Catalog

Diffuse epidermolytic palmoplantar keratoderma

No MMS code

Link to WHO Definition

Diffuse epidermolytic palmoplantar keratoderma – http://id.who.int/icd/entity/1505856808

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.