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.
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.
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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Litt’s DERM 19th edition.
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Editor's Notes
HIV: 1/1000
detached (e.g. blisters, erosions) ordetachable skin (Nikolsky positive) should be includedin the evaluation of the extent of skin involvement.
basket weave-like pattern of the stratum corneum.
Maintainurine output 50 -80 mL per hour with 0.5% NaCl supplemented with 20mEq of KCl.2- mortality of patient transferred to a burn unitewithin 7 days after disease-onset compared with patientsadmitted after 7 days (29.8% vs 51.4% (p < 0.05)).3- without skin debridement which is often performed inburn units, as blistered skin acts as a natural biologicaldressing which likely favors re-epithelialization.
Trentet al. analyzed the published literature between 1992and 2006notably the mortality was zero percentin the subset of 30 patients treated with more than 3 g/kg total dose of IVIG.d (p = 0.1), suggesting that,although not statistically significant, ciclosporin may beuseful for the treatment of TEN.The published data is cur-rently insufficient to draw a conclusion on thetherapeutic potential of TNF antagonists in TEN.
It should be emphasized that only necrotic skin,which is already detached (e.g. blisters, erosions) ordetachable skin (Nikolsky positive) should be includedin the evaluation of the extent of skin involvement.