[Familial palmo-plantar keratoderma with epidermolytic hyperkeratosis (author's transl)]

Three cases of palmo-plantar keratoderma coming from three different families are reported; Clinical features were those of Thost-Unna's disease; histo-pathological and ultrastructural aspects resembled those of the epidermolytic hyperkeratosis. The literature, 13 cases within 7 families with the same clinical and histopathological characteristics (though without study of the ultrastructure), have been reported. The authors propose to term "familial palmo-plantar keratoderma with epidermolytic hyperkeratosis", those cases of palmo-plantar keratoderma with dominant autosomal transmission and the histopathological aspects of the epidermolytic hyperkeratosis (Frost and Van Scott). The relationship of this disease with the naevus unius lateris and the bullous ichtyosiform hyperkeratosis are discussed. All three diseases could represent various phenotypical aspects of the same genetic abnormality of the keratinization process.