PEMPHIGUS VULGARIS: ORAL MANIFESTATIONS OF AN AUTOIMMUNE DISORDER

Abstract

Pemphigus vulgaris (PV) comprises 70% of cases of pemphigus, a group of rare, life-threatening autoimmune disorders. The disease leads to the formation of intraepithelial blisters in the skin and mucous membranes that often first appear in the mouth. The pathogenesis of PV is driven by anti-desmoglein 1 (anti-Dsg-1) and anti-desmoglein 3 (anti- Dsg-3) auto-antibodies produced by B lymphocytes, which react to epithelial transmembrane cell-junction proteins desmoglein-1 and desmoglein-3, disrupting the intercellular bonds. We report a case of a 54-year-old man who had been treated for an unspecific rheumatic disease for 5 years, without proper results. Clinically, he presented with multiple oral ulcerations and a few blisters in the chest. Nikolsky’s sign was positive. The biopsy of oral lesions with subsequent histopathologic analysis revealed an epithelium with acantholysis and acantholytic cells, confirming the diagnosis of PV. The patient is currently being treated with corticosteroids and immunosuppressants, and the disease is controlled.