Canadian Medical Guide > Diseases > Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Abnormalities > Skin Abnormalities > Ichthyosis > Ichthyosiform Erythroderma, Congenital Terms and Definitions

Ichthyosiform Erythroderma, Congenital

Medical Definition:	Designation for several severe forms of ichthyosis, present at birth, that are characterized by hyperkeratotic scaling. Infants may be born encased in a collodion membrane which begins shedding within 24 hours. This is followed in about two weeks by persistent generalized scaling. The forms include bullous (HYPERKERATOSIS, EPIDERMOLYTIC), non-bullous (ICHTHYOSIS, LAMELLAR), wet type, and dry type.
Guide Notes:	an ichthyosis rather than an erythroderma; do not use /congen & do not coord with INFANT, NEWBORN, DISEASES
Also Called:	Congenital Ichthyosiform Erythroderma, Dry Type,Congenital Ichthyosiform Erythroderma, Wet Type
Previously Indexed:	Ichthyosis (1966-1990)

Ichthyosiform Erythroderma, Congenital Categories.

	Hyperkeratosis, Epidermolytic - 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.
	Ichthyosis, Lamellar - A chronic, congenital ichthyosis inherited as an autosomal recessive trait. Infants are usually born encased in a collodion membrane which sheds within a few weeks. Scaling is generalized and marked with grayish-brown quadrilateral scales, adherent at their centers and free at the edges. In some cases, scales are so thick that they resemble armored plate.

Ichthyosiform Erythroderma, Congenital Medical Definitions and Terms

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