PEMPHIGUS VULGARIS: AN UPDATE ON ETIOPATHOGENESIS, CLINICAL MANIFESTATIONS, DIAGNOSIS AND TREATMENT MODALITIES

Abstract

Pemphigus Vulgaris is an autoimmune, mucocutaneous disorder characterized by occurrence of multiple chronic ulcerations. Although exact etiology is still obscure, the underlying pathogenesis in Pemphigus vulgaris involves autoimmune attack on the epithelial cell adhesion molecules, namely desmosomes and hemidesmosomes. Oral lesions generally appear before the onset of skin lesions, and in 60-70% cases, oral lesions may be the only presenting symptoms. The initial oral lesions manifest as thin walled flaccid bullae, which eventually rupture, leading to formation of large erosive lesions. Early and accurate diagnosis is extremely essential and entails a meticulous history taking, thorough oral and systemic examination along with characteristic histopathology and immunofluorescence features. Although corticosteroids are still the cornerstone of pharmacotherapy, a number of emerging therapies have also evolved with good results.