steven johnson syndrome

1. Stevens-Johnson Syndrome
Dr Abdullah Alzahrani
PEM Fellow
KSUMC

2. • A 7-year-old boy with a chief complaint of a new rash.
• 3 days ago ,he has small circular red bumps on his arms. bumps
have darkened in color and are now scattered across his body.
• He has not been drinking as well as normal, resulting in dry
chapped lips.
• He has started to complain of general fatigue, but his parents have
not noticed him itching the rash.
• He has a mild persistent cough the last week, but deny any fever,
vomiting, or diarrhea.

3. • On examination, the patient is tired
appearing with mild conjunctival
injection .
• Vital signs are as follows:
temperature of 37.3°C, a heart rate
of 130 beats/min, respiratory rate of
28 beats/min, blood pressure of
110/68 mm Hg, and a pulse oximetry
of 96% on room air.
• He has mild diffuse crackles on his
lung examination, but no focal
wheezes or increased work of
breathing.
• His heart, abdominal, and neurologic
examinations are normal.
• On his skin examination, you notice
he has irritated (cracked,
erythematous) and edematous lips
.
• Diffuse papular lesions on his
extremities that are slightly raised,
do not blanch on palpation, and are
tender to palpation.

4. Of the following, the BEST next step in managing this patient would be to
administer
A. broad spectrum antibiotics and admit him to the general hospital ward
B. cephalexin and discharge with follow up with primary care physician
C. intravenous fluids and admit him to the intensive care
D. oral steroids and antihistamines and discharge with follow up with his
primary care physician

5. • Definition
• Etiology
• Clinical features
• Diagnosis and DDx
• Management
• Prognosis

6. Definition
• SJS and TEN are severe cell-mediated mucocutaneous
reactions, characterized by extensive necrosis and detachment of
the epidermis, usually at two or more sites (ocular, oral, and
genital).
• SJS and TEN differ only along a spectrum of severity based upon the
percentage of body surface affected by blisters and erosions.
• There are 1 to 7 for SJS and 0.4 to 1.5 for TEN cases per million
people per year.
• Approximately equal incidence between male and female children.

7. Erythema
Multiforme
TENOverlap
SJS/TEN
SJS
Detachment less
than 10 percent
of BSA
Detachment of
greater than 30
percent of BSA
Detachment
between 10 and
30 percent of
BSA
Detachment less
than 10 percent
of BSA
Typical targets or
Raised, atypical
targets
Widespread
macules or
Flat, atypical
targets
Widespread
macules or
Flat, atypical
targets
Widespread
macules or
Flat, atypical
targets

8. Etiology
• Medications are the leading trigger of (SJS/TEN) in both
adults and children.
• The risk of SJS/TEN seems to be limited to the first eight
weeks of treatment.
• The typical exposure period before reaction onset is four
days to four weeks of first continuous use of the drug.

9. Suspected association/lower riskAssociatedStrongly associated
PantoprazoleDiclofenacLamotrigine
GlucocorticoidsDoxycyclineSulfamethoxazole
OmeprazoleAmoxicillin/ampicillinCarbamazepine
TetrazepamCiprofloxacinPhenobarbital
Dipyrone (metamizole)LevofloxacinAllopurinol
TerbinafineAmifostinePhenytoin
LevetiracetamOxcarbazepineSulfasalazine
acetaminophenRifampinOther sulfonamides
Oxicam NSAIDs (piroxicam, tenoxicam)
Nevirapine

10. • Mycoplasma pneumoniae infection is the next most common
trigger.
• A systematic review of case series and single case reports suggests
that M. pneumoniae-associated cases may be characterized more
often by moderate to severe involvement of two or more mucosal
sites and sparse, or even absent, skin involvement .
• Rarely reported causes of SJS/TEN include vaccinations, systemic
diseases, contrast medium, external chemical exposure, herbal
medicines, and foods.
• In over one-third of SJS/TEN cases, no cause can be identified .
Meyer Sauteur PM, Goetschel P, Lautenschlager S. Mycoplasma pneumoniae and mucositis--part of the Stevens-Johnson syndrome spectrum. J Dtsch
Dermatol Ges 2012; 10:740.

