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Arch Dermatol Res ; 312(4): 289-294, 2020 May.
Article in English | MEDLINE | ID: mdl-31587106

ABSTRACT

Anti-desmoglein (anti-Dsg) antibodies are key players in the pathogenesis of pemphigus vulgaris (PV) disease. We aimed to evaluate the pathogenicity of anti-Dsg antibodies of PV patients using human organ culture assay and to assess the correlation between the pathogenicity and the disease score. In this cross-sectional study, sera from 37 PV patients were included. The organ culture acantholysis index (OCAI) was calculated as (width of blister/total width of the specimen) × 100. The sera were analyzed using Dsg ELISAs and ethylenediaminetetraacetic acid (EDTA) treated ELISAs. OCAI ranged from 0 to 100%, median = 50%. There was a moderate significant correlation between OCAI and the disease severity, r = 0.503, p = 0.002. There was a moderate significant correlation between OCAI and non-Ca2+-dependent anti-Dsg3 and anti-Dsg1 antibodies, p values were 0.01 and 0.021, respectively. The OCAI was assessed along the disease time course of four patients. The OCAI fluctuated parallel to the disease severity along the time course of the four patients. Human organ culture assay is an objective tool that detects the pathogenicity of anti-desmoglein antibodies. It can be used before stopping systemic steroids especially in patients in remission with high titer or if the Dsg ELISA is not available.


Subject(s)
Acantholysis/immunology , Autoantibodies/immunology , Desmoglein 1/immunology , Desmoglein 3/immunology , Pemphigus/immunology , Acantholysis/pathology , Adult , Aged , Autoantibodies/isolation & purification , Cross-Sectional Studies , Drug Administration Schedule , Female , Glucocorticoids/administration & dosage , Humans , Male , Middle Aged , Organ Culture Techniques , Pemphigus/blood , Pemphigus/diagnosis , Pemphigus/drug therapy , Severity of Illness Index , Skin/immunology , Skin/pathology
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