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Pemphigus is an autoimmune disorder that causes blistering and raw sores on skin and mucous membranes. As with other autoimmune disorders, it is caused when the body's defenses mistake its own tissues as foreign, and attack the cells. Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. ...
A section of Human Skin In zootomy and dermatology, skin is an organ of the integumentary system made up of a layer of tissues that guard underlying muscles and organs. ...
The mucous membranes (or mucosae; singular: mucosa) are linings of ectodermic origin, covered in epithelium, and are involved in absorption and secretion. ...
Cells in culture, stained for keratin (red) and DNA (green). ...
Types There are three types of pemphigus which vary in severity: pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus. - The most common form of the disorder is pemphigus vulgaris (ICD-10 L10.0). It occurs when antibodies attack Desmoglein 3, a protein that keeps cells bound together. Thus, cells simply fall apart, causing skin to slough off. Although pemphigus vulgaris may occur at any age, it is quite rare in children, and most common in the middle aged and elderly. Sores often originate in the mouth, making eating difficult and uncomfortable. Pemphigus vulgaris often affects people between the ages of 40-60, and mainly of Jewish or Mediterranean decent. Some patients are associated with myasthenia gravis,but it's unusual.
- Foliaceus is the least severe of the three varieties. Desmoglein 1, the protein that is destroyed by the body's immune system is only found in the top dry layer of the skin, so mouth sores do not occur. Pemphigus foliaceus is characterized by crusty sores that often begin on the scalp, and may move to the chest, back, and face. It is not as painful as pemphigus vulgaris, and is often mis-diagnosed as dermatitis or eczema.
- The least common and most severe type of pemphigus is the neoplastic variety. This disorder is usually found in conjunction with an already-existing malignancy. Very painful sores appear on the mouth, lips, and the esophagus. In this variety of Pemphigus, the disease process often involves Bronchiolitis obliterans, a fatal destruction of alveoli in lung tissue. A diagnosis of neoplastic pemphigus may prompt a search for an existing tumor. Sometimes, the tumor is not malignant. In these cases, tumor removal may lead to a remission of the pemphigus. However, any decline in pulmonary function is generally irreversible.
Wikipedia does not yet have an article with this exact name. ...
Schematic of antibody binding to an antigen An antibody is a protein used by the immune system to identify and neutralize foreign objects like bacteria and viruses. ...
Sagittal section of nose mouth, pharynx, and larynx. ...
Myasthenia gravis (MG, Latin: grave muscle weakness) is a neuromuscular disease leading to fluctuating weakness and fatiguability. ...
The scalp is the skin on the head from which head hair grows. ...
Dermatitis is a term literally meaning inflammation of the skin. It is usually used to refer to eczema, which is also known as Dermatitis eczema. ...
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When normal cells are damaged beyond repair, they are eliminated by apoptosis. ...
The esophagus (also spelled oesophagus/Å“sophagus), or gullet is the muscular tube in vertebrates through which ingested food passes from the mouth area to the stomach. ...
The alveoli (singular:alveolus), tiny hollow sacs which are continuous with the airways, are the sites of gas exchange with the blood. ...
Treatment If not treated, pemphigus is fatal, due to overwhelming systemic infection. The most common treatment is the administration of oral steroids, especially prednisone. Mild cases sometimes respond to the application of topical steroids. All of these drugs may cause severe side effects, so the patient should be closely monitored by doctors. Once the outbreaks are under control, dosage is often reduced, to lessen side effects. Infection is also the title of an episode of the television series Babylon 5, and the English title of the Japanese film Kansen. ...
Prednisone is a synthetic corticosteroid drug which is usually taken orally and can be used for a large number of different conditions. ...
- If paraneoplastic pemphigus is diagnosed with pulmonary disease, a powerful cocktail of immune supressent drugs is sometimes used in an attempt to halt the rapid progression of bronchiolitis obliterans. Some drugs used include solumedrol, ciclosporin, azathioprine, and in rare instances, extremely controlled use of thalidomide in eligible patients. Immune phoresis procedures are also a possible treatment.
If skin lesions do become infected, antibiotics may be used for treatment. In addition, talcum powder is helpful to prevent oozing sores from adhering to bedsheets and clothes. Ciclosporin (INN), cyclosporine or cyclosporin (former BAN), is an immunosuppressant drug. ...
Azathioprine is a chemotherapy drug, now rarely used for chemotherapy but more for immunosuppression in organ transplantation, autoimmune disease such as rheumatoid arthritis or inflammatory bowel disease such as Crohns disease. ...
It has been suggested that Neurosedyn be merged into this article or section. ...
An antibiotic is a drug that kills or slows the growth of bacteria. ...
Talc block Talc is a mineral composed of hydrated magnesium silicate with the chemical formula H2Mg3(SiO3)4 or Mg3Si4O10(OH)2. ...
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