Stevens-Johnson syndrome is a rare, severe skin and mucous membrane reaction, most often to a medicine, in which the top skin layer dies and detaches over less than 10 percent of the body. It is a medical emergency and lies on the same spectrum as toxic epidermal necrolysis.
Key symptoms
- Fever, sore throat and malaise for a few days before the rash
- Painful red or dusky spots that blister and peel
- Painful mouth sores and trouble eating or drinking
- Red, painful, gritty eyes and painful urination
- Started a new medicine in the past 1 to 8 weeks
What you find on exam
- Dusky red or purple macules and atypical target lesions
- Blisters and skin that slides off with gentle pressure (Nikolsky sign)
- Erosions on at least two mucosal sites: lips, mouth, eyes, genitals
- Estimate the percent of body surface with detached skin
- Vital signs, hydration and signs of infection
Tests
- Skin biopsy confirms full-thickness epidermal necrosis
- Blood count, electrolytes, kidney and liver function, glucose
- Mycoplasma testing, especially in children
- SCORTEN score to estimate risk of death
Treatment basics
- Stop the suspected drug at once (common: allopurinol, sulfonamides, carbamazepine, lamotrigine, phenytoin)
- Admit to a burn unit or ICU for wound care, fluids and temperature control
- Urgent ophthalmology review and daily eye care
- Pain control, nutrition and mouth care; no prophylactic antibiotics
- Cyclosporine or etanercept may be used with specialist input
Red flags
- Skin detachment spreading beyond 10 percent of the body
- Eye involvement, breathing difficulty or inability to drink
- Fever with low blood pressure suggesting sepsis
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Sources: NCCPA PANCE Content Blueprint; NBOME COMLEX-USA clinical presentations; AAMC Core Entrustable Professional Activities (Core EPAs). Written by Making Synapses. Making Synapses is not affiliated with these organizations.