Oral pemphigus vulgaris: A case report with direct immunofluorescence study

Abstract

Pemphigus vulgaris (PV) is a chronic, autoimmune, intraepidermal blistering disease of the skin and mucous membranes. The initial clinical manifestation is frequently the development of intraoral lesions, and later, the lesions involve the other mucous membranes and skin. The etiology of this disease still remains obscure although the presence of autoantibodies is consistent with an autoimmune disease. These antibodies are targeted against the adhesion proteins of keratinocytes, leading to acantholysis (disruption of spinous layer, leading to intraepidermal clefting) and blister formation. Because only oral lesions are present initially, the chances of misdiagnosing the disease as another condition are increased, leading to inappropriate therapy. In this article, we report a case of PV with only oral manifestations in a 36-year-old male.

Oral Pemphigus Vulgaris: A case report

“…7 In 70-90% of the cases of pemphigus vulgaris oral mucosa is involved prior to the involvement of the skin. 5 The common site for oral mucosal lesions are gingiva, buccal mucosa, lips, and soft and hard palate. 8 In the present case erosive and ulcerative lesions were present on the buccal mucosa, retromolar area, lateral border of tongue, floor of mouth and soft palate.…”

“…Oral lesions have been associated with only PV and PNP. 5 Here, we report a case of pemphigus vulgaris evident on oral mucosa with minor involvement of skin.…”

Atypical Clinical Presentation of Pemphigus and Its Management: A Case Report

“…Pemphigus as a term refers to the Greek word pemphix which means vesicle or blister . PV is an autoimmune blistering disorder of skin and mucous membranes, affecting patients in their middle age of life . Pemphigus is characterized by formation of intraepidermal or intraepithelial bullae and is classified into five major groups: PV, pemphigus foliaceus, paraneoplastic pemphigus (PNP), drug‐induced pemphigus and immunoglobulin (Ig)A pemphigus.…”

“…PV is an autoimmune blistering disorder of skin and mucous membranes, affecting patients in their middle age of life . Pemphigus is characterized by formation of intraepidermal or intraepithelial bullae and is classified into five major groups: PV, pemphigus foliaceus, paraneoplastic pemphigus (PNP), drug‐induced pemphigus and immunoglobulin (Ig)A pemphigus. PV comprises about 80% of cases and shows oral lesions as an initial manifestation in 50% of cases .…”

“…Women are affected more often with an onset usually in the fourth and sixth decade . PV pathology is related to antibody formation against the desmosomal proteins desmoglein 1 and desmoglein 3 . Diagnosis is based on three main criteria: clinical findings, histopathological findings, and direct immunofluorescence (DIF) .…”