Related MeSH Hierarchy (6)
Diseases [C] » Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16] » Congenital Abnormalities » Skin Abnormalities » Ichthyosis » Ichthyosiform Erythroderma, Congenital » Hyperkeratosis, Epidermolytic
Diseases [C] » Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16] » Genetic Diseases, Inborn » Skin Diseases, Genetic » Ichthyosiform Erythroderma, Congenital » Hyperkeratosis, Epidermolytic
Diseases [C] » Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16] » Infant, Newborn, Diseases » Ichthyosis » Ichthyosiform Erythroderma, Congenital » Hyperkeratosis, Epidermolytic
Diseases [C] » Skin and Connective Tissue Diseases [C17] » Skin Diseases » Keratosis » Ichthyosis » Ichthyosiform Erythroderma, Congenital » Hyperkeratosis, Epidermolytic
Diseases [C] » Skin and Connective Tissue Diseases [C17] » Skin Diseases » Skin Abnormalities » Ichthyosis » Ichthyosiform Erythroderma, Congenital » Hyperkeratosis, Epidermolytic
Diseases [C] » Skin and Connective Tissue Diseases [C17] » Skin Diseases » Skin Diseases, Genetic » Ichthyosiform Erythroderma, Congenital » Hyperkeratosis, Epidermolytic
Description
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. MeSH
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