Rodríguez AM, Ramos GA, Sánchez FD

Language: Spanish
References: 20
Page: 25-30
PDF size: 158.30 Kb.

ABSTRACT

Familial benign pemphigus (Hailey-Hailey disease) is a chronic disease transmitted through an autosomic pattern with variable penetrance. It is clinically characterized by the presence of erythematous excoriated and macerated blisters, especially in intertriginous areas. Histologically, it presents suprabasal acantholysis. Around two thirds of the patients present a family background. The background of cursing the disease with multiple remissions and exacerbations is very characteristic. The pathogenesis is still unknown, even though it is believed that the ATP2 C1 gene has some kind of participation. This mentioned gene codifies a dependant calcium pump. There are limited therapeutic options. Basically, they are palliative.

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