Čes. stomatol. Prakt. zub. lék. (Czech Dental Journal) 2010; 110(3): 39-44

Stevens-Johnson Syndrome and Its Manifestation in Oral Cavity

J. Vokurka1,*, A. Fassmann1, S. Zgažarová2, P. Augustín1, L. Izakovičová Hollá1,3, J. Vaněk1
1 Stomatologická klinika LF MU a FN u sv. Anny, Brno
2 1. dermatovenerologická klinika LF MU a FN u sv. Anny, Brno
3 Ústav patologické fyziologie LF MU, Brno

Stevens-Johnson syndrome (SJS) is a disease with typical dermal and mucosal manifestations. The etiology is multifactorial. The characteristic skin manifestation is the occurrence of brick-red stains or papular dermatitis with central decrease, sc. iris form. In the efflorescence center blebs are formed. The mucosa of oral cavity displays planar erosions which originate from ripping off the blebs by mastication movements.
At the periodontology ward of Stomatology Clinic Medical Faculty MU and FNBUSA in Brno the authors examined a nineteen-year patients with hemorrhagic crusts on the lips and erosions with exudation and fibrin cover, which occurred on the buccal mucosa. The tongue became swollen with edema with intensified white coating and later impressions of teeth. The patient suffered form increased salivation and pains during food intake and impossibility to maintain oral hygiene, being weakened and dehydrated. Two days later skin efflorescence occurred in the area of knees, loins and glans penis. The manifestations in oral cavity were treated at the periodontology ward and the patient was sent to hospitalization at the 1st Dermatovenerology Clinic Medical Faculty MU and FNBUSA in Brno.
The case report presents the etiology, clinical course, diagnostics and therapy of the disease.

Keywords: Stevens-Johnson syndrome (SJS); Erythema exsudativum multiforme (Hebrae); SCAR; iris; corticoids; IVIG; cyclosporin

Published: September 1, 2010  Show citation

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Vokurka J, Fassmann A, Zgažarová S, Augustín P, Izakovičová Hollá L, Vaněk J. Stevens-Johnson Syndrome and Its Manifestation in Oral Cavity. Čes. stomatol. Prakt. zub. lék. 2010;110(3):39-44.
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References

