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Descriptor English: Hyperkeratosis, Epidermolytic
Descriptor Spanish: Hiperqueratosis Epidermolítica
Descriptor hiperqueratosis epidermolítica
Entry term(s) eritrodermia ictiosiforme bullosa
eritrodermia ictiosiforme bullosa congénita
Scope note: Forma de ictiosis congénita que se hereda como rasgo autosómico dominante y que se caracteriza por eritroderma e hiperqueratosis grave. Se manifiesta en el momento del nacimiento por la existencia de ampollas en todo el cuerpo, que más tarde dan lugar a costras gruesas, verrugosas y coaguladas, con predominio en las áreas de flexión.
Descriptor Portuguese: Hiperceratose Epidermolítica
Descriptor French: Hyperkératose épidermolytique
Entry term(s): Bullous Congenital Ichthyosiform Erythroderma
Bullous Erythroderma Ichthyosiforme
Bullous Erythroderma Ichthyosiformes
Bullous Erythroderma Ichthyosiformis Congenita of Brocq
Bullous Ichthyosiform Erythroderma
Bullous Ichthyosiform Erythroderma Congenital
Bullous Ichthyosiform Erythrodermas
Congenital Bullous Ichthyosiform Erythroderma
Congenital Ichthyosiform Erythroderma, Bullous
Epidermolytic Hyperkeratoses
Epidermolytic Hyperkeratosis
Epidermolytic Ichthyosis
Erythroderma Ichthyosiforme, Bullous
Erythroderma Ichthyosiformes, Bullous
Erythroderma, Bullous Ichthyosiform
Erythrodermas, Bullous Ichthyosiform
Hyperkeratoses, Epidermolytic
Ichthyosiform Erythroderma, Bullous
Ichthyosiform Erythroderma, Bullous Congenital
Ichthyosiform Erythrodermas, Bullous
Ichthyosiforme, Bullous Erythroderma
Ichthyosiformes, Bullous Erythroderma
Tree number(s): C16.131.831.512.400.375
C16.320.850.400.375
C16.614.492.400.375
C17.800.428.333.250.375
C17.800.804.512.400.375
C17.800.827.400.375
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D017488
Scope note: A form of congenital ichthyosis inherited as an autosomal dominant trait and characterized by ERYTHRODERMA and severe hyperkeratosis. It is manifested at birth by blisters followed by the appearance of thickened, horny, verruciform scales over the entire body, but accentuated in flexural areas. Mutations in the genes that encode KERATIN-1 and KERATIN-10 have been associated with this disorder.
Allowable Qualifiers: BL blood
CF cerebrospinal fluid
CI chemically induced
CL classification
CO complications
DG diagnostic imaging
DH diet therapy
DI diagnosis
DT drug therapy
EC economics
EH ethnology
EM embryology
EN enzymology
EP epidemiology
ET etiology
GE genetics
HI history
IM immunology
ME metabolism
MI microbiology
MO mortality
NU nursing
PA pathology
PC prevention & control
PP physiopathology
PS parasitology
PX psychology
RH rehabilitation
RT radiotherapy
SU surgery
TH therapy
UR urine
VE veterinary
VI virology
Previous Indexing: Ichthyosiform Erythroderma, Congenital (1991-1992)
Ichthyosis (1966-1992)
Keratosis (1972-1992)
Skin Diseases, Vesiculobullous (1972-1992)
Public MeSH Note: 1993; see ICHTHYOSIFORM ERYTHRODERMA, CONGENITAL 1991-1992; for ERYTHRODERMA ICHTHYOSIFORME, BULLOUS see ICHTHYOSIFORM ERYTHRODERMA, CONGENITAL 1991-1992, see SKIN DISEASES, BULLOUS 1979-1990
History Note: 1993(1979); use ICHTHYOSIFORM ERYTHRODERMA, CONGENITAL 1991-1992; for ERYTHRODERMA ICHTHYOSIFORME, BULLOUS use ICHTHYOSIFORM ERYTHRODERMA, CONGENITAL 1991-1992, use SKIN DISEASES, BULLOUS 1979-1990
Related: Keratin-1 MeSH
Keratin-10 MeSH
DeCS ID: 30695
Unique ID: D017488
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 1993/01/01
Date of Entry: 1992/05/20
Revision Date: 2015/06/18
Hyperkeratosis, Epidermolytic - Preferred
Concept UI M0026530
Scope note A form of congenital ichthyosis inherited as an autosomal dominant trait and characterized by ERYTHRODERMA and severe hyperkeratosis. It is manifested at birth by blisters followed by the appearance of thickened, horny, verruciform scales over the entire body, but accentuated in flexural areas. Mutations in the genes that encode KERATIN-1 and KERATIN-10 have been associated with this disorder.
Preferred term Hyperkeratosis, Epidermolytic
Entry term(s) Bullous Congenital Ichthyosiform Erythroderma
Bullous Erythroderma Ichthyosiforme
Bullous Erythroderma Ichthyosiformes
Bullous Erythroderma Ichthyosiformis Congenita of Brocq
Bullous Ichthyosiform Erythroderma
Bullous Ichthyosiform Erythroderma Congenital
Bullous Ichthyosiform Erythrodermas
Congenital Bullous Ichthyosiform Erythroderma
Congenital Ichthyosiform Erythroderma, Bullous
Epidermolytic Hyperkeratoses
Epidermolytic Hyperkeratosis
Epidermolytic Ichthyosis
Erythroderma Ichthyosiforme, Bullous
Erythroderma Ichthyosiformes, Bullous
Erythroderma, Bullous Ichthyosiform
Erythrodermas, Bullous Ichthyosiform
Hyperkeratoses, Epidermolytic
Ichthyosiform Erythroderma, Bullous
Ichthyosiform Erythroderma, Bullous Congenital
Ichthyosiform Erythrodermas, Bullous
Ichthyosiforme, Bullous Erythroderma
Ichthyosiformes, Bullous Erythroderma



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