Stevens-Johnson Syndrome

Czech version

Authors: L. Čábalová ¹; Pavel Komínek ¹; Karol Zeleník ^1,2
Authors‘ workplace: Otorinolaryngologická klinika Fakultní nemocnice Ostrava ; přednosta doc. MUDr. P. Komínek, Ph. D., MBA Katedra chirurgických oborů, Lékařské fakulta, Ostravská Univerzita v Ostravě ¹; vedoucí katedry doc. MUDr. P. Zonča, Ph. D., FRCS ²
Published in: Otorinolaryngol Foniatr, 62, 2013, No. 4, pp. 218-220.
Category: Case History

Overview

Stevens-Johnson syndrome (SJS) is a severe skin and musocal dmini with eruption of hemorrhagic dmini lesions, caused by hypersensitive reaction to drugs, rarely it can be parainfectious. It is a from of toxic epidermal necrolysis, with involvement of less then 10 % of skin surface. Cutaneous and mucous lesions are caused by necrosis of epithelial cells mediated by T-lymphocytes. The diagnosis is based on clinical features, while a definitive confirmation requires histologic and immunohistologic examination of the lesions. The therapy is based on administration of high doses of corticosteroids and especially thorough local, supportive and symptomatic therapy. The authors present a case of parainfectious SJS and discuss the latest knowledge about SJS as well as therapeutical possibilities.

Keywords:
Stevens-Johnson syndrome, toxic epidermal necrolysis, erythema multiforme

Sources

1. Clayton, N. A., Kennedy, P. J.: Management of dysphagia in toxic epidermal necrolysis (TEN) and Stevens-Johnson Syndrome (SJS). Dysphagia, 22, 2007, s. 187-192.

2. Colebunders, R., Vanwolleghem, T., Meurrens, P., Moer-man, F.: Efavirenz-associated Stevens-Johnson Syndrome. Infection, 32, 2004, s. 306-307.

3. Génin, E., Schumacher, M., Roujeau, J. C., Naldi, L., Liss, Y., Kazma, R., Sekula, P., Hovnovian, A., Mockenhaupt, M.: Genome-wide association study of Stevens-Johnson Syndrome and toxic epidermal necrolysis in Europe. Orphanet. J. Rare Dis., 6, 2011, s. 52-61.

4. Harr, T., French, L. E.: Toxic epidermal necrolysis and Stevens-Johnson syndrome. Orphanet. J. Rare Dis., 5, 2010, s. 39-49.

5. Lang, F.: Encyclopedia of molecular mechanisms of disease. Springer-Verlag GmbH, Berlin Heidelberg, 2009, 2270 s.

6. Manwani, N. S., Balasubramanian, S., Dhanalakshmi, K., Sumanth, A.: Stevens-Johnson Syndrome in association with mycoplasma pneumoniae. Indian J. Pediatr, published online, 20, 2011. Dostupné z: http://www.springerlink.com/content/h08u347w10406827/.

7. Revuz, J., Roujeau, J. C., Kerdel, F. A., Valeyrie-Allanore, L.:Life-treatening dermatoses and emergencies in dermatology. Springer-Verlag GmbH, Berlin Heidelberg, 2009, 329 s.

8. Vokurka, M., Hugo, J. a kol.: Velký lékařský slovník. 9. vyd., Maxdorf, Praha, 2010, 1160 s.