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Therapeutics in Dermatology
A reference textbook in dermatology

Palmoplantar keratoderma

14 October 2019, by COELHO M.

1 - ACKNOWLEDGEMENTS

Chapter written with the help of the EADV, the Fondation René Touraine and the Therapeutics in Dermatology

2 - OVERVIEW

Palmoplantar keratoderma (PPK) is a disorder of cornification, as it results from a disturbance of the formation of the outermost skin barrier (stratum corneum).

There are several disease variants, including inherited and acquired forms of PPK. Disease prevalence is not established for inherited PPKs, but diffuse epidermolytic PPK, the most common of these, is estimated to reach 4:100000 in some populations. Acquired PPK is relatively frequent.

3 - SIGNS AND SYMPTOMS

PPK is clinically characterised by skin thickening (“keratoderma”) affecting the palms of the hands and the soles of the feet (“palmoplantar”), although sometimes disease extends to the top of the hands and feet (transgrediens).

There are three major patterns of palmoplantar skin involvement:

  • Diffuse: PPK affecting most of the surface of the palms and soles;
  • Focal: PPK affecting mainly pressure areas;
  • Punctate: PPK manifesting as small callus-like skin plaques.

The affected skin is initially red and later becomes thick and yellow, with a waxy surface and a sharply demarcated, pink-red border. Patients may experience severe, disabling pain, a burning sensation, and sometimes pruritus.

Additional clinical features include digital constrictions (pseudoainhum), excessive sweating, moist skin, blistering, fungal infections and malodour.

Patients with inherited PPKs usually present with symptoms during the first months or years of life, although some patients do not show manifestations until adulthood. Onset and severity of disease expression depend also on mechanical stress, including pressure and friction. Disease presentation can vary over time (e.g.: focal PPK may become diffuse) and some patients may improve spontaneously. Patients with inherited PPKs can also have other associated manifestations, like nail dystrophy, hair anomalies, oral lesions, ocular involvement, deafness and cardiac disease.

Skin manifestations of acquired PPK resemble those of the inherited forms but begin in adulthood.

4 - WHO GETS AND CAUSES

4.1 - INHERITED PKKS

Inherited PPKs are due to genetic mutations that affect the structure and function of several skin proteins (e.g.: keratins), being transmitted in different patterns within families. These PPKs can either represent a skin-limited disease or be part of a syndromic, multiorgan disease, with extracutaneous manifestations. Some of the inherited PPKs are listed in categories bellow:

Diffuse PPK

  • Skin disease only: Vörner-Unna-Thost type (diffuse epidermolytic PPK); “Greither” type; Mal de Meleda
  • With associated features: Vohwinkel syndrome; Huriez syndrome; Olmsted syndrome; Naxos disease

Focal PPK

  • Skin disease only: Striate PPK
  • With associated features: Pachyonychia congenita; Howel-Evans syndrome; Carvajal syndrome

Punctate PPK

  • Skin disease only: Buschke-Fischer-Brauer type; Marginal papular keratoderma
  • With associated features: Cole disease

4.2 - ACQUIRED PKK

Acquired PPK encompasses the non-hereditary form of disease, and is usually associated with systemic conditions or drugs, including:

  • Peri-menopause (keratoderma climactericum)
  • Immersion in water (aquagenic PPK)
  • Inflammatory skin diseases (e.g.: pityriasis rubra pilaris)
  • Cancer (e.g.: skin lymphomas; breast, lung, colon, stomach, kidney cancers)
  • Other internal diseases (e.g.: hypothyroidism/myxedema; dermatomyositis)
  • Anti-cancer drugs (e.g.: tyrosine kinase inhibitors; Capecitabine)
  • Other drugs (e.g.: Lithium, Verapamil, Venlafaxine)

5 - DIAGNOSIS AND TREATMENT

Dermatologists can often diagnose these disorders clinically, but a skin biopsy is usually performed to confirm the diagnosis and the type of PPK. Further diagnostic work-up, including genetic testing, may be warranted.

There is no cure for inherited PPKs. Acquired PPK usually resolves with treatment of the underlying disease or withdrawal of the causative drug.

General skin care measures recommended for patients with PPK include regular application of moisturising creams containing keratolytics (e.g.: urea, salicylic acid, lactic acid, propylene glycol) on the affected skin of palms and soles. Topical retinoids and topical vitamin D (Calcipotriol) may be helpful. Mechanical debridement of the most troublesome hyperkeratotic areas, using a blade or a dental drill, may be necessary.

Treatment with systemic retinoids, like Acitretin, may be beneficial in severe forms.

6 - TIPS FOR MANAGING

Every patient with suspected PPK should see a Dermatologist for adequate diagnosis and treatment.

People suffering from PPK require daily skin care, consisting of regular application of moisturising creams containing keratolytics on the affected areas of palms and soles, to reduce skin thickness and associated pain. Controlling bothersome malodour is also important for these patients.

Secondary fungal infections must be prevented and promptly treated.

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