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Facts about the skin from DermNet New Zealand Trust. Topic index: A B C D E F G H I J K L M N O P Q R S T U V W X Y Z


Diffuse hereditary palmoplantar keratodermas

What are diffuse hereditary palmoplantar keratodermas?

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.

Clinical features

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.

Increased sweating (hyperhidrosis) is quite common and there is a tendency to fungal and bacterial infections of the feet.

Diffuse palmoplantar keratoderma Diffuse palmoplantar keratoderma Diffuse palmoplantar keratoderma
Diffuse hereditary palmoplantar keratoderma

‘Transgradient’ is a term used to describe the extent of the skin thickening:

Non-transgradient diffuse palmoplantar keratodermas

The non-transgradient diffuse inherited keratodermas are known as Vorner's 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.

Diffuse palmoplantar keratoderma Diffuse palmoplantar keratoderma Diffuse palmoplantar keratoderma
Unna-Thost palmoplantar keratoderma

Transgradient diffuse palmoplantar keratodermas

Olmsted syndrome
  • Most likely to have autosomal recessive inheritance
  • Associated with scaly rash around the mouth and nose
  • Lines of skin thickening occur the inner aspects of the forearms
  • Fingers may be lost due to tight bands of skin around the fingers
Mal-de Meleda
  • Autosomal recessive inheritance
  • Associated with a mild rash around the mouth and nose
  • Atopic dermatitis
  • Marked increase in sweating
Huriez syndrome
  • Autosomal dominant inheritance
  • Tightening of the skin over the fingers
  • Squamous cell carcinoma may arise in affected skin

Diffuse palmoplantar keratoderma associated with other abnormalities

Deafness

Hair, nail and teeth abnormalities

Treatment of diffuse palmoplantar keratoderma

The following treatments soften the thickened skin and make them less noticeable.

What is the prognosis?

These conditions persist for life and may be passed on to the next generation.

Related information

References:

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Author: Dr Amy Stanway, dermatology registrar, Nottingham

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