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List Of Target Antigens In Pemphigus
Circulating auto-antibodies in the human body can target normal parts of the skin leading to disease. This is a list of antigens in the skin that may become targets of circulating auto-antibodies leading to the various types of pemphigus. Footnotes See also * List of target antigens in pemphigoid * List of conditions caused by problems with junctional proteins * List of immunofluorescence findings for autoimmune bullous conditions * List of cutaneous conditions * List of genes mutated in cutaneous conditions A number of gene mutations have been linked to conditions of or affecting the human integumentary system. See also * List of radiographic findings associated with cutaneous conditions * List of cutaneous conditions caused by mutations in ke ... * List of histologic stains that aid in diagnosis of cutaneous conditions References * * {{DEFAULTSORT:Target antigens in pemphigus Cutaneous conditions Dermatology-related lists ...
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Pemphigus
Pemphigus ( or ) is a rare group of blistering autoimmune diseases that affect the skin and mucous membranes. The name is derived from the Greek root ''pemphix'', meaning "pustule". In pemphigus, autoantibodies form against desmoglein, which forms the "glue" that attaches adjacent epidermal cells via attachment points called desmosomes. When autoantibodies attack desmogleins, the cells become separated from each other and the epidermis becomes detached, a phenomenon called acantholysis. This causes blisters that slough off and turn into sores. In some cases, these blisters can cover a large area of the skin. Originally, the cause of this disease was unknown, and "pemphigus" was used to refer to any blistering disease of the skin and mucosa. In 1964, researchers found that the blood of patients with pemphigus contained antibodies to the layers of skin that separate to form the blisters. In 1971, an article investigating the autoimmune nature of this disease was published ...
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Stevens–Johnson Syndrome
Stevens–Johnson syndrome (SJS) is a type of severe skin reaction. Together with toxic epidermal necrolysis (TEN) and Stevens–Johnson/toxic epidermal necrolysis (SJS/TEN), it forms a spectrum of disease, with SJS being less severe. Erythema multiforme (EM) is generally considered a separate condition. Early symptoms of SJS include fever and flu-like symptoms. A few days later, the skin begins to blister and peel, forming painful raw areas. Mucous membranes, such as the mouth, are also typically involved. Complications include dehydration, sepsis, pneumonia and multiple organ failure. The most common cause is certain medications such as lamotrigine, carbamazepine, allopurinol, sulfonamide antibiotics and nevirapine. Other causes can include infections such as '' Mycoplasma pneumoniae'' and cytomegalovirus, or the cause may remain unknown. Risk factors include HIV/AIDS and systemic lupus erythematosus. The diagnosis of Stevens–Johnson syndrome is based on involvemen ...
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Pemphigus Vegetans Of Hallopeau
Pemphigus vegetans is a localized form of pemphigus vulgaris. in which there is a localized vegetating papillomatous response. The eroded areas do not heal like usual but form papillomatous growth and vegetation. Accounts for 1-2% of pemphigus cases and is a relatively benign variant of pemphigus vulgaris. Two forms are recognized: * ''Pemphigus vegetans of Neumann'' is a localized disease of pemphigus vulgaris slightly more extensive than pemphigus vegetans of Hallopeau. This type is more common and characterized by early lesions similar to Pemphigus Vulgaris with large bullae and erosive areas. Healing is through formation of granulation tissue. It is named for the Austrian Dermatologist, Isidor Neumann. * ''Pemphigus vegetans of Hallopeau'' is a disease of localized pemphigus vulgaris. It is named for François Henri Hallopeau. This type is less aggressive and has pustules not bullae. These pustules heal by verrucous hyperkeratotic vegetations. See also * List of cutan ...
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Pemphigus Vegetans
Pemphigus vegetans is a localized form of pemphigus vulgaris. in which there is a localized vegetating papillomatous response. The eroded areas do not heal like usual but form papillomatous growth and vegetation. Accounts for 1-2% of pemphigus cases and is a relatively benign variant of pemphigus vulgaris. Two forms are recognized: * ''Pemphigus vegetans of Neumann'' is a localized disease of pemphigus vulgaris slightly more extensive than pemphigus vegetans of Hallopeau. This type is more common and characterized by early lesions similar to Pemphigus Vulgaris with large bullae and erosive areas. Healing is through formation of granulation tissue. It is named for the Austrian Dermatologist, Isidor Neumann. * ''Pemphigus vegetans of Hallopeau'' is a disease of localized pemphigus vulgaris. It is named for François Henri Hallopeau. This type is less aggressive and has pustules not bullae. These pustules heal by verrucous hyperkeratotic vegetations. See also * List of cutane ...
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Fogo Selvagem
Pemphigus foliaceus is an autoimmune blistering disease ( bullous disorder) of the skin. Pemphigus foliaceus causes a characteristic inflammatory attack at the subcorneal layer of epidermis, which results in skin lesions that are scaly or crusted erosions with an erythematous (red) base.Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). Page 558–562. McGraw-Hill. . Mucosal involvement is absent even with widespread disease. If there is an autoimmune IgG buildup in the epidermis, then nearly all of the antibodies are aimed against desmoglein 1. The effect of the antibodies and the immunological pathway is most likely one of three mechanisms: * Steric hindrance of the desmoglein 1: The antibody caps off the site for intracellular binding to another keratinocyte. * Activation of an endocytic pathway: The antibody activates a pathway which causes an internalization of desmogleïn 1, which in turn causes a loss of adhesion. * Disruption of fun ...
