Advertisement

Purpuric Macules and Oral Erosions

  • Dipak Kr. Agarwalla
  • Piyush Kumar
Chapter

Abstract

  • Presence of widespread purpuric macules and patches, and atypical target lesions along with erosive and hemorrhagic mucositis of the mouth, eyes, nose and genitalia suggest the clinical diagnosis of Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN)

  • SJS/TEN is primarily a drug-induced phenomenon with the culprit drug being demonstrated in approximately 85% of cases

  • There is a latent period of 7–10 days, ranging from 5 to 28 days, between initiation of the culprit drug and onset of SJS/TEN

  • SCORTEN score is a reliable and validated tool to assess severity of disease and predict mortality of SJS/TEN

Keywords

Stevens-Johnson syndrome Toxic epidermal necrolysis Target lesions SCORTEN 

References

  1. 1.
    Dodiuk-Gad RP, Chung WH, Valeyrie-Allanore L, Shear NH. Stevens-Johnson syndrome and toxic epidermal necrolysis: an update. Am J Clin Dermatol. 2015;16(6):475–93.CrossRefGoogle Scholar
  2. 2.
    Kasperkiewicz M, Ellebrecht CT, Takahashi H, Yamagami J, Zillikens D, Payne AS, Amagai M. Pemphigus. Nat Rev Dis Primers. 2017;3:17026.CrossRefGoogle Scholar
  3. 3.
    Handler MZ, Schwartz RA. Staphylococcal scalded skin syndrome: diagnosis and management in children and adults. J Eur Acad Dermatol Venereol. 2014;28(11):1418–23.CrossRefGoogle Scholar
  4. 4.
    Roujeau JC. Immune mechanisms in drug allergy. Allergol Int. 2006;55(1):27–33.CrossRefGoogle Scholar
  5. 5.
    Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis: Part I. Introduction, history, classification, clinical features, systemic manifestations, etiology, and immunopathogenesis. J Am Acad Dermatol. 2013;69(2):173.e1–13; quiz 85–6CrossRefGoogle Scholar
  6. 6.
    Viard-Leveugle I, Gaide O, Jankovic D, Feldmeyer L, Kerl K, Pickard C, Roques S, Friedmann PS, Contassot E, French LE. TNF-alpha and IFN-gamma are potential inducers of Fas-mediated keratinocyte apoptosis through activation of inducible nitric oxide synthase in toxic epidermal necrolysis. J Invest Dermatol. 2013;133(2):489–98.CrossRefGoogle Scholar
  7. 7.
    Viard I, Wehrli P, Bullani R, Schneider P, Holler N, Salomon D, Hunziker T, Saurat JH, Tschopp J, French LE. Inhibition of toxic epidermal necrolysis by blockade of CD95 with human intravenous immunoglobulin. Science (New York, NY). 1998;282(5388):490–3.CrossRefGoogle Scholar
  8. 8.
    Khalili B, Bahna SL. Pathogenesis and recent therapeutic trends in Stevens-Johnson syndrome and toxic epidermal necrolysis. Ann Allergy Asthma Immunol. 2006;97(3):272–80; quiz 81–3, 320CrossRefGoogle Scholar
  9. 9.
    Chung WH, Hung SI, Yang JY, Su SC, Huang SP, Wei CY, Chin SW, Chiou CC, Chu SC, Ho HC, Yang CH, Lu CF, Wu JY, Liao YD, Chen YT. Granulysin is a key mediator for disseminated keratinocyte death in Stevens-Johnson syndrome and toxic epidermal necrolysis. Nat Med. 2008;14(12):1343–50.CrossRefGoogle Scholar
  10. 10.
    Burster T, Macmillan H, Hou T, Boehm BO, Mellins ED. Cathepsin G: roles in antigen presentation and beyond. Mol Immunol. 2010;47(4):658–65.CrossRefGoogle Scholar
  11. 11.
    Sassolas B, Haddad C, Mockenhaupt M, Dunant A, Liss Y, Bork K, Haustein UF, Vieluf D, Roujeau JC, Le Louet H. ALDEN, an algorithm for assessment of drug causality in Stevens-Johnson syndrome and toxic epidermal necrolysis: comparison with case-control analysis. Clin Pharmacol Ther. 2010;88(1):60–8.CrossRefGoogle Scholar
  12. 12.
    Vujic I, Shroff A, Grzelka M, Posch C, Monshi B, Sanlorenzo M, Ortiz-Urda S, Rappersberger K. Mycoplasma pneumoniae-associated mucositis--case report and systematic review of literature. J Eur Acad Dermatol Venereol. 2015;29(3):595–8.CrossRefGoogle Scholar
  13. 13.
    Harr T, French LE. Toxic epidermal necrolysis and Stevens-Johnson syndrome. Orphanet J Rare Dis. 2010;5:39.CrossRefGoogle Scholar
  14. 14.
    Bastuji-Garin S, Fouchard N, Bertocchi M, Roujeau JC, Revuz J, Wolkenstein P. SCORTEN: a severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol. 2000;115(2):149–53.CrossRefGoogle Scholar
  15. 15.
    Gupta LK, Martin AM, Agarwal N, D'Souza P, Das S, Kumar R, Pande S, Das NK, Kumaresan M, Kumar P, Garg A, Singh S. Guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis: an Indian perspective. Indian J Dermatol Venereol Leprol. 2016;82(6):603–25.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Dipak Kr. Agarwalla
    • 1
  • Piyush Kumar
    • 2
  1. 1.Department of DermatologyGauhati Medical College and HospitalGuwahatiIndia
  2. 2.Department of DermatologyKatihar Medical CollegeKatiharIndia

Personalised recommendations