Thesis (M.Sc.) - Cairo University - Faculty of Medicine - Department of Dermatology and Venerology

Background: Pemphigus vulgaris (PV) is a potentially fatal autoimmune blistering skin disease characterized by intraepithelial blister formation involving skin and mucous membranes where autoantibodies play a critical role in the pathogenesis of PV. The autoantibodies are directed against keratinocyte self adhesion antigens; Dsg1 (desmoglein1) and Dsg3 (desmoglein3). Thus, they inhibit the intercellular adhesion between keratiocytes resulting in suprabasalacantholysis pathologically that manifests clinically by the pemphigus vulgaris phenotype. Aim of work: To correlate the clinical disease severity and the pathogenicity of the autoantibodies of pemphigus vulgaris patients. Patients and method: Thirty egyptian PV patients recruited from Kasr Al- Aini outpatient clinic were examined for assessment of severity of the disease using pemphigus disease area index (PDAI) score. Blood samples were obtained from all the subjects, sera were extracted. One ml of sera was injected into prepared 4mm normal skin specimens that were incubated at 37o C for 48 hours, processed,stained byhematoxylin and eosin, and examined under light microscopy for assessment of the pathogenicity by organ culture acantholysis index (OCAI). The remaining sera were used for detection of anti-Dsg3 and anti-Dsg1 antibodies titres using ELISA. We also used EDTA treated ELISA to detect autoantibodies against non calcium dependent epitopes

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