 |
| Image Credit: Wikimedia Commons |
 |
| This post was peer reviewed. Click to learn more. |
Authors: Taraneh Matin, OMS-IV MA; Victoria Comeau, DO; Daniel F. Leiva, DO, MS
Nova Southeastern University
Rashes in the emergency department can often be overlooked as benign skin conditions. Being able to differentiate between life-threatening and non-life-threatening rashes is vital for the emergency physician. Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) comprise a spectrum of the same subset of vesiculobullous diseases with an incidence that ranges from 0.4 to 7 per million per year, with a mortality that ranges from 1-5% for SJS and 25-40% for TEN.[1,2] The majority of cases are caused by medications while a lesser extent are caused by infections.[1] Typical causative agents include allopurinol, antibiotics, nonsteroidal anti-inflammatory drugs, and anticonvulsants.
Although it is a fatal condition with serious systemic complications, SJS presents with very subtle and often times overlooked or misdiagnosed prodromal symptoms including fever, painful eyes, or pain with swallowing. These are often mistaken for a benign viral illness. When evaluating a patient with these findings, obtaining a thorough history is important due to the high correlation of these symptoms with the onset of a new medication. The cutaneous manifestations present within days of the initial symptoms as ill-defined, erythematous macules or purpura beginning on the trunk and spreading outward. The oral, genital, and/or ocular mucosa are involved in 90% of cases as well as frequent involvement of the palms and soles.[3] With progression, vesicles and bullae form and the skin begins to slough leading to a positive Nikolsky sign on examination.[5-7] Ocular involvement can present as erythema, conjunctivitis, keratitis, and endophthalmitis and has been seen in 50-80% of cases. One-third of these cases can lead to vision impairing ocular sequelae.[8] Other organ systems including the gastrointestinal or pulmonary systems may be involved. It is important when evaluating these patients to note the body surface area (BSA) involved. SJS has a BSA involvement of <10% while TEN has a BSA involvement of >30%. Risk factors include co-morbid HIV infection or an immunocompromised state.[6]
