[Pemphigus].
Pemphigus is an infrequent, organ-specific, autoimmune bullous disease, which affects the skin, mucous membranes and appendages. Histopathologically, it is characterized by acantholysis. Pemphigus has classically been divided into two major groups, pemphigus vulgaris and pemphigus foliaceus, with their respective clinical variants pemphigus vegetans and pemphigus erythematosus. In recent years, new variants of pemphigus have been described: paraneoplastic pemphigus, IgA pemphigus and pemphigus herpetiformis. This article reviews the epidemiology, etiopathogenesis, clinical symptoms, diagnosis, treatment and prognosis of pemphigus. Advances in molecular biology techniques have made it possible to more precisely identify the different antigens against which antibodies are directed, and to fine-tune ELISA diagnostic techniques. Treating pemphigus vulgaris and foliaceus with general steroids has modified their prognosis; it is estimated that mortality in recent decades is less than 10 %. Managing the clinical complications that appear during the evolution of the pemphigus has contributed to reducing morbidity and mortality.
Servicio de Dermatología, Hospital Universitario de La Princesa, Madrid, Spain. jsanchezperez@aedv.es
Javier
J
Sánchez-Pérez
Amaro
A
García-Díez
D006801:Humans; D010257:Paraneoplastic Syndromes / Q000209:etiology / Q000473:pathology; D010392:Pemphigus / Q000150:complications / Q000209:etiology / Q000473:pathology / Q000628:therapy