Autoimmune blistering diseases: bullous Pemphigold, Pemphigus vulgaris and follaceus

• Autor(a) principal: Almeida, Rita Alexandra Silva de
• Data de Publicação: 2016
• Tipo de documento: Dissertação
• Idioma: eng
• Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
• Texto Completo: http://hdl.handle.net/10773/22358
• topic: Pênfigo; Doenças da pele; Doenças autoimunes

Bullous pemphigoid and pemphigus belong to a group of autoimmune blistering diseases that attacks the body healthy tissue, causing blisters and erosions on the skin. Bullous pemphigoid is characterized by an attack to the basal keratinocytes, making them to lose adhesion to the basement membrane zone, whereas in pemphigus happens that keratinocytes in epidermis and mucous membranes lose cell-to-cell adhesion. A single mouse click on the topic pemphigus in PubMed reveals more than 9 000 articles providing one or the other information about this group of autoimmune blistering disease. This could only demonstrate the enormous scientific and clinical interest in unraveling the mysteries that still surrounds these diseases, which began over 60 years ago with the differentiation of bullous pemphigoid from pemphigus. BP is the most common ABD affecting mainly the elderly. It has a high mortality rate, mainly due to therapy and some other complications disease-associated. Lesions are caused by autoantibodies that fix the complement and thus mediate an inflammatory process. Pemphigus has an increased incidence in women. PV has a mean age of onset between 60 and 70 years old in European countries and between the ages of 30 and 50 in the remaining countries of the world. PF has it between 30 to 45 years old. Moreover, the mortality rates of BP patients are two times higher and of pemphigus patients three times higher, than general population. Much information points out a clear genetic predisposition for disease, combined with triggering factors. Regarding the diagnosis, the physical evaluation is a milestone, where the skin, mucous membranes and the nails are examined. Also, patients are submitted to meticulous questions about symptoms where the medical history is not left out. Some quantitative and qualitative histologic examination is always performed, like ELISA. Nowadays, corticosteroids are still the main therapy; however, novel therapeutic targets have been developed. The therapeutic management comprised a series of drugs, like corticosteroids, mycophenolate mofetil, rituximab or innovative approaches associated to biotechnology. Using two associated drugs really improved patients’ prognosis. However, these medications could lead to the arising of other disorders, namely respiratory ones, as they debilitate the immune system. The contraction of such disease in a hospital environment often occurs due to one or more strains, which makes it difficult to choose a suitable therapy. Infections with bacterial origin, viruses, etc., also associate themselves to the diseases, sometimes. There have been documented cases of patients with pemphigus or BP associated with other disorders, such as neoplasia, neurologic disorders, other immune disorders. This brief review will focus on three autoimmune diseases – BP, PV and PF.