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SJS TEN ER guidelines;
Anti-fas may be promising & steroid still controversial

Daifallah Almansouri MD
Venue: KF Hospital...
Outcomes
1. Definition and epidemiology.
2. Etiology
3. Pathogenesis.
4. Symptomology.
5. Clinical examination.
6. Exclude...
1. Definition and epidemiology
• SJS/ TEN is immune-complex–mediated
hypersensitivity causes extensive
apoptosis of the sk...
NB. BSA= detached+ detachable (+Nikolsky)
Nikolsky+
2. Etiology
I. 25-50% iodopathic.
II. Defined cause:
– infection: mostly in pediatric
GABHS (URTI), HSV.
– malignancy: mos...
Medications association
Hx of Gout
3. Pathophysiology
4. Symptomology
• HPI:
– Prodromal systemic symptoms: fever, maliase, ....
– Cutanous: abrubt erythematous tender rashes.
...
5. Physical examination
• Skin: targetoid lesion (only two zones)
– macules that develop into papules, vesicles,
bullae, u...
6. Exclude DDx
• EMM: same mucus lesion but different
cutanous: Target lesion + no blisters,
recurrent, mainly extremities...
?
Atypical Target (targetoid): 2
zones> SJS/TEN

Typical Target: 3 zones > EM
7. Investigations
• Looking For diagnosis: Biopsy
confirmative but not practical.
• Looking For complications: CBC (2ry
in...
Dermatopathology
– Dermal infiltration.
– Epidermal full thickness necrosis.
8. Management
• ABCDEF
• BURN! grade 3 but lesser fluid.
• Symptomatic Rx.
All except one study [72], confirm
the known excellent tolerability and
a low toxic potential
with each 1 g/Kg increase in...
Wound care
9. Complications;
More than 50% of patients surviving TEN suffer from long-term sequelae

I.

Cutaneous: 2ry infection, de...
10. Prognosis
• Mostly cure within 2 wks.
• mortality rate: SJS(1-5%), TEN (25-35%)
• SCORTEN predict the mortality;
Prevention
• Detailed history for any pt: allergy
• Start anti epileptic gradually
• Allergological testing; to prevent se...
Home message
• Target lesion + mucus involvement 》 call
the dermatologist.
• ABCDE management and stop ALL not
necessary d...
References
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Litt’s DERM 19th edition.
http://www.ojrd.com/content/...
References
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Belkahia A, Hillaire-Buys D, Dereure O, Guillot B, Raison-P...
Stevens-Johnson syndrome/ Toxic epidermal necrolysis emergency guidelines
Stevens-Johnson syndrome/ Toxic epidermal necrolysis emergency guidelines
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Stevens-Johnson syndrome/ Toxic epidermal necrolysis emergency guidelines

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  • 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.
  • Transcript of "Stevens-Johnson syndrome/ Toxic epidermal necrolysis emergency guidelines"

