Autoimmune bullous diseases are rare disorders affecting skin and mucous membranes which are mediated by pathogenic autoantibodies against target antigens whose function is adhesion within the epidermis or adhesion of epidermis to dermis. The pathogenesis of these disorders has been extensively investigated with advanced techniques in recent years. This review focuses on the etiopathogenesis of main autoimmune bullous disorders including pemphigus, bullous pemphigoid, anti-p200 pemphigoid, cicatricial pemphigoid, pemphigoid gestationis, dermatitis herpetiformis, linear IgA bullous dermatosis and
epidermolysis bullosa acquisita.
Keywords: Autoimmune disease, skin diseases, vesiculobullous, pemphigus, pemphigoid, bullous, benign mucous membrane, laminin gamma 1, pemphigoid gestationis, dermatitis herpetiformis, immunoglobulin A, dermatosis, epidermolysis bullosa acquisita
Otoimmün büllöz hastal?klar, epidermis içinde veya epidermisin dermise ba?lant?s?nda i?lev gören hedef antijenlere kar?? geli?mi? patojenik antikorlarla karakterize deri ve mukozalar? tutan nadir görülen tablolard?r. Son y?llarda bu hastal?klar?n patogenezi ileri tetkikler kullan?larak yap?lan çal??malarla yo?un olarak ara?t?r?lm??t?r. Bu derlemede temel otoimmün büllöz hastal?klardan pemfigus, büllöz pemfigoid, anti-p200 pemfigoid, sikatrisyel pemfigoid, pemfigoid gestasyones, dermatitis herpetiformis, lineer IgA hastal??? ve edinsel epidermolizis büllozan?n etyopatogenezi üzerinde durulacakt?r.
Anahtar Kelimeler: Otoimmün büllöz hastal?k, pemfigus, büllöz pemfigoid, anti-p200 pemfigoid, sikatrisyel pemfigoid, pemfigoid gestasyones, dermatitis herpetiformis, lineer IgA hastal???, edinsel epidermolizis bülloza