RESUMO
Recent rapid advances in the basic research into pemphigus have provided amny insights into its pathophysiology. In particular, a recenthy developed enzymelinked immunosorbent assay (ELISA) for desmogleins 1 and 3 (Dsg1 and Dsg3), antigens for phemphigus foliaceus (PF) and pemphigus vulgaris (PV), respectively, has led to great progress in the diagnosis and classification of pemphigus, as well as in understanding its pathomechanisms. Studies with the anti-Dsg1 and Dsg3 antibodies heve indicated that there are two types of PV, the mucosal dominant type and the mucocutaneous type. The same ELISA has identified the antigens in pemphigus herpetiformis. The autoantigens detected by this ELISA correlate well with the clinical features in pemphigus patients in showing the shift between PV and PF. In addition, the Dsg compensation theory proposed by Stanley and Amagai can reasonably explain the different depths of skin lesions and the different occurrences of skin and oral mucosal lesions between PV and PF. Furthermore, a complicated profile of autoantigens in paraneoplastic pemphigus (PNP) has been indicated in various biochemical studies, and IgG anti-Dsg1 and antiDsg3 antibodies have been detected in serum from all the PNP patients by the above ELISA. On the other hand, serum from subcorneal pustular dermatosis type IgA pemphigus patients have been shown to react with Dsc1, another type of desmosomal cadherin, by a novel cDNA transfection method. In addition, IgA antibodies. Various autoimmune bullous diseases, including several types of phemphigus, are the only diseases in which the pathogenic role of circulationg antoantibodies has been confirmed using the newborn mouse animal model. Therefore, studies of the pathophysiology of pemphigus are extremely important as a paradigm for research into various types of autoimmune diseases in other fields
Assuntos
Humanos , Pênfigo/complicações , Pênfigo/diagnóstico , Pênfigo/fisiopatologia , Pênfigo/microbiologiaRESUMO
Pemphigus is a group of organ-specific autoimmune disorders with an established immunologic basis. The presence of intraepithelial blisters and erosions of the skin and variable involvement of the mucous menbranes characterize its three major variants, pemphigus vulgaris, pemphigus follaceus, and paraneoplastic pemphigus. Prior to the use of corticosteroids in the 1950s, the natural history of pemphigus vulgaris was relentless progression, with a 50% mortality at 2 years, and almost 100% at 5 years. Today, with mortality rates less than 5% the focus has changed towards reducing corticosteroid side effects and maintaining optional quality of life under treatment. this can be achived by the appropriate use of steroid-spating agents. This article addresses the comprehensive management of patients with pemphigus
Assuntos
Humanos , Ciclofosfamida/uso terapêutico , Pênfigo/microbiologia , Pênfigo/patologia , Pênfigo/terapiaRESUMO
Background - Pemphigus foliaceus is an autoimmune skin disease mediated by autoantibodies against desmoglein 1. The endemic form is thought to ahve an environmental cause. The Terena reservation of Limao Verde in Mato Grosso do Sul, Brazil, is a recently identifies focus of the disease, with a prevalence of 3.4 percent in the population. We tested the hypothesis taht normal subjects living in an endemic area have antibodies agaisnt desmoglein 1. Methods - We used an enzyme-linked immunosorbent assay to defect antibodies agaisnt desmoglein 1 in serum samples from 60 patients with endemic pemphigus foliaceus (fogo selvagem) who lived in Limao Verde or elsewhere in Brazil, 372 normal subjects (without pemphigus foliaceus) from Limao Verde and surrounding location, and 126 normal subjects from the United States and Japan. Results - Antibodies against desmoglein 1 were detected in 59 of the 60 patients with fogo selvagem (98 percent) but in only 3 of the 126 normal subjects from the United States and Japan (2 percent). Antibodies were also detected in 51 of the 93 normal subjects from Limao Verde (55 percent) and in 54 of the 279 normal subjects from surrounding areas (19 percent). Serum samples obtained one to four years before the onset of disease were available for five patients; all five had antibodies in the initial serum serumsamples, and the onset of disease was associated with a marked increase in antibody values. Conclusions - The prevalence of antibodies against desmoglein 1 is high among normal subjects living in an area where fogo selvagem is endemic, and the onset of the disease is preceded by a sustained antibody response. These findings support the concept that the production of antibodies against desmoglein 1 is initiated by exporure to an unknown environmental agent