Episode 162.0 – Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis

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

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

Consider protecting airway if significant oral mucosal involvement
Stop the offending agent (if there is one)
Advanced wound care and pain control
Consults: Derm to do a biopsy, +/- ophthalmology, gyn / urology to prevent strictures or contractures
Consider transferring to a burn center

Dispo:

Low threshold for ICU admission
SCORTEN ( max of 7 points)

1 point each for

Age over 40
Current cancer
>30% body surface area affected
HR >120
BUN >28
Glucose >240
Bicarb <20
Score of 2 points or higher should -> ICU

Take Home Points

SJS may begin like the flu, with lesions appearing 1-3 days after the prodrome starts
Have to have a high suspicion for SJS because it is deadly. It’s a clinical diagnosis — derm biopsy is supportive
A thorough history and physical exam are key.  Remember the characteristic rash and bullae, and always look in the mouth and eyes.  Ask about dysuria, sore throat, and eye irritation, as well as preceding medications or infections.  Think SATAN!
Prompt supportive care focused on ABCs and IVF repletion are critical.  These patients can get sick really fast, so consider an ICU or burn unit.

References:

Barrett W.  Quick Consult: Symptoms: Rash, Dysuria, and Mouth Sores.  Emergency Medicine News.  41(4): 15-16, April 2019.

Bivins H, Comes J.  Stevens-Johnson Syndrome.  Rosen and Barkin’s 5-Minute Emergency Medicine Consult.  2015; 1076-1077.

Ergen EN, Hughey LC. Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. JAMA Dermatol.2017;153(12):1344. doi:10.1001/jamadermatol.2017.3957

Gerull R, Nelle M, Schaible T.  Toxic epidermal necrolysis and Stevens-Johnson syndrome: A review.  Crit Care Med.  2011; 39:1521-1532.

Lerch M, Mainetti C, Terziroli Beretta-Piccoli B, Harr T. Current Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. Clin Rev Allergy Immunol. 2018;54(1):147-76.

McNeil, D. (2019). Measles Cases Surpass 700 as Outbreak Continues Unabated. [online] Nytimes.com. Available at: https://www.nytimes.com/2019/04/29/health/measles-outbreak-cdc.html [Accessed 6 May 2019].

Mustafa SS, Ostrov D, Yerly D. Severe Cutaneous Adverse Drug Reactions: Presentation, Risk Factors, and Management. Curr Allergy Asthma Rep. 2018;18(4):26.

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