Snapshot A young boy is brought to the emergency room after visiting his primary care physician, who noted erythematous, desquamative lesions all over his body (well over 30% of body surface area) and ulcerations of the mucosal membranes of the mouth and eyes. He was recently treated with penicillin for an infection. Introduction Stevens-Johnson Ssyndrome (SJS) and toxic epidermal necrolysis (TEN) – two diseases on the same spectrum login to view 3 more bullets Severe, febrile blistering disease of skin and mucous membranes login to view 4 more bullets Immunocompromised patients like those with HIV or SLE are more at risk for SJS Can be fatal Erythema multiforme (EM) is a distinct disease from SJS/TEN according to the current consensus definition Presentation Symptoms login to view 14 more bullets Physical exam login to view 5 more bullets Most common cause of death is sepsis Evaluation Based on clinical history and symptoms Skin biopsy: mainly to distinguish staphylococcal scalded skin syndrome and TEN login to view 1 more bullet Labs: normal Differential Staphylococcal scalded skin syndrome Graft versus host disease Pemphigus vulgaris Erythema multiforme Treatment Discontinue causative agent login to view 1 more bullet Hospitalization login to view 3 more bullets Supportive care login to view 2 more bullets Treat underlying infection Pharmaceutical options to lower mortality: cyclosporine, IVIG, corticosteroids login to view 1 more bullet Complications Skin hypo/hyperpigmentation Ocular complications - dry eyes, corneal scarring, photophobia Pulmonary complications - chronic bronchitis, bronchiectasis, bronchiolitis obliterans Sepsis Prognosis High mortality login to view 2 more bullets