Junctional Epidermolysis Bullosa (medicine)
Junctional epidermolysis bullosa is a skin condition characterized by blister formation within the lamina lucida of the basement membrane zone. Signs and symptoms People with the condition experience very fragile skin, with blisters and skin erosion occurring in response to relatively benign trauma. Blisters may form all over the body, including the mucous membranes. Chronic scarring can lead to the formation of granulation tissue, which may bleed easily, predisposing to infection. Hands and fingers may be affected, as well as various joints. Pathophysiology α6β4 integrin is a transmembrane protein found in hemidesmosomes. As a heterodimer molecule containing two polypeptide chains its extracellular domain enters the basal lamina and interacts with type IV collagen suprastructure containing laminins (laminin-5), entactin/nidogen or the perlecan on the extracellular surface of the hemidesmosome, laminin-5 molecules form threadlike anchoring filaments that extend from the int ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
|
Lamina Lucida
The lamina lucida is a component of the basement membrane which is found between the epithelium and underlying connective tissue (e.g., epidermis and dermis of the skin). It is a roughly 40 nanometre wide electron-lucent zone between the plasma membrane of the basal cells and the (electron-dense) lamina densa of the basement membrane.James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology'' (10th ed.). Saunders. Page 5. . Similarly, electron-lucent and electron-dense zones can be seen between enamel of teeth and the junctional epithelium. The electron-lucent zone is adjacent to the cells of the junctional epithelium and might be considered a continuation of the lamina lucida as both are seen to harbour hemidesmosomes. However, unlike the lamina densa, the electron-dense zone adjacent to enamel show no signs of hemidesmosomes. Some theorize that the lamina lucida is an artifact created when preparing the tissue, and that the la ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
|
Autosomal Recessive
In genetics, dominance is the phenomenon of one variant (allele) of a gene on a chromosome masking or overriding the Phenotype, effect of a different variant of the same gene on Homologous chromosome, the other copy of the chromosome. The first variant is termed dominant and the second is called recessive. This state of having Heterozygosity, two different variants of the same gene on each chromosome is originally caused by a mutation in one of the genes, either new (''de novo'') or Heredity, inherited. The terms autosomal dominant or autosomal recessive are used to describe gene variants on non-sex chromosomes (autosomes) and their associated traits, while those on sex chromosomes (allosomes) are termed X-linked dominant, X-linked recessive or Y-linked; these have an inheritance and presentation pattern that depends on the sex of both the parent and the child (see Sex linkage). Since there is only one Y chromosome, Y-linked traits cannot be dominant or recessive. Additionally, ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
|
Genodermatoses
Genodermatosis is a hereditary Skin condition, skin disease with three inherited modes including single gene inheritance, multiple gene inheritance and chromosome inheritance. There are many different types of genodermatosis; the prevalence of genodermatosis ranges from 1 per 6000 people to 1 per 500,000 people.Fields, D. (2019, June). Types of Genodermatoses. Retrieved September 08, 2020, from https://www.news-medical.net/health/Types-of-Genodermatoses.aspx Genodermatosis has influence on the texture, color and structure of skin cuticle and connective tissue, specific lesion site and clinical manifestations on the body vary depending on the type. In the spite of the variety and complexity of genodermatosis, there are still some common methods that can help people diagnose. After diagnosis, different types of genodermatosis require different levels of therapy including interventions, nursing interventions and treatments.Fondation René Touraine. (n.d.). Genodermatoses & Rare Skin D ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
|
ILDS
The International League of Dermatological Societies (ILDS) is a non-governmental organization that works closely with the World Health Organization. It was founded in 1935, but because of World War II no congresses were held until 1952. It is governed by the International Committee of Dermatology. The ILDS is the parent organization of the International Foundation for Dermatology, founded in 1987. After the publication of ICD-10, the ILDS produced a series of compatible extensions for use in dermatology Dermatology is the branch of medicine dealing with the Human skin, skin.''Random House Webster's Unabridged Dictionary.'' Random House, Inc. 2001. Page 537. . It is a speciality with both medical and surgical aspects. A List of dermatologists, .... References External links Official site HistoryInternational Foundation for DermatologyApplication to Dermatology of International Classification of Disease (ICD-11) Organizations established in 1935 Dermatology organi ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
|
Skin Lesion
A skin condition, also known as cutaneous condition, is any medical condition that affects the integumentary system—the organ system that encloses the body and includes skin, nails, and related muscle and glands. The major function of this system is as a barrier against the external environment. Conditions of the human integumentary system constitute a broad spectrum of diseases, also known as dermatoses, as well as many nonpathologic states (like, in certain circumstances, melanonychia and racquet nails). While only a small number of skin diseases account for most visits to the physician, thousands of skin conditions have been described. Classification of these conditions often presents many nosological challenges, since underlying causes and pathogenetics are often not known. Therefore, most current textbooks present a classification based on location (for example, conditions of the mucous membrane), morphology ( chronic blistering conditions), cause ( skin conditions ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
|
Junctional Epidermolysis Bullosa (veterinary Medicine)
