Pemphigus

Year 2011, , 468 - 473, 01.12.2011

Derya Uçmak

https://doi.org/10.5799/ahinjs.01.2011.04.0099

Abstract

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. J Clin Exp Invest 2011; 2 (4): 468-473

Keywords

Bullous diseases, pemphigus, corticosteroids, treatment

Pemfigus

Abstract

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.

Keywords

Büllöz hastalıklar, pemfigus, kortikosteroidler, tedavi

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