Pemfigus

Pemfigus deri ve mükoz membranların büllöz, sık görülmeyen otoimmün kökenli bir hastalığıdır. Hastalığın patogenezinde keratinosit yüzeyindeki proteinlere karşı gelişen otoantikorların önemli rolü mevcuttur. Pemfigusun insidansı hastalığın tipine göre ülkeden ülkeye değişiklik gösterir. Pemfigus vulgaris tüm pemfigusluların yaklaşık %80’nini oluşturmaktadır ve pemfigus foliaseustan 10 kat daha fazla gözlenmektedir. Tedavide başta sistemik glukokortikoidler olmak üzere çeşitli immünsupresifler kullanılmaktadır ve son yıllarda yeni tedavi ajanları geliştirilmiştir. Bu derlemede pemfigus epidemiyolojisi, patogenezi, kliniği ve tedavisi güncel bilgiler ışığında gözden geçirilmiştir.

Pemphigus

Pemphigus is an uncommon autoimmune mucocutaneous blistering disease, mediated by antibodies directed against proteins present on the surface of keratinocytes that provide mechanical structure to the epidermis. Incidences of the various forms of pemphigus vary from country to country. Pemphigus vulgaris consists of the 80% of pemphigus cases and it occurs 10 times more than pemphigus foliaceus. There have been several recent advances in the management of pemphigus. Treatment of with systemic steroids and cytotoxic immunosuppressants can be highly effective. This article will discuss its epidemiology, pathogenesis, clinical variants and approaches to treatment.

PDF

___

1. Stanley JR. Pemphigus. In: Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz SI, Eds. Fitzpatrick’s Dermatology in General Medicine. 6th Ed, New York: McGraw-Hill Company 2003:558-67.
2. Amagai M. Vesiculobullous diseases, Pemphigus: In Dermatology. Bolognia JL, Jorizzo JL, Rapini RP 2nd Ed, London: Mosby, 2008;31:417-29.
3. Heng A, Nwanneshıudu A, Hashimoto T. Intraepidermal neutrophilic IgA/IgG antidesmocollin 1 pemphigus. Brit J Dermatol 2006;154(8):1018-9.
4. Uzun S, Durdu M, Akman A, et al. Pemphigus in the Mediterranean region of Turkey: A study of 148 cases. Int J Dermatol 2006;45(4):523-8.
5. Odom RB, James WD, Berger TG. Andrew’s Diseases of the Skin. 9th Ed, Philadelphia: WB Saunders Company 2000:574-605.
6. Ruocco E, Aurilia A, Ruocco V. Precautions and Suggestions for Pemphigus patients. Dermatology 2001;203(2):201-7.
7. Wucherpfennig KW, Yu B, Bhol K, Monos SD, et al. Structural basis for major histocompatibility complex (MHC).-linked susceptibility to autoimmunity: charged residues of a single MHC binding pocket confer selective presentation of self-peptides in pemphigus vulgaris. Proc Natl Acad Sci USA 1995;92(22):11935-9.
8. Uzun S. Büllü hastalıklar. Dermatoloji’de. Tüzün Y, Gürer MA, Serdaroğlu S, Oğuz O, Aksungur VL, ed. 3. baskı. İstanbul Nobel Tıp Kitabevi, 2008:807-32. 9. Brenner S, Sason A, Sharon O. Pemphigus and Infections. Clin Dermatol 2002;20(1):114-8.
10. Udey MC, Stanley JR. Pemphigus-Diseases of Antidesmosomal Autoimmunity. JAMA 1999;282(6):572-6.
11. Braun-Falco O, Plewig G, Wolff HH, Burgdorf WHC. Dermatology. 2nd Ed, Berlin:Springer-Verlag 2000;649-95.
12. Nguyen VT, Ndoye A, Grando SA. Pemphigus vulgaris antibody identifies pemphaxin. A novel keratinocyte annexin-like molecule binding acetylcholine. J Biol Chem 2000;275(38):29466-76.
13. Amagai M. Autoimmunity against desmosomal cadherins in pemphigus. J Dermatol Sci 1999;20(2):92-102.
14. Ishii N, Ishida-Yamamoto A, Hashimoto T. Immunolocalization of target autoantigens in IgA pemphigus. Clin Exp Dermatol 2004;29(1):62-6.
15. V’lckova-Laskosa MT, Laskosi DS, Kamberova S, Caca-Biljanovska N, Volckova N. Epidemiology of pemphigus in Macedonia: A 15-year retrospective study 1990-2004. Int J Dermatol 2007;46(2):253-8.
16. Cohen LM, Skopicki DK, Harrist TJ, Clark WH. Noninfectious Vesiculobullous and Vesiculopustular Diseases. In: Elder D, Elenitsas R, Jaworsky C, Johnson B, Eds. Lever’s Histopathology of the Skin. 8th Ed, Philadelphia: Lippincott-Raven 1997:209-52.
17. Bystryn JC, Rudolph JL. Pemphigus. Lancet 2005;366(25):61-73. 18. Uzun S. Otoimmün büllöz hastalıklarda laboratuar tanı. Dermatose 2002;2(1):42-6.
19. Turgutalp SÇ, Harman M. Pemfigusta Seyir ve Prognoz: 42 hastanın değerlendirilmesi. Dicle Tıp Dergisi 2008;35(1):29-31.
20. Yeh SW, Ahmed B, Sami N, Razzaque A. Blistering disorders: diagnosis and treatment. Dermatol Ther 2003;16(3):214-23.
21. Fellner MJ, Sapadin AN. Current therapy of pemphigus vulgaris. Mt Sinai J Med 2001; 68(4-5):268-78.
22. Harman KE, Albert S, Black MM; British Association of Dermatologists. Guidelines for the management of pemphigus vulgaris. Br J Dermatol 2003;149(8):926-37.
23. Bystryn JC. How should pemphigus be treated? J Eur Acad Dermatol Venereol 2002;16(4):562-3.
24. Mutasim DF. Management of autoimmune bullous diseases: pharmacology and therapeutics. J Am Acad Dermatol 2004;51:859-77.
25. Korman NJ. New immunomodulating drugs in autoimmune blistering diseases. Dermatol Clin 2001;19(1):37-48.
26. Scully C, Challacombe SJ. Pemphigus vulgaris: update on etiopathogenesis, oral manifestations, and management. Crit Rev Oral Biol Med 2002;13(4):397-408.
27. El Tal AK, Posner MR, Spigelman Z, Ahmed AR. Rituximab: a monoclonal antibody to CD20 used in the treatment of pemphigus vulgaris. J Am Acad Dermatol 2006;55(3):449-59.