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Pemphigus

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Endemic pemphigus foliaceus (PF), also known as fogo selvagem, is a skin autoimmune blistering disease due to acantholysis, the loss of adhesion among keratinocytes. PF is characterized by autoantibodies against desmoglein-1 (DSG1). These autoantibodies are found in patients’ sera as well as bound to their epidermal keratinocytes. The mechanism that allows sensitization to self antigens and result in the generation of pathogenic autoantibodies in pemphigus are not understood, although both environmental and genetic factors are likely to be involved [1-4]. Brazilian endemic form of PF shares similar clinical, histological and immunological features with the non-endemic form of PF seen in the USA and around the world. Pemphigus is endemic not…show more content…
Therefore, it is hypothesized that immune response to antigens released in the saliva of the insects ultimately results in a pathogenic cross-reactive response to autoantigens in genetically prone individuals [12]. To date, major affected Brazilian regions include the mid-west and the southeastern states. However, new sites have been characterized in the northern parts of Brazil [7]. PF patients are outdoor workers, usually farmers or family members of a farmer. The daily activities of a typical family in these settings include agriculture, care of livestock and home chores (i.e. cooking, caring for small animals, or washing laundry in nearby rivers or streams) [9]. Young adults and children are typically affected. Gender or racial predisposition has not been…show more content…
Although originally thought to be responsible for drug metabolism almost exclusively in the liver, it has now been realized that all xenobiotic-metabolizing enzymes participate in many crucial endogenous functions, probably in every eukaryotic cell. The cytochrome P450 (CYP) enzymes are among the most important xenobiotic-metabolizing enzymes and are products of the CYP superfamily of genes [23]. High CYP bioactivity inter-subject variability occurs, and it has been largely attributed to gene polymorphism some of which affect enzyme activity and/or their transcription level
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