Pemphigus Vulgaris: A Review On Etiopathogenesis, Clinical Features and Challenges In The Management

Pemphigus vulgaris is a chronic autoimmune intraepithelial disease that forms flaccid blisters and erosions of skin and mucous membranes. Pemphigus vulgaris is a rare disease resulting from an autoimmune process in which IgG serum antibodies are produced against normal desmosomal adhesion molecules (desmoglein 3 and to lesser extent desmoglein 1) on the cell membrane of keratinocytes causing loss of cell adhesion, with separation of epithelial layers (acantholysis) and also appearance of blisters on skin or mucosa. Patients have ill-defined, irregularly shaped, gingival, buccal, or palatine erosions, which are painful and slow to heal. Pemphigus vulgaris shows have an approximately equal prevalence among men and women. PV Causes genetics like HLA DR4 and HLA DR14. The mucous membranes often affected are those of oral cavity other surfaces involved including conjunctiva, oesophagus labia, vagina, cervix, vulva, penis, urethra, nasal mucosa, and anus. The diagnosis is based on set of criteria: clinical features, histology and immunological tests. Laboratory examinations include: Tzanck smear to detect acantolytic cells, direct and indirect immunofluorescence, ELISA test and when diagnosis remain uncertain, immunoprecipitation and immunoblotting techniques are helpful. The main objective of therapeutic management is to control the disease, heal the bullous skin and mucous lesions and to minimize the associated functional impairment. The standard treatment for pemphigus vulgaris is use of systemic corticosteroids. Azathioprine and mycophenolate mofetil are used in case of nonsteroidal treatment. In case of recalcitrant pemphigus, Rituximab is extremely effective when other treatments fail to control the disease. The present review emphasized the etiopathogenesis, clinical characteristics, diagnosis and treatment strategies for Pemphigus vulgaris and challenges in the management of this uncommon disease. Key words: Pemphigus vulgaris (PV), Acantholysis, keratinocytes, Oral lesions, Nikolsky sign, prednisolone, immunosuppressive agent,