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Keratosis Follicularis Spinulosa Decalvans
Keratosis follicularis spinulosa decalvans is a rare X-linked disorder described by Siemens in 1926. It is a disease that begins in infancy with keratosis pilaris localized on the face, then evolves to more diffuse involvement.James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. .Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). McGraw-Hill. . An association with SAT1 has been suggested. See also * Keratosis follicularis * Hermann Werner Siemens * Cicatricial alopecia Scarring hair loss, also known as cicatricial alopecia, is the loss of hair which is accompanied with scarring. This is in contrast to non scarring hair loss. It can be caused by a diverse group of rare disorders that destroy the hair follicle, ... * List of cutaneous conditions References External links Genodermatoses {{Genodermatoses-stub ...
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Hermann Werner Siemens
Hermann Werner Siemens (August 20, 1891 (Charlottenberg) -1969)Ruggieri, Martino. "Neurocutaneous Disorders: Phakomatoses & Hamartoneoplastic Syndromes." Springer-Werlag/Vien, 2008. was a German dermatologist who first described multiple skin diseases and was one of the inventors of the twin study. Siemens' work in twin studies is influential in modern genetics and is used to address the environmental and genetic impacts upon traits.Rende, R.D., Plomin, R. & Vandenberg, S.G. Behav Genet (1990) 20: 277. https://doi.org/10.1007/BF01067795 Siemens was involved in racial hygiene and affiliated with the Nazi Party.Joseph, Jay & Wetzel, Norbert A. (2012). Ernst Rüdin: Hitler's Racial Hygiene Mastermind. Journal of the History of Biology. 46. 10.1007/s10739-012-9344-6. Early life and career Siemens studied at Berlin and Munich universities, receiving his doctorate in 1918.Prakken, J.R. "The Development of Dermatology in the Netherlands." Dermatology in the European Economic Community, Br ...
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Keratosis Pilaris
Keratosis pilaris (KP; also follicular keratosis, lichen pilaris, or colloquially chicken skin) is a common, autosomal- dominant, genetic condition of the skin's hair follicles characterized by the appearance of possibly itchy, small, gooseflesh-like bumps, with varying degrees of reddening or inflammation. It most often appears on the outer sides of the upper arms (the forearms can also be affected), thighs, face, back, and buttocks; KP can also occur on the hands, and tops of legs, sides, or any body part except glabrous (hairless) skin (like the palms or soles of feet). Often the lesions can appear on the face, which may be mistaken for acne or folliculitis. The several types of KP have been associated with pregnancy, type 1 diabetes mellitus, obesity, dry skin, allergic diseases (e.g., atopic dermatitis), and rarely cancer. Many rarer types of the disorder are part of inherited genetic syndromes. The cause of KP is not completely understood. As of 2018, KP is thought t ...
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SAT1 (gene)
Diamine acetyltransferase 1 is an enzyme that in humans is encoded by the ''SAT1'' gene found on the X chromosome. Function Spermidine/spermine N(1)-acetyltransferase (SPD/SPM acetyltransferase) is a rate-limiting enzyme in the catabolic pathway of polyamine metabolism. It catalyzes the N(1)-acetylation of spermidine and spermine and, by the successive activity of polyamine oxidase, spermine can be converted to spermidine and spermidine to putrescine. The ''SAT1'' gene is used to help regulate polyamine levels inside the cell by regulating their transport in and out of the cell. ''SAT1'' is also involved in the first step to synthesize N-acetylputrescine from putrescine. PMF1 and NRF2 work together to transcript the ''SAT1'' gene. Structure The ''SAT1'' gene is 3,069 base pairs long. There are 171 amino acids and its molecular mass is 20024 Da (daltons). In 1992 at The Johns Hopkins University School of Medicine, Lei Xiao and several others cloned over 4000 base pairs of t ...
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Keratosis Follicularis (other)
Keratosis follicularis may refer to: * Darier's disease * Focal palmoplantar keratoderma with oral mucosal hyperkeratosis See also: * Isolated dyskeratosis follicularis * Keratosis follicularis spinulosa decalvans Keratosis follicularis spinulosa decalvans is a rare X-linked disorder described by Siemens in 1926. It is a disease that begins in infancy with keratosis pilaris localized on the face, then evolves to more diffuse involvement.James, William; Ber ... {{disambig Genodermatoses Palmoplantar keratodermas ...
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Cicatricial Alopecia
Scarring hair loss, also known as cicatricial alopecia, is the loss of hair which is accompanied with scarring. This is in contrast to non scarring hair loss. It can be caused by a diverse group of rare disorders that destroy the hair follicle, replace it with scar tissue, and cause permanent hair loss. A variety of distributions are possible.Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). Page 647. McGraw-Hill. . In some cases, hair loss is gradual, without symptoms, and is unnoticed for long periods. In other cases, hair loss is associated with severe itching, burning and pain and is rapidly progressive. The inflammation that destroys the follicle is below the skin surface and there is usually no "scar" seen on the scalp. Affected areas of the scalp may show little signs of inflammation, or have redness, scaling, increased or decreased pigmentation, pustules, or draining sinuses. Scarring hair loss occurs in otherwise healthy people of al ...
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List Of Cutaneous Conditions
Many skin conditions affect the human integumentary system—the organ system covering the entire surface of the body and composed of skin, hair, nails, and related muscle and glands. The major function of this system is as a barrier against the external environment. The skin weighs an average of four kilograms, covers an area of two square metres, and is made of three distinct layers: the epidermis, dermis, and subcutaneous tissue. The two main types of human skin are: glabrous skin, the hairless skin on the palms and soles (also referred to as the "palmoplantar" surfaces), and hair-bearing skin.Burns, Tony; ''et al''. (2006) ''Rook's Textbook of Dermatology CD-ROM''. Wiley-Blackwell. . Within the latter type, the hairs occur in structures called pilosebaceous units, each with hair follicle, sebaceous gland, and associated arrector pili muscle. In the embryo, the epidermis, hair, and glands form from the ectoderm, which is chemically influenced by the underlying mesoderm th ...
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