Palmoplantar keratoderma


Palmoplantar keratoderma

Description, Causes and Risk Factors:

Abbreviation: PPK.

Palmoplantar keratodermas are a group of disorders characterized by thickening of the skin on the palms of the hands and soles of the feet of affected individuals. The various forms of PPK can be divided into hereditary forms with only skin problems, hereditary syndromes with PPK as an associated feature, and acquired forms. The more commonly seen hereditary PPKs are discussed here.

Hereditary forms may be localized to the hands and feet, or they may be associated with a more generalized skin disorder. Classification of hereditary PPK is frequently confusing. A simple classification incorporates 3 factors: 1) specific form and structure and distribution of the palmoplantar keratosis, 2) presence of associated skin and other tissue (such as nails, hair, mucous membranes) disease in sites other than the palms and the soles, and 3) presence or absence of fragile or blistering skin.

The palmoplantar keratodermas are caused by abnormal proteins in the superficial layer of skin (epidermis). We have several genes responsible for producing these proteins. A mistake (mutation) in any one of these genes causes production of a faulty protein, and this in turn affects the structure of the skin of the palms and soles. Most cases of palmoplantar keratoderma are caused by mutations in the gene that produces an epidermal protein called keratin 9. But there are several other genes and mutations responsible for the rarer types of palmoplantar keratoderma.

Diffuse nonepidermolytic palmoplantar keratoderma is an autosomal dominantly inherited condition traced to KRT1 and KRT16 keratins. Palmoplantar keratodermas are further distinguished by their mode of inheritance and by the presence or absence of associated features. These may include hyperhidrosis, exacerbation of hyperkeratosis by manual labour, nail changes, frequent dermatophyte infections, and severe malodor.

Symptoms:

Even, widespread thickened skin (keratosis) over the palms and soles.

    A red band at the edges of the keratosis is frequent.

  • Other keratotic lesions may appear on the tops of the hands, feet, knees, and elbows.

  • Excessive perspiration.

  • Nails may be thickened.

Palmoplantar keratoderma also causes difficulty with walking, because of pain in the feet. The thick skin and sweating of the feet makes them particularly susceptible to odor, and to fungal infection (athlete's foot). The thick skin on the palms may reduce sensitivity in the finger tips, impairing manual dexterity. All these problems, together with the unusual appearance can be stressful and lead to psychological difficulties.

Diagnosis:

Diagnosis of palmoplantar keratoderma depends mainly on history,clinical ?ndings, and appropriate histologic or metabolic data. The most common ?ndings on biopsy areimpressive hyperkeratosis, acanthosis, and hypergranulosis.Some forms of palmoplantar keratoderma exhibit prominentepidermolytic hyperkeratosis, characterized by pronounced vacuolization of the keratinocytes of themiddle and upper portions of the epidermis. Thisphenomenon is not speci?c and is also seen in bullous congenital ichthyosiform erythroderma, linearepidermal nevus, and various unrelated conditions.

Treatment:

At present there is no cure for palmoplantar keratoderma. The skin cells are programmed to make a faulty protein and this cannot be corrected. But the symptoms can be improved by the following measures:

    Regular use of moisturizing creams and ointments.

  • The use of simple antiseptics if odor is a problem.

  • Antifungal cream or tablets if fungal infection is present.

  • Regular chiropody to pare down the thick skin.

  • Customized insoles to relieve pressure-points on the feet.

Medication options:

    Topical keratolytics, such as 6% salicylic acid in white soft paraffin, or a gel of 6% salicylic acid in 70% propylene glycol.

  • Benzoic acid compounds.

  • Oral retinoids.

  • In severe cases a dermatologist may prescribe the drug acitretin. This thins the affected skin. Treatment has to be closely monitored to avoid excessive thinning of the skin and various side-effects of the drug. It is a drug that must never be taken during pregnancy because it can damage the baby.

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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