Gudelines on Management of Palmo-Plantar Keratoderma

National Skin Centre, Singapore

Guidelines on Management of Palmo-Plantar Keratoderma

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Synonyms – ‘hyperperkeratosis of the palms and soles’, ‘palmoplantar hyperkeratosis’, ’tylosis’, ‘palmoplantar hyperkeratosis’ and ‘keratodermia’

Introduction

Palmoplantar keratoderma(PPK) is a diverse group of congenital or acquired disorders characterized by excessive formation of keratin on palms and soles. The thickening can present as a diffuse, focal or punctate pattern. Acquired and congenital PPK may be the only feature, PPK may accompany other diseases or be a part of a syndrome. Also, PPK may be a component of a generalized disorder of keratinisation such as lamellar ichthyosis.

In the classification of PPK, the following characteristics are considered; congenital or acquired, inheritance pattern, presence of skin lesions on areas other than palms and soles, other external or systemic abnormalities, age at onset, prognosis and severity of the disease and histopathologic and ultrastructural findings.

Hereditary Diseases with Palmoplantar Keratoderma

Thorst-Unna type(diffuse) – commonest type of PPK

Epidermolytic hyperkeratosis

Punctate(Buschke-Fischer and Brauer-Fuhs)

Areata/striata(Siemen’s)

Pachyonychia congenita

* Rarer forms of hereditary palmo-plantar keratoderma are listed in Appendix A

Some congenital keratodermas have one or more associated disorder/s involving bone, muscle, hair, nails, teeth, skin colour, nervous system and the eyes.

Acquired Palmo-Plantar Keratoderma

Acquired palmoplantar keratoderma may also be due to many causes. Some of the keratodermas which appear later in life may be genetically determined diseases manifesting later. Management of acquired PPK mostly depends of the cause of keratoderma.

Causes of acquired keratoderma;

Internal malignancy(Howel- Evans syndrome, and Bazex syndrome)

Due to constant friction or repeated trauma(frictional hyperkeratosis)

Chronic eczema

Lichen planus

Psoriasis

Dermatophytosis

Pityriasis rubra pilaris

Arsenical hyperkeratosis

Diagnosis

1. Usually on clinical grounds – consider ethnicity, parental consanguinity, presence from birth, other associated abnormalities, progression of disease and clinical features at presentation. In acquired PPK try to identify cause.

2. Histopathology

3. Electron Microscopy – not for routine cases, usually for research purposes.

4. Genetic studies – for special cases, study of keratin types- usually for research purposes

Managenent of keratodermas

I. General Measures

1. Treat cause - ifa cause is identified (in acquired keratoderma)

e.g. underlying malignancy, chronic eczema or psoriasis(please refer to the respective management guidelines), hyperkeratosis due to friction

2. Treatment of hyperhidrosois if associated with PPK

3. Paringdown keratinous plaques

4. Advice on proper footwear(not too tight, soft leather, soft soles, partially open etc.) and proper foot-care

5. Podiatric assistance– if needed

II. Topical Therapy – Fisrt Line Therapy

Keratolytics and related products– usually for symptomatic treatment of keratodermas

Depending on how thick the keratin layer is, whether it is focal or diffuse keratoderma, patient’s motivation for treatment, whether the surface is wet or dry etc. the treatment may be aggressive or minimal.

a.Salicylic acid – in a vehicle, singly or in combination with other agents

e.g. 6% - 40% salicylic acid in petrolatum base

Salicylic acid is the most commonly used keratolytic.

Start with 10% and modify strength depending on response

Keratolytic agents loosen the scales, but do not dissolve them. Ask patients to use a rough sponge to scrub them off.

Beware of salicylism, especially in children. Do not use more than 2g. of salicylic acid within 24 hours (which is approximately 33 g. of 6% salicylic acid ointment) in a child

b. Lactic acid 3%-17%

c. Propylene glycol 40%-70%, This also helps in controlling fungal infection esp. feet

d.Hydrating agents for dry keratodermas e.g 10 % urea

e. Topical retinoids esp. Tazarotene has been claimed to be useful, but is of limited use(not available at NSC)

III. Specific systemic therapy – Second Line Therapy

Reserved for more severe cases, especially severe hereditary PPK and PPK associated with more diffuse keratodermas.

NB. Acquired PPK which should be treated according to the cause of the PPK. Please refer to the relevant guidelines where available(e.g. psoriasis, eczema)

*Use of oral retinoids for keratodermas should be approved by a consultant

Acitretin(a metabolite of etretinate) is effective in many keratodermas e.g.Darier’s disease, epidermolytic hyperkeratosis, pityriasis rubra pilaris, severe diffuse palmoplantar keratoderma(Thorst-Unna) and keratosis palmoplantaris areata(Siemens).

