Stevens Johnson Syndrome and Toxic Epidermal Necrolysis

Both Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, severe, life-threatening reactions that are almost always drug related. The most common offending medications are NSAIDS, antibiotics, and antiseizure medications. SJS or TEN typically occurs 7 to 21 days after the start of the medication.

Stevens-Johnson syndrome often presents with a prodrome of fever, painful swallowing, stinging eyes, and painful skin. Two or more mucosal surfaces eventually become involved (conjunctiva, oral mucosa, genitalia) with vesicles, bullae, erosions or hemorrhagic crusts. Skin lesions usually begin as dusky purpuric macules that progress to bullae or erosions on the trunk and spread centrifugally. Targetoid lesions, similar to EM, may also be present. By definition, less than 10% body surface area (BSA) is involved in SJS. A similar presentation occurs in TEN, but the bullae and erosions involve greater than 30% BSA. Also, the skin that initially appears normal may easily slide off with gentle tangential pressure (Nikolsk/s sign), leaving more denuded skin. When skin and mucosal involvement is 10% to 30% BSA, it is considered SJS/TEN overlap.

Both SJS and TEN can be fatal; mortality is 1% to 5% for SJS and 25% to 35% for TEN, but may be even higher in elderly patients or those with comorbidities. Above all, removal of the offending agent is paramount. Most patients are treated in a burn unit because the skin is necrotic and no longer functioning as a barrier. Care is supportive and aimed at regulation of fluids, electrolytes, protein, temperature, and prevention of infection. Neither systemic steroids nor intravenous immune globulin (IVIG) demonstrate consistent clinical efficacy in clinical studies, which are limited because of the rarity of SJS and TEN.

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