- 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 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 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* * *
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.
