epidermolysis

A condition in which the epidermis is loosely attached to the corium, readily exfoliating or forming blisters. [epidermis + G. lysis, loosening]
- e. bullosa [MIM*131800] a group of inherited chronic noninflammatory skin diseases in which large bullae and erosions result from slight mechanical trauma; a form localized to the hands and feet is called Weber-Cockayne syndrome, of autosomal dominant inheritance caused by mutation in either the gene encoding keratin-5 (KRT5) on chromosome 12q or the gene for keratin-14 (KRT14) on 17q. SYN: mechanobullous disease.
- e. bullosa, dermal type SYN: e. bullosa dystrophica.
- e. bullosa dystrophica [MIM*131705] a form of e. bullosa in which scarring develops after separation of the entire epidermis with blistering; it is inherited as an autosomal dominant (appearing in infancy or childhood) or recessive (present at birth or appearing in early infancy) trait, the latter including lethal and nonlethal types; both dominant and recessive forms are caused by mutation in the gene for type VII collagen (COL7A1) on chromosome 3p. SYN: dermolytic bullous dermatosis, e. bullosa, dermal type.
- e. bullosa, epidermal type (bu′lo-sa) SYN: e. bullosa simplex.
- e. bullosa, junctional type SYN: e. bullosa lethalis.
- e. bullosa lethalis [MIM*226700] a form of e. bullosa characterized by persistent and nonhealing perioral and perinasal crusted lesions with bullae often present in the oral mucosa and trachea, but not on the palms and soles, complicated by dermal sepsis and serum protein and electrolyte loss leading to death; autosomal recessive inheritance, caused by mutation in any one of the three distinct polypeptides of laminin-5; alpha-3 (LAMA3) on chromosome 18q, beta-3 (LAMB3) and gamma-2 (LAMC2) on 1q or the gene encoding integrin, beta-4 (ITGB4) on 17q. SYN: e. bullosa, junctional type, Herlitz syndrome.
- e. bullosa simplex [MIM*131900] e. bullosa in which lesions heal rapidly without scarring; bulla formation is intraepidermal and microscopy reveals basal cell vacuolation and dissolution of tonofibrils; occurs most frequently on the feet of adults after unaccustomed trauma such as long marches; autosomal dominant inheritance caused by mutation in the keratin-5 gene (KRT5) on chromosome 12q or in the keratin-14 gene (KRT14) on 17q. SYN: e. bullosa, epidermal type.

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ep·i·der·mol·y·sis .ep-ə-(.)dər-'mäl-ə-səs n, pl -y·ses -.sēz a state of detachment or loosening of the epidermis

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epi·der·mol·y·sis (ep″ĭ-dər-molґə-sis) [epidermis + -lysis] a loosened state of the epidermis, with formation of blebs and bullae, either spontaneously or following trauma. epidermolytic adj

Medical dictionary. 2011.

Look at other dictionaries:

  • epidermolysis — ep·i·der·mol·y·sis …   English syllables

  • epidermolysis — …   Useful english dictionary

  • Epidermolysis bullosa — (EB) is a rare genetic disease characterized by the presence of extremely fragile skin and recurrent blister formation, resulting from minor mechanical friction or trauma. The condition was brought to public attention in the UK through the… …   Wikipedia

  • Epidermolysis bullosa — (EB) ist eine genetisch bedingte Hautkrankheit, die je nach Subtyp autosomal dominant oder rezessiv vererbt wird. Ursache ist ein angeborene Mutation in bestimmten Genen, deren Genprodukte (Proteine) u. a. für den intakten zellulären Aufbau der… …   Deutsch Wikipedia

  • epidermolysis bullosa — epidermolysis bul·lo·sa bə lō sə n any of a group of inherited disorders of variable severity marked esp. by the formation of large fluid filled blisters which develop chiefly in response to minor mechanical trauma see dystrophic epidermolysis… …   Medical dictionary

  • Epidermolysis bullosa herpetiformis — Classification and external resources OMIM 131760 DiseasesDB 33248 Epidermolysis bullosa herpetiformis (also known as Dowling Meara epide …   Wikipedia

  • Epidermolysis bullosa acquisita — Classification and external resources ICD 10 L12.3 ICD 9 694.8 …   Wikipedia

  • epidermolysis bullosa acquisita — epidermolysis bullosa ac·qui·si·ta .ak wə sīt ə n an autoimmune skin disorder similar to epidermolysis bullosa that occurs in adults and is usu. associated with another disorder (as Crohn s disease or diabetes) * * * acquired epidermolysis… …   Medical dictionary

  • epidermolysis bullosa simplex — epidermolysis bullosa sim·plex sim .pleks n any of several forms of epidermolysis bullosa that are marked by blister formation within the epidermis sometimes accompanied by thickening of the skin but usu. no scarring and that are chiefly… …   Medical dictionary

  • epidermolysis bullosa — epidermolysis bullosa. См. буллезный эпидермоз. (Источник: «Англо русский толковый словарь генетических терминов». Арефьев В.А., Лисовенко Л.А., Москва: Изд во ВНИРО, 1995 г.) …   Молекулярная биология и генетика. Толковый словарь.

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