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Author: Dr Amy Stanway, Dermatology Registrar, Nottingham, United Kingdom, 2005.
Diffuse hereditary palmoplantar keratodermas are the palmoplantar keratodermas that affect most of the palms and soles and are caused by a genetic abnormality. Several family members may be affected. Some types of keratoderma are associated with abnormalities of internal organs.
They may be inherited from one affected parent (known as autosomal dominant inheritance) or from both parents, who are generally unaffected (known as autosomal recessive inheritance). The affected gene has been specificaly identified for many of these keratodermas.
Diffuse hereditary palmoplantar keratodermas present in early childhood with redness of the palms and soles. The palms and soles gradually become thicker and develop a yellowish, waxy appearance. There is a clear cut-off between affected and unaffected skin and the edge of the thickening is often red. This is usually obvious by the age of 3 to 4.
‘Transgradient’ is a term used to describe the extent of the skin thickening:
The non-transgradient diffuse inherited keratodermas are known as Vorner disease and Unna-Thost disease. They are inherited as autosomal dominant conditions. There are no associated abnormalities. They look the same but have slightly different findings on skin biopsy.
The following treatments soften the thickened skin and make them less noticeable.
These conditions persist for life and may be passed on to the next generation.
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