Of the specific therapies directed at keratodermas, acitretin is the most effective drug. However, some types of PPK do not respond even to oral retinoids and in some, the side effects of acitretin limit its use.

Dose:

Initially 25-30 mg. acitretin daily for 2-4 weeks, then adjusted according to response, may go upto 50 mg daily for 6-8 weeks. Once stable, drop the dose slowly to a lower maintenance dose. Maintenance treatment of certain keratodermas may be as low as 10 mg per day.

In children, 0.5-1 mg/kg daily (to a maximum of 25 mg daily for limited periods) with careful monitoring of musculoskeletal development.

Congenital keratodermas may require lifelong treatment with oral retinoids. Lifelong treatment often poses practical problems due to high cost and side effects, especially premature closure of epiphyses, teratogenicity, effects on the liver, serum lipid levels and bones.

In females of child bearing age, a pregnancy test should be done a few days before expected menstruation; start treatment on day 2 or 3 of menstruation. Patients should avoid pregnancy 1 month before, during treatment and at least 2 years after stopping acitretin.

Monitor liver function tests and serum lipids levels before treatment, after 1 month, then every 3 months for one year, half yearly thereafter. A skeletal survey should be done every 3 years.

Contra indications: hepatic and renal impairment, hyperlipidaemia, pregnancy, breast feeding

* Before treatment get the patient (or a parent/guardian) to sign a document, after informing the possible side effects, as in the case of isotretinoin in acne.

IV. Surgery – Third Line Therapy

a. Excision of constricting bands - in mutilating keratoderma (Voh Winkel’s syndrome)

b. Excision of keratotic skin and skin grafting - in exceptional cases where walking is very painful due to localized keratoderma

V. Genetic counselling – for severe PPK & PPK associated with diseases such as epidermolytic hyperkeratosis

VI. Low phenyl alanine diet(in Tyrosinaemia Type II only)

In keratoderma due to oculocutaneous tyrosinaemia, early institution of low phenyl alanine and tyrosine diet - to prevent ocular and cutaneous symptoms and behavioural disorders.

Appendix A – Rare forms of hereditary palmo-plantar keratoderma

Voh winkel (mutilating)

Mal de Meleda

Greither’s

Papuloverrucous(Jakac-Wolf)

Focal acral hyperkeratosis (acrokeratoelastoidosis without elastorrhexis)

Keratoderma with scleroatrophy(Huriez syndrome)

Olmsted syndrome

Papillon Lefevre syndrome

Lamellar ichthyosis

Tyrosinaemia Type II (Richner-Hanhart syndrome)

Cowden disease

Dowling Meara syndrome

Dyskeratosis congenita

Cantu syndrome

Hydrotic ectodermal dysplasia (Clouston syndrome)

Francischetti-Jadassohn syndrome

Gottron’s syndrome

KID syndrome

Kindler syndrome

Oculo-osteocutaneous syndrome

Oudsthoorn disease(erythrokeratolysis haemalis)

Sjogren –Larsson syndrome

Darier disease

Appendix B - Rare forms of acquired keratoderma

Lithium therapy

Yaws

Sezary syndrome

Keratoderma climactericum (in females)

Secondary syphilis

Pagetoid reticulosis

References

1. Mallory SB, Leal-Khouri S. eds. An illustrated Dictionary of Syndromes. New York; Parthenon Publishing1994.
2. Thestrup-Pedersen K et al. Treatment of hyperkeratotic dermatitis of palms(eczema keratoticum) with oral acitretin. A single blind placebo controlled study. Acta Derm Venereol 2001;81:353-5
3. Sybert VP, Dale BA, Holbrook KA. Palmar-plantar keratoderma. a clinical, ultrastructural and biochemical study. J Am Acad Dermatol 1988;75-86.
4. Mehta DK ed. British National Formulary September 2002. London: British Medical Association 2002:557,559.
5. Champion RH, Burton JL, Burns DA, Breathnath eds. Textbook of Dermatology. 6th ed. Oxford: Blackwell Scientific Publications 1998;
6. Odom RB, James WD, Berger TG. eds. Andrew’s Diseases of the Skin 9th ed. Philadelphia; W B Saunders: 2000;245-247

Steijlen PM, Lucker GPH. Palmoplantar keratoses In: Textbook of Paediatric .Dermatology. Harper J, Oranje A, Prose N. eds. Oxford: Blackwell Science 2000;1122-1142.