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Bracket
A bracket is either of two tall fore- or back-facing punctuation marks commonly used to isolate a segment of text or data from its surroundings. Typically deployed in symmetric pairs, an individual bracket may be identified as a 'left' or 'right' bracket or, alternatively, an "opening bracket" or "closing bracket", respectively, depending on the directionality of the context. Specific forms of the mark include parentheses (also called "rounded brackets"), square brackets, curly brackets (also called 'braces'), and angle brackets (also called 'chevrons'), as well as various less common pairs of symbols. As well as signifying the overall class of punctuation, the word "bracket" is commonly used to refer to a specific form of bracket, which varies from region to region. In most English-speaking countries, an unqualified word "bracket" refers to the parenthesis (round bracket); in the United States, the square bracket. Various forms of brackets are used in mathematics, with s ...
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Pemphigus Erythematosus
Pemphigus erythematosus is simply a localized form of pemphigus foliaceus with features of lupus erythematosus.Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). Page 562. McGraw-Hill. . See also * Pemphigus * List of cutaneous conditions * List of conditions caused by problems with junctional proteins Mutations of proteins that hold the cells of the skin together can cause disease. Autoantibodies against proteins that hold the cells of the skin together can also cause disease. See also * List of keratins expressed in the human integume ... References External links Chronic blistering cutaneous conditions Syndromes {{Cutaneous-condition-stub ...
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Intraepidermal Neutrophilic Type Of IgA Pemphigus
IgA pemphigus is a subtype of pemphigus with two distinct forms: * ''Subcorneal pustular dermatosis'' (also known as Sneddon–Wilkinson disease and pustulosis subcornealis) is skin condition that is a rare, chronic, recurrent, pustular eruption characterized histopathologically by subcorneal pustules that contain abundant neutrophils.James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. . This is distinct from and not to be confused with subcorneal pustular dermatosis type of IgA pemphigus. Sneddon's syndrome, also known as Ehrmann-Sneddon syndrome, is also a different syndrome. * ''Intraepidermal neutrophilic IgA dermatosis'' is characterized histologically by intraepidermal bullae with neutrophils, some eosinophils, and acantholysis. History Early descriptions were made by Darrell Wilkinson, a British dermatologist. See also * Pemphigus * List of cutaneous conditions * List of conditions ...
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Desmocollin 1
Desmocollins are a subfamily of desmosomal cadherins, the transmembrane constituents of desmosomes. They are co-expressed with desmogleins to link adjacent cells by extracellular adhesion. There are seven desmosomal cadherins in humans, three desmocollins and four desmogleins. Desmosomal cadherins allow desmosomes to contribute to the integrity of tissue structure in multicellular living organisms. Structure Three isoforms of desmocollin proteins have been identified. * Desmocollin-1, coded by the DSC1 gene * Desmocollin-2, coded by the DSC2 gene * Desmocollin-3, coded by the DSC3 gene Each desmocollin gene encodes a pair of proteins: a longer 'a' form and a shorter 'b' form. The 'a' and 'b' forms differ in the length of their C-terminus tails. The protein pair is generated by alternative splicing. Desmocollin has four cadherin-like extracellular domains, an extracellular anchor domain, and an intracellular anchor domain. Additionally, the 'a' form has an intracellu ...
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IgA1
Immunoglobulin A (Ig A, also referred to as sIgA in its secretory form) is an antibody that plays a role in the immune function of mucous membranes. The amount of IgA produced in association with mucosal membranes is greater than all other types of antibody combined. In absolute terms, between three and five grams are secreted into the intestinal lumen each day. This represents up to 15% of total immunoglobulins produced throughout the body. IgA has two subclasses (IgA1 and IgA2) and can be produced as a monomeric as well as a dimeric form. The IgA dimeric form is the most prevalent and is also called ''secretory IgA'' (sIgA). sIgA is the main immunoglobulin found in mucous secretions, including tears, saliva, sweat, colostrum and secretions from the genitourinary tract, gastrointestinal tract, prostate and respiratory epithelium. It is also found in small amounts in blood. The secretory component of sIgA protects the immunoglobulin from being degraded by proteolytic enz ...
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Subcorneal Pustular Dermatosis Type Of IgA Pemphigus
IgA pemphigus is a subtype of pemphigus with two distinct forms: * ''Subcorneal pustular dermatosis'' (also known as Sneddon–Wilkinson disease and pustulosis subcornealis) is skin condition that is a rare, chronic, recurrent, pustular eruption characterized histopathologically by subcorneal pustules that contain abundant neutrophils.James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. . This is distinct from and not to be confused with subcorneal pustular dermatosis type of IgA pemphigus. Sneddon's syndrome, also known as Ehrmann-Sneddon syndrome, is also a different syndrome. * ''Intraepidermal neutrophilic IgA dermatosis'' is characterized histologically by intraepidermal bullae with neutrophils, some eosinophils, and acantholysis. History Early descriptions were made by Darrell Wilkinson, a British dermatologist. See also * Pemphigus * List of cutaneous conditions * List of conditions ca ...
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Drug-induced Pemphigus
Pemphigus ( or ) is a rare group of blistering autoimmune diseases that affect the skin and mucous membranes. The name is derived from the Greek root ''pemphix'', meaning "pustule". In pemphigus, autoantibodies form against desmoglein, which forms the "glue" that attaches adjacent epidermal cells via attachment points called desmosomes. When autoantibodies attack desmogleins, the cells become separated from each other and the epidermis becomes detached, a phenomenon called acantholysis. This causes blisters that slough off and turn into sores. In some cases, these blisters can cover a large area of the skin. Originally, the cause of this disease was unknown, and "pemphigus" was used to refer to any blistering disease of the skin and mucosa. In 1964, researchers found that the blood of patients with pemphigus contained antibodies to the layers of skin that separate to form the blisters. In 1971, an article investigating the autoimmune nature of this disease was publishe ...
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