Pemphigus: skin failure mediated by autoantibodies
Article Abstract:
Pemphigus, which comes from the Greek word for ''blister'', actually represents a wide variety of bullous diseases (bullae are large blister-like vesicles). When the term is used by itself, however, it often means pemphigus vulgaris. The other major type of pemphigus is pemphigus foliaceus. Although both disorders may be referred to by the inadequately precise term pemphigus, they are distinct clinical entities and have distinct histopathological characteristics. Pemphigus foliaceus usually appears as scaly, well-demarcated skin lesions. Although this disease may be severe and cause great pain, it is generally not fatal. This is not true for pemphigus vulgaris, however. The first signs of pemphigus vulgaris are usually erosive lesions in the mucous membranes of the mouth. If left untreated, most patients with pemphigus vulgaris die, just as severe burn patients die, because the protective function of the skin is lost. The difference in mortality between the two pemphigus diseases is probably due to the fact that pemphigus foliaceus attacks more superficial layers of the epidermis. These diseases are autoimmune in origin; antibodies in patients with both diseases bind to cells in the skin. In fact, staining cells with the immunofluorescent antibody technique is similar for both conditions. In both diseases, antibodies seem to attack the desmosome, which is the structure responsible for attaching cells of the epidermis to their neighbors. This may well account for the histopathological observation that the cells in the affected skin seem to have fallen apart from one another. Molecular analysis reveals that antibodies from patients with pemphigus foliaceus react with a glycoprotein called desmoglein, the desmosome ''glue'', which has a molecular weight of 160,000. Antibodies from pemphigus vulgaris, however, react with a protein of 130,000 molecular weight. While this protein is clearly associated with other proteins of the desmosome, its role is not known. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: JAMA, The Journal of the American Medical Association
Subject: Health
ISSN: 0098-7484
Year: 1990
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Pemphigus--Diseases of Antidesmosomal Autoimmunity
Article Abstract:
Drug therapy has improved the prognosis of patients with pemphigus. Pemphigus is a skin disease characterized by widespread blistering. The blisters are caused by antibodies against desmogleins (Dsgs), which are glycoproteins present in the junctions between skin cells. The cause of this autoimmune disease is not known, but the autoantibodies against Dsgs cause the skin layers to separate from each other. Corticosteroids such as prednisone are the main treatment and immunosuppressive drugs are also used.
Publication Name: JAMA, The Journal of the American Medical Association
Subject: Health
ISSN: 0098-7484
Year: 1999
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Early infant feeding and risk of developing type 1 diabetes-associated autoantibodies
Article Abstract:
Giving a baby cereals containing gluten before the age of six months may increase the baby's risk of developing type 1, or juvenile-onset, diabetes, according to a study of 1,610 babies. Four of the 17 children who were fed gluten cereals before the age of three months developed a precursor of type 1 diabetes. All four had a specific gene called DRB1*03/04, DQB1*0302.
Publication Name: JAMA, The Journal of the American Medical Association
Subject: Health
ISSN: 0098-7484
Year: 2003
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