Management of a rare chronic blistering autoimmune skin disease: Pemphigus Vulgaris

Pemphigus vulgaris is an autoimmune, intraepithelial, blistering disease affecting the skin and mucous membrane. The immune system produces anti bodies against specific proteins in the skin and mucous membrane. These anti bodies break the bonds between skin cells. This leads to the formation of a blister. The exact cause is unknown. It is mediated by circulating auto antibodies directed against keratinocyte cell surface. The disease arises most often in middle aged or older people, usually starting with a blister that ruptures easily. The lesions can become quite extensive. As potentially life threatening disease, it has a mortality rate of approximately 5-15%. There are 5 main variant of pemphigus, pemphigus vulgaris, pemphigus foliaceous, pemphigus erythematous, drug induced pemphigus and Para neoplastic pemphigus. Corticosteroids and other immunosuppressive drugs are used to reduce the symptoms of pemphigus. An established alternative to steroids are monoclonal antibody such as rituximab, which are increasingly being used as first line treatment.