Human Leukocyte Antigen (HLA-class II) Alleles: Correlation with Pemphigus Vulgaris Clinical Status Among Egyptian Patients

Abstract

Pemphigus refers to a group of potentially life-threatening diseases that affect the skin and mucous membrane. This group of diseases has in common the presence of desmosomal damage by autoantibodies against desmogleins, resulting in epidermal acantholysis. Pemphigus vulgaris (PV) is the most common variant of pemphigus (70% of cases) and frequently affects the oral cavity, PV may affect the skin, the oral cavity, mucosa of the nose, genitals, esophagus, pharynx, and larynx. If untreated, widespread erosion and blisters may occur with major consequences, even death. The theory that PV is inherited had been initially suggested by the unique susceptibility of some ethnic groups and populations such as Ashkenazi Jewish and people from India and the Mediterranean. However, the disease is multifactorial and there is a linkage between PV and the HLA alleles but there is also a gap in understanding how the environment and genetics may alter gene expression triggering the disease. Determining the primary associations within the HLA region and PV will not only increase our understanding of the mechanisms behind disease pathogenesis but may also aid in the development of no