Pemphigus

Abstract

Pemphigus refers to a group of potentially life-threatening autoimmune diseases of the skin and mucous membranes, characterized by the formation of blisters and erosions of the skin. An autoimmune process, directed against keratinocyte desmosomal cadherins, interferes with the adhesive function of these molecules. This results in the separation of keratinocytes and clinical manifestation of blistering. Differences in the particular antigens targeted by the antibodies and in the distribution of these antigens in the different regions of the body and in the separate layers of the epidermis result in different clinical manifestations of the disease. Diagnosis of pemphigus is based on three independent groups of criteria: clinical features (flaccid blisters and erosions on skin and oral mucosa), histologic findings (epidermal acantholysis) and immunological tests (circulating and skin-fixed antibodies against keratinocyte surface antigens). The principle aim of treatment is to reduce inflammatory response and autoantibody production, thereby achieving disease remission.Systemic corticosteroids are still the most useful drugs in the treatment of pemphigus and continue to be the mainstay of therapy for this disease.Adjuvant drugs, such as immunosuppressants, are commonly used in combination, in order to increase efficacy and have a steroid-sparing effect,thereby allowing reduced maintenance doses and less side effects of systemic corticosteroids. Other options include intravenous immunoglobulin and plasmapheresis. However, more research is needed to develop treatments with the least possible toxicity.