Stevens Johnson Syndrome

Diagnosis: Stevens Johnson Syndrome

Clinical History

Acute onset of skin pain, fever, and mucosal erosions 1-3 weeks after starting a new medication. Rapidly progressive epidermal detachment. Nikolsky sign positive.

Treatment

Discontinue offending drug immediately. Supportive care in burn unit/ICU. Wound care. Ophthalmology, urology, gynecology consultation for mucosal involvement. Cyclosporine (emerging evidence). IVIG controversial. Avoid systemic corticosteroids (controversial).

Differential Diagnosis

• Erythema multiforme • Toxic epidermal necrolysis • Staphylococcal scalded skin syndrome • Drug reaction with eosinophilia and systemic symptoms (DRESS) • Paraneoplastic pemphigus • Acute generalized exanthematous pustulosis (AGEP) • Bullous pemphigoid

Key Learnings

• SJS/TEN are a spectrum: SJS (30%) • Drug-induced in ~80% of cases — most common culprits: allopurinol, sulfonamides, carbamazepine, phenytoin, lamotrigine, nevirapine, NSAIDs (oxicam) • HLA-B*5801 screening before allopurinol (especially in Southeast Asian, Korean, African American populations) • HLA-B*1502 screening before carbamazepine (Han Chinese, Southeast Asian) • SCORTEN score predicts mortality • Mucosal involvement (≥2 sites) is characteristic — oral, ocular, genital • Ocular complications can be devastating — ophthalmology consultation essential • Treatment is largely supportive — transfer to burn unit for severe cases • Cyclosporine may reduce progression if started early (emerging evidence)

Tags: derm, dermatologist, dermatology, SJS, Stevens Johnson Syndrome