  1. Araki, Y., Sotozono, C., Anatomi, T., Ueta, M., Yokoi, N., Ueda, E., Kishimoto, S., Kinoshita, S.: Successful treatment of Stevens-Johnson syndrome with steroid pulse therapy at disease onset. Am. J. Ophthalmol., roč. 147, 2009, s. 1004-1011. Go to original source... Go to PubMed...
  2. Aurelian, L., Ono, F., Burnett, J.: Herpes simplex virus (hsv)-associated erythema multiforme (haem): A viral disease with an autoimmune component. Dermatol. Online J., roč. 9, 2003, s. 1. Go to original source...
  3. Bastuji-Garin, S., Rzany, B., Stern, R., Sudar, N., Naldi, L., Roujeau, J.: Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme. Arch. Dermatol., roč. 129, 1993, s. 92-96. Go to original source... Go to PubMed...
  4. Caproni, M., Torchia, D., Schincaglia, E., Volpi, W., Frezzolini A., Schena D., Marzano A., Quaglino P., De Simone C., Parodi A., Barletta E., Fabbri P.: The cd40/cd40 ligand system is expressed in the cutaneous lesions of erythema multiforme and Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum. Br. J. Dermatol., roč. 154, 2006, s. 319-324. Go to original source... Go to PubMed...
  5. French, L., Trent, J., Krdel, F.: Use of intravenous immunoglobulin in toxic epidermal necrolysis and Stevens-Johnson syndrome: Our current understanding. Int. Immunopharmacol, roč. 6, 2006, s. 543-549. Go to original source... Go to PubMed...
  6. Fromowitz, J., Ramos-Caro, F., Flowers, F.: Practical guidelines for the management of toxic epidermal necrolysis and Stevens-Johnson syndrome. Int. J. Dermatol., roč. 46, 2007, s. 1092-1094. Go to original source... Go to PubMed...
  7. Chave, T. A., Mortimer, N. J., Sladden, M. J., Hall, A. P., Hutchinson, P. E.: Toxic epidermal necrolysis: Current evidence, practical management and future directions. British Journal of Dermatology, roč. 153, 2005, s. 241-253. Go to original source... Go to PubMed...
  8. Chung, W., Hung, S., Hong, H., Hsih, M., Yang, L., Ho, H., Wu, J., Chen, Y.: Medical genetics: A marker for Stevens-Johnson syndrome. Nature, roč. 428, 2004, s. 486. Go to original source... Go to PubMed...
  9. Izakovičová Hollá, L., Fassmann, A., Kupletová, M., Sazmová, V.: Papillon-Lefevre syndrom: kazuistika. Praktické zubní lékařství, roč. 57, 2009, s. 31-36.
  10. Khalili, B., Bahna, S.: Pathogenesis and recent therapeutic trends in Stevens-Johnson syndrome and toxic epidermal necrolysis. Ann Allergy Asthma Immunol., roč. 97, 2006, s. 272-280. Go to original source... Go to PubMed...
  11. Lam, N., Yang, Y., Wang, L., Lin, Y., Chiang, B.: Clinical characteristics of childhood erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis in taiwanese children. J. Microbiol. Immunol. Infect, roč. 37, 2004, s. 366-370. Go to PubMed...
  12. Le Clech, L., Delaire, S., Boumsell, L., Bagot, M., Bourgault-Villada, I., Bensussan, A., Roujeau, J.: Blister fluid t lymphocytes during toxic epidermal necrolysis are functional cytotoxic cells which express human natural killer (nk) inhibitory receptors. Clin. Exp. Immunol., roč. 119, 2000, s. 225-230. Go to original source... Go to PubMed...
  13. Levi, N., Bastuji-Garin, S., Mockenhaupt, M., Roujeau, J. C., Flahault, A., Kelly, J. P., Martin, E., Kaufman, D. W., Mason, P.: Medications as risk factors of Stevens-Johnson syndrome and toxic epidermal necrolysis in children: A pooled analysis. Pediatrics, roč. 123, 2009, s. 297-304. Go to original source... Go to PubMed...
  14. Locharernkul, C., Loplumlert, J., Limotai, C., Korkij, W., Desudchit, T., Tongkobpetch, S., Kangwanshiratada, O., Hirankarn, N., Suphapeetiporn, K., Shotelersuk, V.: Carbamazepine and phenytoin induced Stevens-Johnson syndrome is associated with hla-b*1502 allele in thai population. Epilepsia, roč. 49, 2008, s. 2087-2091. Go to original source... Go to PubMed...
  15. Lonjou, C., Borot, N., Sekula, P., Ledger, N., Thomas, L., Halevy, S., Naldi, L., Bouwes-Bavinck, J., Sidoroff, A., de Toma, C., Schumacher, M., Roujeau, J., Hovnanian, A., Mockenhaupt, M.: A european study of hla-b in Stevens-Johnson syndrome and toxic epidermal necrolysis related to five high-risk drugs. Pharmacogenet Genomics, roč. 18, 2008, s. 99-107. Go to original source... Go to PubMed...
