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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.

Stevens-Johnson syndrome/ Toxic epidermal necrolysis emergency guidelines Stevens-Johnson syndrome/ Toxic epidermal necrolysis emergency guidelines Presentation Transcript

  • SJS TEN ER guidelines; Anti-fas may be promising & steroid still controversial Daifallah Almansouri MD Venue: KF Hospital, SA. Date: 22nd Dec 2013
  • 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.
  • 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.
  • NB. BSA= detached+ detachable (+Nikolsky)
  • Nikolsky+
  • 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.
  • Medications association
  • Hx of Gout
  • 3. Pathophysiology
  • 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).
  • 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
  • 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.
  • ? 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 infection, anemia, neutropenia), electrolytes( dehydration), RFT (renal failure). • Looking for the cuase: LFT.
  • 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 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.
  • Wound care
  • 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%.
  • 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 second episode (not practical; under investigations” ex vivo/in vitro” ).
  • Home message • Target lesion + mucus involvement 》 call the dermatologist. • ABCDE management and stop ALL not necessary drugs.
  • 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].
  • 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].