| ichthyosiform erythroderma | A genodermatosis characterised by diffuse chronic erythema and scale formation which may be separated into bullous and nonbullous forms. Synonym: ichthyosiform erythroderma, ichthyosis spinosa, keratoma malignum. (05 Mar 2000) |
|---|---|
| ichthyosiform erythroderma, congenital | Designation for several severe forms of ichthyosis, present at birth, that are characterised 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 generalised scaling. The forms include bullous (hyperkeratosis, epidermolytic), non-bullous (ichthyosis, lamellar), wet type, and dry type. (12 Dec 1998) |
| bullous congenital ichthyosiform erythroderma | Diffusely red, eroded skin at birth, with subsequent scaling, tending to improve in later life, characterised by generalised epidermolytic hyperkeratosis and autosomal dominant inheritance. See: epidermolytic hyperkeratosis. Synonym: generalised epidermolytic hyperkeratosis, ichthyismus hystrix, ichthyosis hystrix. (05 Mar 2000) |
|---|---|
| congenital ichthyosiform erythroderma | A genodermatosis characterised by diffuse chronic erythema and scale formation which may be separated into bullous and nonbullous forms. Synonym: ichthyosiform erythroderma, ichthyosis spinosa, keratoma malignum. (05 Mar 2000) |
| nonbullous congenital ichthyosiform erythroderma | Erythroderma or a collodion membrane at birth, usually without improvement during childhood, characterised by proliferation of epidermal keratinocytes with lipid accumulation; autosomal recessive inheritance. (05 Mar 2000) |