Validação do índice de comprometimento cutaneomucoso do pênfigo vulgar para a avaliação clínica de pacientes com pênfigo vulgar

Abstract

Pemphigus vulgaris is a severe bullous disease, produced by the autoimmune destruction of desmosomes, resulting in the formation of intraepidermal blisters, affecting skin and mucous membranes, with a mortality of 5 to 10%. Side effects of therapy contributed to increased morbidity, accounting for considerable part of the immediate causes of death due to PV today. There is no reproducible score for clinical evaluation of patients with Pemphigus vulgaris, making the therapeutic decision subjective, and its results, uncertain. Create and evaluate the reproducibility of a scoring system for clinical evaluation of patients with pemphigus vulgaris. The Commitment Index of Skin and Mucous in Pemphigus Vulgaris was created, scoring easily observed findings on clinical examination. During 3 years, 7 patients with active PV were submitted to pairings of Commitment Index of Skin and Mucous in Pemphigus Vulgaris conduced by independent examiners for determinate its reproducibility. The Commitment Index of Skin and Mucous in Pemphigus Vulgaris proved that it is reproducible in all the statistical methods used to assess agreement between the independent examiners. In adition, it permited us to separate the patients into classes of severity. The Commitment Index of Skin and Mucous in Pemphigus Vulgaris can classify the severity of Pemphigus Vulgaris, contributing to medical research, and to the standardization of the therapy in the near future.