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
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