A look at this deadly mucocutaneous reaction and how to best manage these patients in the ED https://media.blubrry.com/coreem/content.blubrry.com/coreem/SJS.mp3 Download Leave a Comment Tags: Critical Care, Dermatology Show Notes * Rash with dysuria should raise concern for SJS with associated urethritis * Dysuria present in a majority of cases * SJS is a mucocutaneous reaction caused by Type IV hypersensitivity * Cytotoxic t-lymphocytes apoptose keratinocytes → blistering, bullae formation, and sloughing of the detached skin * Disease spectrum * SJS = 30% TBSA * SJS/ TEN Overlap = 10-30% TBSA * Incidence is estimated at around 9 per 1 million people in the US * Mortality is 10% for SJS and 30-50% for TEN * Mainly 2/2 sepsis and end organ dysfunction. * SJS can occur even without a precipitating medication * Infection can set it off especially in patients with risk factors including HIV, lupus, underlying malignancy, and genetic factors * SATAN for the most common drugs * Sulfa, Allopurinol, Tetracyclines, Anticonvulsants, and NSAIDS * Anti-epileptics include carbamazepine, lamictal, phenobarb, and phenytoin * Can have a curious course * Hypersensitivity reaction can develop while taking medication, or even one to four weeks after exposure * In pediatric population, mycoplasma pneumonia and herpes simplex have been identified as precipitating infections * Patients often have a prodrome 1-3 days prior to the skin lesions appearing * May complain of fever, myalgias, headaches, URI symptoms, and malaise * Rash may be the sole complaint * Starts as dark purple or erythematous lesions with purpuric centers that progress to bullae * Skin surrounding the lesions detaches from the dermis with just light pressure (Nikolsky Sign) * Up to 95% of patients will have mucous membrane lesions * ~85% will have conjunctival lesions * Symptoms: Burning or itching eyes, a cough or sore throat, pain with eating, pain with urinating or defecating Source: JAMA Dermatol. 2017 * Differential Diagnosis: SSSS, autoimmune bullous diseases, bullous fixed drug eruption, erythema multiforme, thermal burns, phototoxic reactions, and TSS * SJS is a clinical diagnosis * Basic workup: CBC, chemistry panel, LFTs, and a UA * Treatment * Supportive care * IV fluid repletion guided by TBSA affected,