11. Clinical Features
• Prodrome — Fever, often exceeding 39°C (102.2°F), and influenza-like
symptoms precede by one to three days the development of
mucocutaneous lesions.
• Myalgia, arthralgia, dysphagia, photophobia, conjunctival itching/burning.
• Signs and symptoms that should alert the clinician to the possibility of
SJS/TEN include fever >38°C (100.4°F), mucositis, skin tenderness, and
blistering.
Bircher AJ. Symptoms and danger signs in acute drug hypersensitivity. Toxicology 2005; 209:201.

15. Laboratory abnormalities
• Anemia and lymphopenia, are common in SJS/TEN .
• Neutropenia in one-third of patients and is correlated
with a poor prognosis.
• Eosinophilia is unusual.
• Hypoalbuminemia, electrolyte imbalance, and
increased blood urea nitrogen and glucose in severe
cases .
• Mild elevations in serum aminotransferase levels.

16. Clinical Course
• The acute phase lasts 8 to 12 days and is characterized by
persistent fever, severe mucous membrane involvement, and
epidermal sloughing .
• Re-epithelialization may begin after several days and typically
requires two to four weeks , it is faster in children.
• In severe cases, massive loss of fluids through the denuded skin,
electrolyte imbalance, hypovolemic shock with renal failure,
bacteremia, insulin resistance, hypercatabolic state, and
multiple organ failure.

17. Diagnosis
• There are no universally accepted diagnostic criteria.
• The diagnosis of SJS or TEN would be appropriate in a patient with
the following clinical features :
o A suggestive history of drug exposure
o A prodrome of acute-onset febrile illness and malaise.
o A painful rash that progresses rapidly.
o Erythematous macules, targetoid lesions, or diffuse erythema progressing to vesicles and
bullae
o Positive Nikolsky sign
o Oral, ocular, and/or genital mucositis with painful mucosal erosions
o Necrosis and sloughing of the epidermis of varying degree
Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis: Part II. Prognosis, sequelae, diagnosis, differential diagnosis, prevention, and treatment. J Am
Acad Dermatol 2013; 69:187.e1.

18. Differential Diagnosis
Erythema multiforme Staphylococcal scalded skin
syndrome
KD Classic morbilliform drug
eruption
localized eruption of skin and
mucous membranes.
progress from widespread
erythema to blister formation
and desquamation
atypical to have
bullous or vesicular
lesions, more
commonly
morbilliform
rashes
generalized and symmetric
pruritic or asymptomatic
peripherally located typical
target lesions or raised,
atypical, targetoid lesions
A prodrome of sore throat or
purulent conjunctivitis may
be present for 48 hours
followed by fever, malaise,
and skin findings.
bilateral conjunctivitis
is nonexudative
eruptions occur within the 5
to 14 days ofexposure
Mucous membrane
involvement is absent or
limited to 1 surface, most
often the mouth.
lesions are never dusky or
purpuric in appearance
cervical
lymphadenopathy
and changes in the
extremities
lack mucosal involvement
and skin pain
associated with infection with
herpes simplex virus and
rarely with drugs.
No mucous membrane
involvement.
Nikolsky sign on unaffected
skin

19. Management
GENERAL PRINCIPLES
o Early detection .
o Withdrawal of the offending agent.
o Initiation of supportive care.
o Early consultation with appropriate specialists (ophthalmology,
dermatology ).
o Early intensive care unit (ICU) admission or burn center
referral.

20. Supportive Care
• The main principles of supportive care are the same as for major
burns
• Wound care
o wound debridement with biologic dressing, nonadherent monocrystalline gauze materials,silver
nitrate or chlorhexidine dressing on infected wounds.
• Ocular management
o daily lubrication, daily erythromycin drops to prevent infection, corticosteroid drops to reduce
inflammation .
• Fluid and electrolyte management
o (volume loss approximately one third less than that for burn victims).

