Scleroatrophic syndrome of Huriez
Encyclopedia
Scleroatrophic syndrome of Huriez (also known as "Huriez syndrome," "Palmoplantar keratoderma with scleroatrophy," "Palmoplantar keratoderma with sclerodactyly," "Scleroatrophic and keratotic dermatosis of the limbs," and "Sclerotylosis") is an autosomal dominant keratoderma
with sclerodactyly
present at birth with a diffuse symmetric keratoderma of the palms and soles.
An association with 4q23 has been described.
It was characterized in 1968.
Keratoderma
-Congenital:* Simple keratodermas** Diffuse palmoplantar keratodermas*** Diffuse epidermolytic palmoplantar keratoderma*** Diffuse nonepidermolytic palmoplantar keratoderma*** mal de Meleda** Focal palmoplantar keratoderma*** Striate palmoplantar keratoderma...
with sclerodactyly
Sclerodactyly
Sclerodactyly is a localized thickening and tightness of the skin of the fingers or toes. Sclerodactyly is commonly accompanied by atrophy of the underlying soft tissues....
present at birth with a diffuse symmetric keratoderma of the palms and soles.
An association with 4q23 has been described.
It was characterized in 1968.