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 SJS: < 10% of body surface area TEN: > 30% of body surface area SJS/TEN overlap: 10 - 30% of body surface area Severe, febrile blistering disease of skin and mucous membranes often caused by drugs (>>> infection) e.g., penicillin, sulfonamides, phenytoin, carbamazepine, lamotrigine, NSAIDs can be caused by infection e.g., Mycoplasma pneumonia 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 very painful skin vs in EM, burning/pain is typically very mild systemic signs fever dehydration hypotension non-skin findings ocular involvement (50-80%) corneal ulceration uveitis pulmonary involvement (25%) pneumonitis bronchiolitis obliterans bronchitis Physical exam initially dusky red macules or patches (not raised)that progress to tense bullae and eventual skin sloughing vs in EM, where lesions are typically papular/raised mucous membranes always involved bullae and erosions in oral, genital, anal mucosa + Nikolsky sign (rubbing of skin easily causes sloughing – splitting of epidermis from dermis) 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 full-thickness epidermal necrosis Labs: normal Differential Staphylococcal scalded skin syndrome Graft versus host disease Pemphigus vulgaris Erythema multiforme Treatment Discontinue causative agent early withdrawal = lower mortality Hospitalization admit to ICU or burn unit if pulmonary involvement, may require mechanical ventilation if ocular involvement, consult ophthalmology and apply topical erythromycin to prevent ocular adhesions Supportive care wound care with petrolatum and gauze fluids, electrolytes, nutrition Treat underlying infection Pharmaceutical options to lower mortality: cyclosporine, IVIG, corticosteroids all controversial Complications Skin hypo/hyperpigmentation Ocular complications - dry eyes, corneal scarring, photophobia Pulmonary complications - chronic bronchitis, bronchiectasis, bronchiolitis obliterans Sepsis Prognosis High mortality TEN: 30 - 50% mortality rate SJS: 5 - 10% mortality rate