21. Supportive Care
• Nutritional support
o early oral feeding ,high calorie requirements similar to those in burn injury.
• Temperature management
o up to 30°C - 32°C to prevent excessive caloric expenditures from epidermal loss.
• Pain control
o Acetaminophen or ibuprofen for mild pain and opioids for sever pain .
• Monitoring or treatment of superinfections
o Prophylactic antibiotics are not indicated, as these can cause emergence of resistant bacteria
and negatively impact survival.
o Sterile handling , Antiseptic solutions , Repeated cultures.

22. Adjunctive Therapies
• Beyond supportive care, there are no established therapies for SJS and
TEN .
• Several immunosuppressive or immunomodulating therapies have been
used .
• Systemic corticosteroids, IVIG, cyclosporine, plasmapheresis, and anti-
tumor necrosis factor (TNF) monoclonal antibodies.
• None of these therapies have been adequately studied in randomized trials
except thalidomide, which was found to be harmful.
• There is therefore no therapeutic intervention that can be definitively
recommended at this time.

23. Prognosis
• Mortality is considerably lower in children, ranging
among centers from 0 to 9.5 percent .
• Sepsis, acute respiratory distress syndrome, and
multiple organ failure are the most common causes of
in-hospital death.
Techasatian L, Panombualert S, Uppala R, Jetsrisuparb C. Drug-induced Stevens-Johnson syndrome and toxic epidermal necrolysis in children: 20 years study in
a tertiary care hospital. World J Pediatr 2017; 13:255.

24. SCORTEN score
• The prognosis of individual patients can be evaluated on
admission by applying a prognostic scoring system
called SCORTEN
• Validated for adults and children combined on days 1
and 3 of hospitalization, though not yet so for children
alone.
• Helpful when discussing a patient's prognosis with family
members or medical staff and transfer to ICU or burn unit

25. • Pediatric SCORTENs
• Pediatric Index of Mortality 2
(PIM2)
• Pediatric Risk of Mortality III
(PRISM III)
• Abbreviated Burn Severity
Index (ABSI)

26. Recurrence
• If the patient is re-exposed to the offending drug.
• The overall risk of recurrence is unknown, although it seems to be higher in
children with SJS/TEN associated with infections.
• In a retrospective study of 55 cases, 10 children had recurrence between 2
months and 7 years after the first episode, 3 had multiple recurrences, and 1
died. Recurrence was most often associated with Mycoplasma
pneumoniae or herpes simplex virus infection and occasionally
with antiepileptic drugs.
• However, an overestimation of the recurrence rate due to the
misclassification of cases of erythema multiforme as SJS cannot be
excluded.
Finkelstein Y, Soon GS, Acuna P, et al. Recurrence and outcomes of Stevens-Johnson syndrome and toxic epidermal necrolysis in children. Pediatrics 2011;
128:723.

27. Take Home Message
• SJS and TEN represent an early, significant diagnostic
challenge for emergency medicine practitioners.
• Suspect SJS/TEN if you are approaching fever +rash.
• Look for mucosal lesion (oral /ocular /urogenital ).
• Don’t forget medication history.

28. References
• https://www.uptodate.com/contents/stevens-johnson-syndrome-and-toxic-
epidermal-necrolysis-pathogenesis-clinical-manifestations-and-
diagnosis?source=history_widget
• https://www.uptodate.com/contents/stevens-johnson-syndrome-and-toxic-
epidermal-necrolysis-management-prognosis-and-long-term-
sequelae?topicRef=2091&source=see_link
• Stephen Alerhand, M. C. C. M. a. A. K. M., Volume 32, Number 7, July 2016.
Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in the Pediatric
Population A Review. Pediatric Emergency Care, pp. 472-478.
• Jennifer Sorrell, M. L. A. M. A. R. P., 2017. Score of Toxic Epidermal Necrosis
Predicts the Outcomes of Pediatric Epidermal Necrolysis. Pediatric Dermatology
, pp. 1-5.
• Bachur, R. G. & Shaw, K. N., 2015. Fleisher & Ludwig's Textbook of Pediatric
Emergency. 7th Ed ed. s.l.:Lippincott Williams & Wilkins (LWW).