1. SJS TEN ER guidelines;
Anti-fas may be promising & steroid still controversial
Daifallah Almansouri MD
Venue: KF Hospital, SA.
Date: 22nd Dec 2013

3. Outcomes
1. Definition and epidemiology.
2. Etiology
3. Pathogenesis.
4. Symptomology.
5. Clinical examination.
6. Exclude DDx.
7. Investigation.
8. Management guidlines.
9. Complication.
10.Prognosis.

4. 1. Definition and epidemiology
• SJS/ TEN is immune-complex–mediated
hypersensitivity causes extensive
apoptosis of the skin and the mucous
membranes.
• Epidemiology; sex, genes, age, chronic
disesse (hiv & brain tumur).
– Generally: 1or 2/1,000,000 annually.

5. NB. BSA= detached+ detachable (+Nikolsky)

6. Nikolsky+

7. 2. Etiology
I. 25-50% iodopathic.
II. Defined cause:
– infection: mostly in pediatric
GABHS (URTI), HSV.
– malignancy: mostly in elderly
– drugs: mostly blamed (> 30% BSA》95% drug).
Antibiotic (most common), anticonvulsant, allopurinol
(most associated).
Genetic: very strong predisposing factor.
TEN: 80% cause by medications.

8. Medications association

9. Hx of Gout

10. 3. Pathophysiology

11. 4. Symptomology
• HPI:
– Prodromal systemic symptoms: fever, maliase, ....
– Cutanous: abrubt erythematous tender rashes.
– Mucus: swallowing or breathing difficulty, burning
urination.
• Past medical hx:
–
–
1- previous episode of SJS.
2- symptoms of recent infection (2wks ago)
e.g. URTI esp. pedia pt.
• Drug hx: recent prescribtion (2wks).

12. 5. Physical examination
• Skin: targetoid lesion (only two zones)
– macules that develop into papules, vesicles,
bullae, urticarial plaques, or confluent
erythema, bulluea, rapture, secondary
infection (death).
– center: vesicular, purpuric, or necrotic
• Mucus: erythema, edema, sloughing,
blistering, ulceration 》 airway obstruction
(death), ophthalmological complications

13. 6. Exclude DDx
• EMM: same mucus lesion but different
cutanous: Target lesion + no blisters,
recurrent, mainly extremities.
• SSSS: in children and rarely adult, biopsy.
• Burn
• Exfoliative dermatitis.

14. ?
Atypical Target (targetoid): 2
zones> SJS/TEN
Typical Target: 3 zones > EM

15. 7. Investigations
• Looking For diagnosis: Biopsy
confirmative but not practical.
• Looking For complications: CBC (2ry
infection, anemia, neutropenia),
electrolytes( dehydration), RFT (renal
failure).
• Looking for the cuase: LFT.

16. Dermatopathology
– Dermal infiltration.
– Epidermal full thickness necrosis.

17. 8. Management
• ABCDEF
• BURN! grade 3 but lesser fluid.
• Symptomatic Rx.

19. All except one study [72], confirm
the known excellent tolerability and
a low toxic potential
with each 1 g/Kg increase in IVIG
dose, there was a 4.2-fold increase
in TEN patient survival, which was
statistically significant, (3 early >
zero mortality). Trent et al.
Contraindications: renal insufficiency,
cardiac insufficiency, IgA deficiency,
thrombo-embolic risk.

20. Wound care

21. 9. Complications;
More than 50% of patients surviving TEN suffer from long-term sequelae
I.
Cutaneous: 2ry infection, deformity,
hypo/hyprerpegmentation.
II. Mucosal: mucosal pseudomembrane formation
lead to mucosal scarring and fibrosis, obstruction
e.g. Esophageal strictures, Renal tubular
necrosis, renal failure, penile scarring, vaginal
stenosis, Tracheobronchial shedding with
resultant respiratory failure.
III. Ophthalmic; up to 40% in TEN; blindness 10%.

22. 10. Prognosis
• Mostly cure within 2 wks.
• mortality rate: SJS(1-5%), TEN (25-35%)
• SCORTEN predict the mortality;

23. Prevention
• Detailed history for any pt: allergy
• Start anti epileptic gradually
• Allergological testing; to prevent second
episode (not practical; under
investigations” ex vivo/in vitro” ).

24. Home message
• Target lesion + mucus involvement 》 call
the dermatologist.
• ABCDE management and stop ALL not
necessary drugs.

25. References
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