Pemphigus

Pemphigus is a rare autoimmune diseases which affects the skin and mucous membranes. It is chronic and occurs in male and female patients between 40 and 60 years of age. The antigens targeted by the auto-antibodies are components of intercellular adhesion, namely desmosomes. Specifically, autoantibodies are formed against desmoglein 3, which is responsible for the formation of oral lesions and against desmoglein 1, which is responsible for skins lesions. Clinically it presents as one or more blisters with a serous or haematic content, situated on the non-keratinised or keratinised oral mucosa. On occasions the appearance of the mucosa appears normal or slightly erythematous but, if subject to slight mechanical stimulation, an epithelial dissociation can occur with the formation of blisters (Nicolsky’s sign), whose outermost layer can sometimes be lost. The lesions are very painful and associated with alteration of stomatological functions: phonotary, deglutary and mastication). A frequent oral clinical variant of pemphigus, involving the gingival tissue, is desquamative gingivitis, which is often erroneously diagnosed as gingivitis, although in the absence of plaques. A biopsy is essential in order to diagnose pemphigus (which renders the intra-epithelial blisters visible) and direct immunofluorescence (as evidence of IgG and C3 deposits in the intercellular spaces with a typical lattice morphology). Research into the specific autoantibodies in the serum is also very useful (indirect immunofluorescence). Pemphigus is a chronic condition, tending to involve various parts of the body (e.g. the genitalia, oesophagus and skin) in addition to the oral cavity. It can have very serious consequences on the life of the patient who, when in an acute stage, must be treated by a multi-specialist team offering suitable systemic treatment (intravenously or per os) with an immunosuppressant action.