Diffuse epidermolytic palmoplantar keratoderma (Q85670): Difference between revisions

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Latest revision as of 11:02, 15 August 2026

Diffuse epidermolytic palmoplantar keratoderma of Vörner-Thost (MIM #144200) is an autosomal dominant disorder which is clinically indistinguishable from diffuse non-epidermolytic palmoplantar keratoderma (Unna) but has different histological appearance (epidermolytic hyperkeratosis) and aetiology (keratin 9 mutations). Diffuse keratoderma develops in infancy, leading to confluent keratoderma, sparing dorsal surfaces, with a sharp demarcation and erythematous edge by adult life.

Language	Label	Description	Also known as
default for all languages	ID_1505856808
English	Diffuse epidermolytic palmoplantar keratoderma	Diffuse epidermolytic palmoplantar keratoderma of Vörner-Thost (MIM #144200) is an autosomal dominant disorder which is clinically indistinguishable from diffuse non-epidermolytic palmoplantar keratoderma (Unna) but has different histological appearance (epidermolytic hyperkeratosis) and aetiology (keratin 9 mutations). Diffuse keratoderma develops in infancy, leading to confluent keratoderma, sparing dorsal surfaces, with a sharp demarcation and erythematous edge by adult life.