Search on: ICHTHYOSIFORME, BULLOUS ERYTHRODERMA 
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Descriptor English:   Hyperkeratosis, Epidermolytic 
Descriptor Spanish:   Hiperqueratosis Epidermolítica 
Descriptor Portuguese:   Hiperceratose Epidermolítica 
Synonyms English:   Bullous Congenital Ichthyosiform Erythroderma
Bullous Erythroderma Ichthyosiforme
Bullous Erythroderma Ichthyosiformis Congenita of Brocq
Bullous Ichthyosiform Erythroderma
Bullous Ichthyosiform Erythroderma Congenital
Congenital Bullous Ichthyosiform Erythroderma
Congenital Ichthyosiform Erythroderma, Bullous
Epidermolytic Hyperkeratosis
Epidermolytic Ichthyosis
Bullous Erythroderma Ichthyosiformes
Bullous Ichthyosiform Erythrodermas
Epidermolytic Hyperkeratoses
Erythroderma Ichthyosiformes, Bullous
Erythroderma, Bullous Ichthyosiform
Erythrodermas, Bullous Ichthyosiform
Hyperkeratoses, Epidermolytic
Ichthyosiform Erythroderma, Bullous
Ichthyosiform Erythrodermas, Bullous
Ichthyosiforme, Bullous Erythroderma
Ichthyosiformes, Bullous Erythroderma
Erythroderma Ichthyosiforme, Bullous
Ichthyosiform Erythroderma, Bullous Congenital  
Tree Number:   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
Definition English:   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. 
See Related English:   Keratin-1
Keratin-10
 
History Note English:   1993(1979); use ICHTHYOSIFORM ERYTHRODERMA, CONGENITAL 1991-1992; for ERYTHRODERMA ICHTHYOSIFORME, BULLOUS use ICHTHYOSIFORM ERYTHRODERMA, CONGENITAL 1991-1992, use SKIN DISEASES, BULLOUS 1979-1990 
Allowable Qualifiers English:  
BL blood CF cerebrospinal fluid
CI chemically induced CL classification
CO complications DI diagnosis
DG diagnostic imaging DH diet therapy
DT drug therapy EC economics
EM embryology EN enzymology
EP epidemiology EH ethnology
ET etiology GE genetics
HI history IM immunology
ME metabolism MI microbiology
MO mortality NU nursing
PS parasitology PA pathology
PP physiopathology PC prevention & control
PX psychology RT radiotherapy
RH rehabilitation SU surgery
TH therapy UR urine
VE veterinary VI virology
Record Number:   30695 
Unique Identifier:   D017488 

Occurrence in VHL: 		 

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