    1. 1. SJS TEN ER guidelines; Anti-fas may be promising & steroid still controversial Daifallah Almansouri MD Venue: KF Hospital, SA. Date: 22nd Dec 2013
    2. 2. 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.
    3. 3. 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.
    4. 4. NB. BSA= detached+ detachable (+Nikolsky)
    5. 5. Nikolsky+
    6. 6. 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.
    7. 7. Medications association
    8. 8. Hx of Gout
    9. 9. 3. Pathophysiology
    10. 10. 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).
    11. 11. 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
    12. 12. 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.
    13. 13. ? Atypical Target (targetoid): 2 zones> SJS/TEN Typical Target: 3 zones > EM
    14. 14. 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.
    15. 15. Dermatopathology – Dermal infiltration. – Epidermal full thickness necrosis.
    16. 16. 8. Management • ABCDEF • BURN! grade 3 but lesser fluid. • Symptomatic Rx.
    17. 17. 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.
    18. 18. Wound care
    19. 19. 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%.
    20. 20. 10. Prognosis • Mostly cure within 2 wks. • mortality rate: SJS(1-5%), TEN (25-35%) • SCORTEN predict the mortality;
    21. 21. 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” ).
    22. 22. Home message • Target lesion + mucus involvement 》 call the dermatologist. • ABCDE management and stop ALL not necessary drugs.
    23. 23. References • • • • • • • • • • • • • • • • • • • • • • • • • • • Litt’s DERM 19th edition. http://www.ojrd.com/content/pdf/1750-1172-5-39.pdf Summerzed from Medscape Aug 2013 French LE. Toxic epidermal necrolysis and Stevens Johnson syndrome: our current understanding. Allergol Int. Mar 2006;55(1):9-16. [Medline]. Lowes R. Acetaminophen poses risk for rare but fatal skin reactions. Medscape Medical News [serial online]. August 1, 2013;Accessed August 4, 2013. Available athttp://www.medscape.com/viewarticle/808807. FDA Drug Safety Communication. FDA warns of rare but serious skin reactions with the pain reliever/fever reducer acetaminophen. Available at http://www.fda.gov/Drugs/DrugSafety/ucm363041.htm. Accessed August 4, 2013. Roujeau JC. Stevens-Johnson syndrome and toxic epidermal necrolysis are severity variants of the same disease which differs from erythema multiforme. J Dermatol. Nov 1997;24(11):726-9. [Medline]. Rotunda A, Hirsch RJ, Scheinfeld N, Weinberg JM. Severe cutaneous reactions associated with the use of human immunodeficiency virus medications. Acta Derm Venereol. 2003;83(1):1-9. [Medline]. Gruchalla RS. 10. Drug allergy. J Allergy Clin Immunol. Feb 2003;111(2 Suppl):S548-59. [Medline]. Ahmed AR, Dahl MV. Consensus statement on the use of intravenous immunoglobulin therapy in the treatment of autoimmune mucocutaneous blistering diseases. Arch Dermatol. Aug 2003;139(8):1051-9.[Medline]. Assier-Bonnet H, Aractingi S, Cadranel J, Wechsler J, Mayaud C, Saiag P. Stevens-Johnson syndrome induced by cyclophosphamide: report of two cases. Br J Dermatol. Nov 1996;135(5):864-6. [Medline]. De Rojas MV, Dart JK, Saw VP. The natural history of Stevens Johnson syndrome: patterns of chronic ocular disease and the role of systemic immunosuppressive therapy. Br J Ophthalmol. Aug 2007;91(8):1048-53. [Medline]. [Full Text]. Morel E, Escamochero S, Cabañas R, Díaz R, Fiandor A, Bellón T. CD94/NKG2C is a killer effector molecule in patients with Stevens-Johnson syndrome and toxic epidermal necrolysis. J Allergy Clin Immunol. Mar 2010;125(3):703-10, 710.e1-710.e8. [Medline]. Inachi S, Mizutani H, Shimizu M. Epidermal apoptotic cell death in erythema multiforme and Stevens-Johnson syndrome. Contribution of perforin-positive cell infiltration. Arch Dermatol. Jul 1997;133(7):845-9.