Clinical Notes : Dermatology

184. Palmoplantar Keratoderma

What is palmoplantar keratoderma ?

‘Keratoderma’ is a term that means marked thickening of the skin.

 

‘Palmoplantar’ refers to the skin on the soles of the feet and palms of the hands; these are the areas keratoderma affects most often. Palmoplantar keratoderma is also sometimes known as ‘keratosis palmaris et plantaris’.

Classification of keratodermas depends on whether or not it is inherited, and its clinical features.

  • Diffuse keratodermas affect most of the palms and soles.

  • Focal keratodermas mainly affect pressure areas.

  • Punctate-type keratodermas result in tiny bumps on the palms and soles.

  • Most often the abnormal skin involves only the palms and soles (non-transgradient) but sometimes it extends on to the top of the hands and feet as well (transgradient).

 

In some rare forms of keratoderma other organs in the body may be affected in addition to the skin, and the keratoderma can be a marker of this internal abnormality.

 

Acquired keratoderma

Psoriasis

Chronic eczema

Psoriasis

Chronic eczema

Psoriasis

Focal keratoderma

Hereditary keratoderma

Punctate

Focal

Diffuse transgradient

Diffuse non-transgradient

Punctate

Focal

Diffuse transgradient

Diffuse non-transgradient

Punctate

Focal

Diffuse transgradient

Diffuse non-transgradient

 

What causes palmoplantar keratoderma ?

Keratoderma may be inherited (hereditary) or, more commonly, acquired.

 

  • Hereditary keratoderma: the condition runs in families and is passed down or from one or both parent(s) to their children

  • Acquired keratoderma: the condition is not inherited and occurs as a result of a change in the health or the environment of the affected person

 

The hereditary keratodermas are caused by a gene abnormality that results in abnormal skin protein (keratin).

They may be inherited either by an autosomal dominant or autosomal recessive pattern.

  • Autosomal dominant keratodermas are likely to occur in every generation of a family. If one parent is affected there is a 50% chance that each child will be affected.

  • Autosomal recessive keratodermas occur less commonly within an affected family. This is because both parents need to pass on an abnormal gene to the child for it to be affected. People with one affected gene only do not have the condition themselves but carry the abnormal gene and are referred to as ‘carriers’ of the disease. They may pass on the abnormal gene to their children but the children will only be affected if their other parent also carries an abnormal gene and passes it on to the same child.

 

Specific types of keratoderma 

  • Hereditary palmoplantar keratoderm​a

 

 

  • Punctate inherited palmoplantar keratodermas

    • result in tiny bumps of thickened skin on the palms and soles and usually appears in late childhood or early adulthood

  • Focal hereditary palmoplantar keratodermas

    • involve only some areas of the palms or soles, usually over pressure points.

    • some types are associated with abnormalities in organs other than the skin.

​​

  • 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.

        • Non-transgradient 

          • do not extend beyond the palms on the hands and the sole on the feet

          • may affect the knuckle pads and nails but do not involve the thin skin on the top of the feet or hands.

        • Transgradient

          • extend beyond the palms and soles onto the back of the hands and feet and in some cases up the wrists and ankles.

          • skin around the mouth, eyes, nose, and over the elbows and knees may also be affected

 

  • Acquired palmoplantar keratodermas

  • more likely to present in adulthood (compared with inherited keratodermas which usually present in childhood).

  • presents with thickening of the skin of the palms and/or soles which may be diffuse (involving most of the palms and soles) or focal (localised mainly to pressure areas).

  • may arise in association with a variety of different skin and internal conditions.

    • Inflammatory skin conditions

      • Psoriasis

      • Dermatitis (eczema)

      • Lupus erythematosus

      • Lichen planus

      • Pityriasis rubra pilaris

    • Infections

      • Reiter syndrome

      • Dermatophyte fungal infection (tinea)

      • Syphilis

      • Crusted scabies

      • Extensive viral warts (usually in immunosuppressed patients)

    • Circulatory problems

      • Lymphoedema

    • Secondary to inherited conditions that may not usually result in keratoderma

      • Ichthyosis

      • Ectodermal dysplasia

      • Epidermolysis bullosa

      • Erythrokeratoderma

    • Medications and toxins

      • Iodine

      • Lithium

      • Tegafur

      • Glucan

      • Halogenated weed-killers

      • Arsenic

      • Dioxin

      • Chemotherapeutic agents used in cancer treatment

      • Targeted therapy for BRAF+ melanoma with vemurafenib or dabrafenib

    • Internal illness

      • Myxoedema (thyroid disease)

      • Internal malignancy (cancer) has been associated with the development of acquired keratoderma

    • Miscellaneous

      • Keratoderma climacterum is a keratoderma that usually develops in middle-aged woman. It has been suggested that this is related to the menopause.

 

 

Treatment of palmoplantar keratoderma 

The following treatments soften the thickened skin and makes it less noticeable.

 
 
 

Keratolytic agent (6% salicylic acid, 70% propylene glycol, 30% water) is not available commercially in Ireland. Ask the pharmacist to mix it to order.

The topical retinoid (isotretinoin) is avalable in Ireland as Roaccutane.

The topical retinoid (adapalene) is avalable in Ireland as Differin.

Topical vitamin D ointment (calcipotriol) is available in Irelanfd as Dovonex.

The systemic retinoid (acitretin) is available in Ireland as Neotigason.

 

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