Journal of IMAB (Apr 2019)
DENTAL PERSPECTIVE OF PEMPHIGUS VULGARIS – A CLINICAL CASE WITH DIAGNOSTIC AND TREATMENT GUIDELINES
Abstract
Pemphigus vulgaris (PV), a rare autoimmune mucocutaneous blistering disorder, has been reported with an incidence of 0.1-0.5 cases per 100,000 individuals worldwide per year. It is slightly more common in women and occurs primarily in adults during the 5th or 6th decade of life. The etiology of PV is uncertain but is supposed to be mediated by circulating immunoglobulin G autoantibodies against the desmosomal cadherins, desmoglein 1 and 3. Biopsy, light microscopic examination, and additional adjuvant tests, such as immunofluorescence studies can be used to establish the diagnosis. In most cases (70-90%), the first signs of disease appear on the oral mucosa, but most patients with oral lesions are initially misdiagnosed and treated improperly for months or years. If these patients are misdiagnosed or left untreated, PV may be fatal with a mortality rate ranging from 60% to 90%. Therefore, here we discuss the basics of diagnosing and treating patients with PV and oral lesions.
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