Pemphigus antibodies are routinely detected by direct or indirect immunofluorescence (IIF) testing using monkey esophagus as a substrate to establish the diagnosis, and for monitoring disease activity that frequently follows antibody titers. 5, 6 In animal studies 7 and in vitro 8, 9 experiments, anti-Dsg3 antibodies have been shown to induce loss of cell adhesion, indicating an important role in disease pathognesis. 1 - 4 Desmoglein 3, a 130-kd transmembrane glycoprotein of the cadherin family, mediates weak calcium-dependent homophilic cell-to-cell adhesion of epidermal keratinocytes. 1 Immunologically, antibodies directed against the keratinocyte cell surface are detected in the skin and in the circulation they recognize the extracellular domain of Dsg3. Histologically, PV is characterized by suprabasal acantholysis and intraepidermal blister formation, and clinically by flaccid blisters and erosions that usually arise on normal-appearing skin and/or mucosa. PEMPHIGUS VULGARIS (PV) is an autoimmune-blistering disease of the skin and mucous membranes. In addition, when multiple serum samples from 1 patient with PV sampled over a 2-year period were tested, ELISA reactivity paralleled both the IIF titers and the clinical course.Ĭonclusion The Dsg3-ELISA is a sensitive, objective, and PV-specific test that should be considered as an adjunct test for the management of patients with PV. There was a positive correlation ( r = 0.654) between ELISA values and IIF titers within the whole population with PV. Interestingly, 1 patient with paraneoplastic pemphigus had positive ELISA results. In addition, negative ELISA results were obtained from 11 of 11 serum samples from patients with bullous pemphigoid, 10 of 12 serum samples from patients with other bullous skin disorders, 7 of 9 serum samples from patients with autoimmune-connective tissue diseases, and 13 of 13 serum samples from patients with other nonbullous skin diseases. Results Forty-seven (98%) of 48 serum samples from patients with PV that were positive by IIF on monkey esophagus were also reactive by Dsg3-ELISA, whereas 4 of 4 IIF-negative PV serum samples showed no reactivity by ELISA. Patients Fifty-two serum samples from 11 patients with PV, and serum samples from 11 patients with bullous pemphigoid, 12 patients with other bullous diseases, 22 patients with various nonbullous skin disorders, and 10 healthy individuals were tested. Setting Ambulatory and hospitalized patients from a university hospital. To compare ELISA values with autoantibody titers obtained by classic indirect immunofluorescence (IIF).ĭesign Serum samples from patients with PV and various other bullous and nonbullous skin diseases were tested for anti-Dsg3 reactivity by ELISA. Objectives To evaluate serum samples from patients with PV and other dermatologic diseases for anti-Dsg3 antibodies. Cloning of the Dsg3gene and expression of the protein in a native conformation enabled the recent development of a specific and sensitive enzyme-linked immunosorbent assay (ELISA) for the detection of PV autoantibodies. Shared Decision Making and Communicationīackground Pemphigus vulgaris (PV) is an autoimmune-blistering disease of the skin and mucous membranes caused by autoantibodies against desmoglein 3 (Dsg3), an epidermal desmosomal adhesion protein of the cadherin family.Scientific Discovery and the Future of Medicine.Health Care Economics, Insurance, Payment.Clinical Implications of Basic Neuroscience.Challenges in Clinical Electrocardiography.
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