotics not recommended Document progression with SCORTEN, photography Supportive care, including early physiotherapy |
Follow-up | Identify a primary contact for the patient after discharge, either a pediatrician or specialist amongst the following:
• Dermatology • Ophthalmology • Gastroenterology • Gynecology (female) and urology (male) • Psychiatry/Psychology for post-traumatic stress disorder • Genetics to review HLA testing and counsel family • Respirology if needed Consider in vitro testing with lymphocyte transformation test or ELISpot (controversial) Give patient a wallet card that identifies their history of SJS-TEN and HLA screening result for future medical encounters. Please refer to Figure 4 in Sukasem et al. 35 for an example. |