Download 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 - if a 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. Paring down 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 9 th
ed. Philadelphia; W B Saunders: 2000;245-247
7.
Steijlen PM, Lucker GPH. Palmoplantar keratoses In: Textbook of
Paediatric .Dermatology. Harper J, Oranje A, Prose N. eds. Oxford:
Blackwell Science 2000;1122-1142.