  16. Man, C., Kwan, P., Baum, L., Yu, E., Lau, K., Cheby, A., Ng, M.: Association between hlab* 1502 allele and antiepileptic drug-induced cutaneous reactions in han chinese. Epilepsia, roč. 48, 2007, s. 1015-1018. Go to original source... Go to PubMed...
  17. Miller, J.: Of race, ethnicity, and rash: The genetics of antiepileptic drug-induced skin reactions. Epilepsy Curr, roč. 8, 2008, s. 120-121. Go to original source... Go to PubMed...
  18. Mockenhaupt, M., Vinouc, C., Dunant, A., Naldi, L., Halevy, S., Bouwes Bavinck, J., Sidoroff, A., Schneck, J., Roujeau, J., Flahault, A.: Stevens-Johnson syndrome and toxic epidermal necrolysis: Assessment of medication risks with emphasis on recently marketed drugs. The euroscar-study. J. Incest. Dermatos., roč. 128, 2008, s. 35-44. Go to original source... Go to PubMed...
  19. Mulvey, J., Padowitz, A., Lindley-Jones, M., Nickels, R.: Mycoplasma pneumonie associated with Stevens Johnson syndrome. Anaesth Intensive Care, roč. 35, 2007, s. 414-417. Go to original source... Go to PubMed...
  20. Pereira, F., Mudgil, A., Rosmarin, D.: Toxic epidermal necrolysis. J. Am. Acad. Dermatol., roč. 56, 2007, s. 181-200. Go to original source... Go to PubMed...
  21. Posada, S., Padial, A., Torres, M., Katorga, C., Leyva, L., Sanchez, E., Alvarez, J., Romano, A., Juarez, C., Blanca, M.: Delayed reactions to drugs show levels of perforin, granzyme b, and fas-l to be related to disease severity. J. Allergy Clin. Immunol., roč. 109, 2002, s. 155-161. Go to original source... Go to PubMed...
  22. Qin, J., Chaturvedi, V., Denning, M., Choubey, D., Diaz, M., Nickoloff, B.: Role of nfkappab in the apoptotic-resistant phenotype of keratinocytes. J. Biol. Chem., roč. 274, 1999, s. 37957-37964. Go to original source... Go to PubMed...
  23. Roujeau, J.: The spectrum of Stevens-Johnson syndrome and toxic epidermal necrolysis: A clinical classification. J. Incest. Dermatol., roč. 102, 1994, s. 28-30. Go to original source... Go to PubMed...
  24. Roujeau, J., Kelly, J., Naldi, L., Rzany, B., Stern, R., Anderson, T., Auquier, A., Bastuji-Garin, S., Correia, O., Počati, F.: Medication use and the risk of Stevens-Johnson syndrome or toxic epidermal necrolysis. N. Engl. J. Med., roč. 333, 1995, s. 1600-1607. Go to original source... Go to PubMed...
  25. Scully, C., Bagan, J.: Oral mucosal diseases: Erythema multiforme. Br. J. Oral Maxillofac. Surg., roč. 46, 2008, s. 90-95. Go to original source... Go to PubMed...
  26. Stur, K., Karlhofer, F., Stingl, G.: Soluble fas ligand: A discriminating feature between drug-induced skin eruptions and viral exanthemas. J. Incest. Dermatol., roč. 127, 2007, s. 802-807. Go to original source... Go to PubMed...
  27. Schneck, J., Fagot, J., Sekula, P., Sassolas, B., Roujeau, J., Mockenhaupt, M.: 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., roč. 58, 2008, s. 33-40. Go to original source... Go to PubMed...
  28. Tassaneeyakul, W., Jantararoungtong, T., Chen, P., Lin, P., Tiamkao, S., Khunarkornsiri, U., Chucherd, P., Konyoung, P., Vannaprasaht, S., Choonhakarn, C., Pisuttimarn, P., Sangviroon, A.: Strong association between hla-b*5801 and allopurinol-induced Stevens-Johnson syndrome and toxic epidermal necrolysis in a thai population. Pharmacogenet Genomics, roč. 19, 2009, s. 704-709. Go to original source... Go to PubMed...
  29. Ueta, M., Sotozono, C., Anatomi, T., Kojima, K., Hamito, J., Kinoshita, S.: Association of fas ligand gene polymorphism with Stevens-Johnson syndrome. Br. J. Ophthalmol., roč. 92, 2008, s. 989-991. Go to original source... Go to PubMed...
  30. Viard, I., Wehrli, P., Bullani, R., Schneider, P., Holler, N., Salomon, D., Hunziker, T., Saurat, J. H., Tschopp, J., French, L. E.: Inhibition of toxic epidermal necrolysis by blockade of cd95 with human intravenous immunoglobulin. Science, roč. 282, 1998, s. 490-493. Go to original source... Go to PubMed...

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