[Medline]. Foster CS, Fong LP, Azar D, Kenyon KR. Episodic conjunctival inflammation after Stevens-Johnson syndrome. Ophthalmology. Apr 1988;95(4):453-62. [Medline]. Murata J, Abe R, Shimizu H. Increased soluble Fas ligand levels in patients with Stevens-Johnson syndrome and toxic epidermal necrolysis preceding skin detachment. J Allergy Clin Immunol. Nov 2008;122(5):992-1000. [Medline]. French LE, Trent JT, Kerdel FA. Use of intravenous immunoglobulin in toxic epidermal necrolysis and Stevens-Johnson syndrome: our current understanding. Int Immunopharmacol. Apr 2006;6(4):543-9.[Medline]. Halevy S, Ghislain PD, Mockenhaupt M, et al. Allopurinol is the most common cause of Stevens-Johnson syndrome and toxic epidermal necrolysis in Europe and Israel. J Am Acad Dermatol. Jan 2008;58(1):25-32.[Medline]. Schlienger RG, Shapiro LE, Shear NH. Lamotrigine-induced severe cutaneous adverse reactions.Epilepsia. 1998;39 Suppl 7:S22-6. [Medline]. Mockenhaupt M, Messenheimer J, Tennis P, Schlingmann J. Risk of Stevens-Johnson syndrome and toxic epidermal necrolysis in new users of antiepileptics. Neurology. Apr 12 2005;64(7):1134-8. [Medline]. Horne NS, Narayan AR, Young RM, Frieri M. Toxic epidermal necrolysis in systemic lupus erythematosus.Autoimmun Rev. Feb 2006;5(2):160-4. [Medline]. Hillebrand-Haverkort ME, Budding AE, bij de Vaate LA, van Agtmael MA. Mycoplasma pneumoniae infection with incomplete Stevens-Johnson syndrome. Lancet Infect Dis. Oct 2008;8(10):586-7. [Medline]. Sendi P, Graber P, Lepère F, Schiller P, Zimmerli W. Mycoplasma pneumoniae infection complicated by severe mucocutaneous lesions. Lancet Infect Dis. Apr 2008;8(4):268. [Medline]. Hällgren J, Tengvall-Linder M, Persson M, Wahlgren CF. Stevens-Johnson syndrome associated with ciprofloxacin: a review of adverse cutaneous events reported in Sweden as associated with this drug. J Am Acad Dermatol. Nov 2003;49(5 Suppl):S267-9. [Medline]. Mockenhaupt M, Messenheimer J, Tennis P, Schlingmann J. Risk of Stevens-Johnson syndrome and toxic epidermal necrolysis in new users of antiepileptics. Neurology. Apr 12 2005;64(7):1134-8. [Medline]. Metry DW, Lahart CJ, Farmer KL, Hebert AA. Stevens-Johnson syndrome caused by the antiretroviral drug nevirapine. J Am Acad Dermatol. Feb 2001;44(2 Suppl):3547. [Medline]. Halevy S, Ghislain PD, Mockenhaupt M, et al. Allopurinol is the most common cause of Stevens-Johnson syndrome and toxic epidermal necrolysis in Europe and Israel. J Am Acad Dermatol. Jan 2008;58(1):25-32.[Medline].
    24. 24. References • • • • • • • • • • • • • • • • • • • • • • • • • Belkahia A, Hillaire-Buys D, Dereure O, Guillot B, Raison-Peyron N. Stevens-Johnson syndrome due to mirtazapine - first case. Allergy. Oct 2009;64(10):1554. [Medline]. Salama M, Lawrance IC. Stevens-Johnson syndrome complicating adalimumab therapy in Crohn's disease.World J Gastroenterol. Sep 21 2009;15(35):444952. [Medline]. [Full Text]. Kardaun SH, Jonkman MF. Dexamethasone pulse therapy for Stevens-Johnson syndrome/toxic epidermal necrolysis. Acta Derm Venereol. 2007;87(2):144-8. [Medline]. Fernando SL, Broadfoot AJ. Prevention of severe cutaneous adverse drug reactions: the emerging value of pharmacogenetic screening. CMAJ. Mar 23 2010;182(5):47680. [Medline]. [Full Text]. Hynes AY, Kafkala C, Daoud YJ, Foster CS. Controversy in the use of high-dose systemic steroids in the acute care of patients with Stevens-Johnson syndrome. Int Ophthalmol Clin. Fall 2005;45(4):25-48.