Pemphigus: An autoimmune disorder of keratinocyte adhesion molecules

Pemphigus is a group of potentially life-threatening autoimmune diseases characterized by cutaneous and/or mucosal blistering. It affects the skin and oral mucosa and may also affect the mucosae of the nose, conjunctivae, genitals, oesophagus, pharynx and larynx and is found mainly in middle aged and elderly patients. There is damage to desmosomes by antibodies directed against the extracellular domains of the cadherin-type epithelial cell adhesion molecules – the desmogleins (Dsg) with immune deposits intraepithelially, and loss of cell–cell contact (acantholysis), leading to intra-epithelial vesiculation. Six types of pemphigus have been established: vulgaris, vegetans, erythematosus, foliaceus, paraneoplastic pemphigus, and pemphigus IgA out of which Pemphigus vulgaris (PV), is the most common variant. As it is a life threatening disease condition it is important that the dentist is able to recognize oral manifestations of PV and treat or refer appropriately. Current treatment is largely based on systemic immunosuppression using corticosteroids, with azathioprine or other adjuvants or alternatives but newer therapies with potentially fewer adverse effects, also appear promising.