Episode 162.0 – Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis - a podcast by Core EM

A look at this deadly mucocutaneous reaction and how to best manage these patients in the ED







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Tags: Critical Care, Dermatology











Show Notes

Episode Produced by Audrey Bree Tse, MD





* 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 = <10% TBSA

* TEN = >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, as well as electrolyte, protein, and energy supplementation

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