[Medline]. Khalili B, Bahna SL. Pathogenesis and recent therapeutic trends in Stevens-Johnson syndrome and toxic epidermal necrolysis. Ann Allergy Asthma Immunol. Sep 2006;97(3):272-80; quiz 281-3, 320. [Medline]. Meth MJ, Sperber KE. Phenotypic diversity in delayed drug hypersensitivity: an immunologic explanation.Mt Sinai J Med. Sep 2006;73(5):769-76. [Medline]. Strom BL, Carson JL, Halpern AC, et al. A population-based study of Stevens-Johnson syndrome. Incidence and antecedent drug exposures. Arch Dermatol. Jun 1991;127(6):831-8. [Medline]. Bastuji-Garin S, Fouchard N, Bertocchi M, et al. SCORTEN: a severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol. Aug 2000;115(2):14953. [Medline]. de Prost N, Ingen-Housz-Oro S, Duong T, et al. Bacteremia in Stevens-Johnson syndrome and toxic epidermal necrolysis: epidemiology, risk factors, and predictive value of skin cultures. Medicine (Baltimore). Jan 2010;89(1):28-36. [Medline]. Sekula P, Dunant A, Mockenhaupt M, Naldi L, Bouwes Bavinck JN, Halevy S, et al. Comprehensive Survival Analysis of a Cohort of Patients with Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis.J Invest Dermatol. Feb 7 2013;[Medline]. Vera LS, Gueudry J, Delcampe A, et al. In vivo confocal microscopic evaluation of corneal changes in chronic Stevens-Johnson syndrome and toxic epidermal necrolysis. Cornea. May 2009;28(4):401-7.[Medline]. Shammas MC, Lai EC, Sarkar JS, Yang J, Starr CE, Sippel KC. Management of acute Stevens-Johnson syndrome and toxic epidermal necrolysis utilizing amniotic membrane and topical corticosteroids. Am J Ophthalmol. Feb 2010;149(2):203-213.e2. [Medline]. Tseng SC. Acute management of Stevens-Johnson syndrome and toxic epidermal necrolysis to minimize ocular sequelae. Am J Ophthalmol. Jun 2009;147(6):94951. [Medline]. Paquet P, Paquet F, Al Saleh W, Reper P, Vanderkelen A, Piérard GE. Immunoregulatory effector cells in drug-induced toxic epidermal necrolysis. Am J Dermatopathol. Oct 2000;22(5):413-7. [Medline]. Sotozono C, Ueta M, Koizumi N, et al. Diagnosis and treatment of Stevens-Johnson syndrome and toxic epidermal necrolysis with ocular complications. Ophthalmology. Apr 2009;116(4):685-90. [Medline]. Sotozono C, Ueta M, Kinoshita S. Systemic and local management at the onset of Stevens-Johnson syndrome and toxic epidermal necrolysis with ocular complications. Am J Ophthalmol. Feb 2010;149(2):354; author reply 355. [Medline]. Araki Y, Sotozono C, Inatomi T, et al. Successful treatment of Stevens-Johnson syndrome with steroid pulse therapy at disease onset. Am J Ophthalmol. Jun 2009;147(6):1004-11, 1011.e1. [Medline]. Koh MJ, Tay YK. Stevens-Johnson syndrome and toxic epidermal necrolysis in Asian children. J Am Acad Dermatol. Jan 2010;62(1):54-60. [Medline]. Patterson R, Dykewicz MS, Gonzalzles A, et al. Erythema multiforme and Stevens-Johnson syndrome. Descriptive and therapeutic controversy. Chest. Aug 1990;98(2):331-6. [Medline]. Power WJ, Ghoraishi M, Merayo-Lloves J, Neves RA, Foster CS. Analysis of the acute ophthalmic manifestations of the erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis disease spectrum. Ophthalmology. Nov 1995;102(11):1669-76. [Medline]. Hebert AA, Bogle MA. Intravenous immunoglobulin prophylaxis for recurrent Stevens-Johnson syndrome. J Am Acad Dermatol. Feb 2004;50(2):286-8. [Medline]. Schneck J, Fagot JP, Sekula P, et al. Effects of treatments on the mortality of Stevens-Johnson syndrome and toxic epidermal necrolysis: A retrospective study on patients included in the prospective EuroSCAR Study. J Am Acad Dermatol. Jan 2008;58(1):33-40. [Medline]. Pehr K. The EuroSCAR study: cannot agree with the conclusions. J Am Acad Dermatol. Nov 2008;59(5):898-9; author reply 899-900. [Medline]. Power WJ, Saidman SL, Zhang DS, et al. HLA typing in patients with ocular manifestations of Stevens-Johnson syndrome. Ophthalmology. Sep 1996;103(9):14069. [Medline].

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