A MULTIDISCIPLINARY APPROACH TO THE TREATMENT

OF PEDIATRIC SJS/TEN AT A LEVEL 1 TRAUMA/ABA

CERTIFIED COMPREHENSIVE BURN CENTER  

Boswick Burn Symposium

4/19/2021

Kevin Yang MD PGY5 General Surgery

Contributors: C Collison, K Stahlfeld, H Sell, H Ferimer, J Ziembicki

University of Pittsburgh Medical Center- Mercy Hospital
UPMC MERCY HOSPITAL

 Level 1 Trauma Center/ABA Certified Comprehensive Burn Center

 108 cases of SJS/TEN since 2010
 4 cases of pediatric SJS/TEN
 2 due to carbamazepine, 1 Bactrim, 1 infectious related
 3 required intubation
 1 required excision/debridement
AS (9 YO MALE) + JV (14 YO M)
STEVENS JOHNSONS SYNDROME AND TOXIC EPIDERMAL
NECROLYSIS (SJS/TEN)

 First reported in 1922 (Mason Stevens + John Chambliss)

 1956: Alan Lyell reported “acute, generalized, sheet like loss of epidermis resembling a scald burn”
 Severe Cutaneous Adverse Reactions (SCAR)
 SJS <10% TBSA
 SJS/TENS 10-30% TBSA
 TEN >30% TBSA
 DRESS (Drug – induced hypersensitivity syndrome with eosinophilia and systemic symptoms)

 No literature on pulmonary complications

ETIOLOGY

 Triggered by medications and URIs in 74-94% of cases

 Sulfa-drugs
 Anti-epileptics (lamotrigine, carbamazepine, phenobarbital)
 NSAIDS, allopurinol
 Mycoplasma, CMV, HSV

 Genetic predisposition
 HLA B subtypes in East Asian populations

 Other contributing factors

 HIV
 SLE
 Hematologic malignancies
FDA ADVERSE EVENTS REPORTING
FDA ADVERSE EVENTS REPORTING
FDA ADVERSE EVENTS REPORTING
FDA ADVERSE EVENTS REPORTING
CLINICAL COURSE

 Initial manifestations
 Fevers
 Flu like symptoms (arthralgia, myalgia, malaise, etc)

 Mucosal + cutaneous involvement in 1-5 days

 Macules with purpuric centers evolve into large blisters and bullae
 Start in the abdominal area/thorax and spread outwards to extremities
 Respiratory compromise
 Bacterial superinfections
 Multisystem organ failure
DIAGNOSIS

 Clinical diagnosis
 Does not require skin biopsy
 Differential (EM, SSSS, drug reaction, Kawasaki’s)
 Oral and mucosal involvement
 ALDEN Score
 Serological/PCR tests for infectious diseases
SCORTEN

 SCORTEN
 Predicts mortality, developed for adults
 7 factors, calculate within 24 hours
 Age > 40
 HR > 120
 Cancer/hematologic malignancy
 TBSA at Day 1
 Serum Urea, Bicarb, Glucose

 Not validated in pediatric population

 Mortality
 SJS/TENS: 6.6%
 TEN: 25%
PATHOGENESIS

 Dysregulation of cellular immunity, unclear etiology

 Cytotoxic T Cells and NK cells recognize drug antigens on HLA molecules on keratinocytes
 Fas/granulysin/perforin mediated apoptosis
 Detachment of skin on the dermal-epidermal junction
 CD8+ T Cells in blister fluid
 IL2, 3, 5, 6, 10
TREATMENT

 Immediate cessation of suspected medication

 Admission/transfer to burn unit when TBSA > 20%
 Multidisciplinary care
 ICU care for fluid/electrolyte imbalances
 Nutritional support
 Pain control
 Wound care
 IVIG/steroids
 TNF alpha inhibitors (infliximab, etanercept)
 Cyclosporine
SPECIALIST HELP

 Ophthalmology
 Gynecology/urology
 Critical care
 Nutrition
 Acute Pain
 Burn/Wound care
CHRONIC SEQUELAE
 No long term studies in pediatric population
SUMMARY AND DISCUSSION

 Increased use of medications in children

 Higher incidence of TEN in pediatric population
 Lower mortality rates
 Transfer to tertiary burn center with multidisciplinary care teams
 High concern for respiratory decompensation
 Unknown long term pulmonary sequelae
 Need for more studies and definitive guidelines
 Newer screening guidelines for at-risk patients (biomarkers, CYP variants, HLA)
THANK YOU

 Questions?
REFERENCES

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 Liotti L, Caimmi S, Bottau P, et al. Clinical features, outcomes and treatment in children with drug induced Stevens-Johnson syndrome and toxic epidermal necrolysis.
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