Pemphigus is a rare acquired autoimmune bullous disease due to the production of auto-antibodies directed against desmoglein 1 and 3 that are adhesion molecules of desmosomes of the skin and mucosae respectively. Binding of auto-antibodies on desmogleins lead to disruption of desmosome and intra- epithelial separation. This is responsible for the formation of blisters and large erosive areas on the skin and mucous membranes; oral, genital, ocular, anal, oesophageal and sometimes ENT mucosae can be involved. Erosions of the skin and mucous membrane are painful and lead to dysphagia, weight loss and infection. Two clinical and histological main subtypes of pemphigus have been described: pemphigus vulgaris which is characterized by mucosal and skin involvement, and pemphigus foliaceus, characterized by exlcusive skin involvement. Treatment of pemphigus is based on corticosteroids, conventional immunosuppressive drugs and more recently biologics targeting B lymphocytes, IVIG and immunoadsorption of pathogenic antibodies.
Prevalence : The prevalence is not very well known. It can be estimated in Europe at between 60 000 and 80 000 patients