Junctional epidermolysis bullosa (JEB) is an inherited disorder that is also known as ''red foot disease'' or ''hairless foal syndrome''. JEB is the result of a genetic mutation that inhibits protein production that is essential for skin adhesion. Therefore, tissues, such as skin and mouth epithelia, are affected. Blisters form over the entire body causing pain and discomfort, and open sores leave newborn foals highly susceptible to secondary infection. The condition can be categorized into two types of mutations: JEB1 and JEB2. JEB1 is found in Belgian Draft horses, as well as other related Draft breeds. In contrast, JEB2 is found in American Saddlebred horses. Breeds affected JEB has been documented in Belgian drafts, American Cream Draft, Breton drafts, Comtois, and American Saddlebreds. Of these horses, 12% of Belgians and 4% of Saddlebreds are thought to carry the disorder. Humans JEB also affects the human population. Symptoms are closely related to those that are se ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
|
Epidermis
The epidermis is the outermost of the three layers that comprise the skin, the inner layers being the dermis and Subcutaneous tissue, hypodermis. The epidermal layer provides a barrier to infection from environmental pathogens and regulates the amount of water released from the body into the atmosphere through transepidermal water loss. The epidermis is composed of stratified squamous epithelium, multiple layers of flattened cells that overlie a base layer (stratum basale) composed of Epithelium#Cell types, columnar cells arranged perpendicularly. The layers of cells develop from stem cells in the basal layer. The thickness of the epidermis varies from 31.2μm for the penis to 596.6μm for the Sole (foot), sole of the foot with most being roughly 90μm. Thickness does not vary between the sexes but becomes thinner with age. The human epidermis is an example of epithelium, particularly a stratified squamous epithelium. The word epidermis is derived through Latin , itself and . ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
|
Germany
Germany, officially the Federal Republic of Germany, is a country in Central Europe. It lies between the Baltic Sea and the North Sea to the north and the Alps to the south. Its sixteen States of Germany, constituent states have a total population of over 84 million in an area of , making it the most populous member state of the European Union. It borders Denmark to the north, Poland and the Czech Republic to the east, Austria and Switzerland to the south, and France, Luxembourg, Belgium, and the Netherlands to the west. The Capital of Germany, nation's capital and List of cities in Germany by population, most populous city is Berlin and its main financial centre is Frankfurt; the largest urban area is the Ruhr. Settlement in the territory of modern Germany began in the Lower Paleolithic, with various tribes inhabiting it from the Neolithic onward, chiefly the Celts. Various Germanic peoples, Germanic tribes have inhabited the northern parts of modern Germany since classical ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
|
Syria
Syria, officially the Syrian Arab Republic, is a country in West Asia located in the Eastern Mediterranean and the Levant. It borders the Mediterranean Sea to the west, Turkey to Syria–Turkey border, the north, Iraq to Iraq–Syria border, the east and southeast, Jordan to Jordan–Syria border, the south, and Israel and Lebanon to Lebanon–Syria border, the southwest. It is a republic under Syrian transitional government, a transitional government and comprises Governorates of Syria, 14 governorates. Damascus is the capital and largest city. With a population of 25 million across an area of , it is the List of countries and dependencies by population, 57th-most populous and List of countries and dependencies by area, 87th-largest country. The name "Syria" historically referred to a Syria (region), wider region. The modern state encompasses the sites of several ancient kingdoms and empires, including the Eblan civilization. Damascus was the seat of the Umayyad Caliphate and ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
|
University Of Modena
The University of Modena and Reggio Emilia (), located in Modena and Reggio Emilia, Emilia-Romagna, Italy, is one of the oldest universities in Europe, founded in 1175, with a population of 20,000 students. The medieval university disappeared by 1338 and was replaced by "three public lectureships" which did not award degrees and were suspended in the 1590s "for lack of money". The university was not reestablished in Modena until the 1680s and did not receive an imperial charter until 1685.Quoted from: Grenler, Paul F. The Universities of the Italian Renaissance Johns Hopkins University Press, 2004. Page 137. Some famous students who attended the university include Ludovico Antonio Muratori, a noted Italian historian and scholar who graduated in 1694, the playwright Carlo Goldoni in the 17th century and, in the last century, Sandro Pertini, who became President of the Italian Republic. Brief History The University of Modena dates back to 1175, a few decades after the birth ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
|
COL17A1
Collagen XVII, previously called BP180, is a transmembrane protein which plays a critical role in maintaining the linkage between the intracellular and the extracellular structural elements involved in epidermal adhesion, identified by Diaz and colleagues in 1990. COL17A1 is the official name of the gene. It encodes the alpha chain of type XVII collagen. Collagen XVII is a transmembrane protein, like collagen XIII, XXIII and XXV. Collagen XVII is a structural component of hemidesmosomes, multiprotein complexes at the dermal-epidermal basement membrane zone that mediate adhesion of keratinocytes to the underlying membrane. It also appears to be a key protein in maintaining the integrity of the corneal epithelium. Mutations in this gene are associated with both generalized atrophic benign and junctional epidermolysis bullosa, as well as recurrent corneal erosions, and expression of this gene is abnormal in various cancers. Two homotrimeric forms of type XVII collagen exist. The ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
|
Basement Membrane
The basement membrane, also known as base membrane, is a thin, pliable sheet-like type of extracellular matrix that provides cell and tissue support and acts as a platform for complex signalling. The basement membrane sits between epithelial tissues including mesothelium and endothelium, and the underlying connective tissue. Structure As seen with the electron microscope, the basement membrane is composed of two layers, the basal lamina and the reticular lamina. The underlying connective tissue attaches to the basal lamina with collagen VII anchoring fibrils and fibrillin microfibrils. The basal lamina layer can further be subdivided into two layers based on their visual appearance in electron microscopy. The lighter-colored layer closer to the epithelium is called the lamina lucida, while the denser-colored layer closer to the connective tissue is called the lamina densa. The electron-dense lamina densa layer is about 30–70 nanometers thick and consists of an und ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |