Palmoplantar keratoderma

Published on 19/03/2015 by admin

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Palmoplantar keratoderma

Ravi Ratnavel

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

image

Palmoplantar keratodermas (PPKs) consist of a heterogeneous group of disorders characterized by thickening of the palms and soles. The condition may be subdivided into hereditary keratodermas, acquired forms, and conditions in which PPK is an associated feature of a specific dermatosis.

Management strategy

PPK may be localized to the hands and feet, or develop as part of a more generalized skin disorder. It is important when making a diagnosis to establish its morphology and the presence of any associated ectodermal disease at sites other than the palms and soles. Biopsy may be necessary to distinguish between some hereditary forms of PPK. PPK can be associated with infections (dermatophytes, human papillomavirus, HIV, syphilis, and scabies), drugs (arsenic exposure), and internal malignancy, or may be a cutaneous manifestation of systemic disease (myxedema, diabetes mellitus, or cutaneous T-cell lymphoma). Hyperkeratosis of the palms and soles can also be a feature of eczema, psoriasis, and cutaneous T-cell lymphoma.

The treatment of PPK is difficult. Most therapeutic options produce only short-term improvement and are frequently complicated by unwanted adverse effects. Treatment options range from simple measures such as salt-water soaks with paring and use of topical keratolytics, to systemic retinoids and reconstructive surgery with total excision of the hyperkeratotic skin followed by grafting.

In patients with limited disease, topical keratolytics containing salicylic acid, lactic acid, or urea in a suitable base may be tried. Examples include 5–10% salicylic acid, 10–40% propylene glycol, or 10% lactic acid in aqueous cream or a combination therapy using 10% urea and 5% lactic acid in aqueous cream to be applied twice daily. These formulations can be made up on an individual basis, or the closest proprietary product prescribed. The efficacy of these agents may be increased by occlusion at night. Topical retinoids such as tretinoin (0.01% gel and 0.1% cream) may also be tried; treatment is however often limited by skin irritation. Potent topical corticosteroids, such as clobetasol propionate 0.05%, with or without keratolytics, are occasionally of value in the management of inflammatory PPK. 5-Fluorouracil 5% has produced dramatic results in spiny keratoderma, but its use in other keratodermas has not been evaluated.

The efficacy of the oral retinoids in keratoderma is well established. Good responses have been seen in mal de Meleda, Papillon–Lefèvre syndrome, and erythrokeratoderma variabilis. In some types of PPK, particularly epidermolytic forms, hyperesthesia may limit the usefulness or practicality of treatment with retinoids. The potential risk of bone toxicity should also be assessed in patients on long-term therapy, although the risks are small. Periodic radiologic bone monitoring and, when possible, prescription of pulsed (intermittent) therapy are recommended. The optimal dosage of acitretin is 25–35 mg daily in adults or 0.7 mg/kg daily in children, which may be adjusted after 4 weeks of therapy.

Psoralen plus UVA (PUVA) therapy or re-PUVA (a synergistic combination of oral retinoids and PUVA) may be effective in PPK secondary to psoriasis or eczema. In oculocutaneous tyrosinemia (an autosomal recessive condition characterized by focal palmoplantar keratosis, corneal ulceration, and mental retardation), dietary restriction of phenylalanine and tyrosine has led to resolution of PPK. Oral administration of 1α,25-dihydroxyvitamin D3 and topical calcipotriol ointment has been reported to be effective. Regular podiatry, careful selection of footwear, and treatment of secondary fungal infections is an integral part of management of all PPK. Regular intermittent use of terbinafine cream and other topical antifungals can reduce skin maceration and improve comfort. Surgical or laser dermabrasion is an option for some patients, with potential amelioration of symptoms and improved penetration of topical agents.

For severe refractory PPK excision and skin grafting may be considered. Excision should remove hyperkeratotic skin, including dermis, epidermis, and subcutis, to prevent any risk of recurrence.

Specific investigations

First-line therapies

imageTopical keratolytics B
imageTopical retinoids B

Second-line therapies

imageSystemic retinoids A

Third-line therapies

imageReconstructive surgery with total excision of hyperkeratotic skin followed by grafting C
imageTopical calcipotriol E
imageOral vitamin D3 analogs E
imageTopical corticosteroids with or without keratolytics E
imagePUVA or re-PUVA D
imageDermabrasion E
imageCO2 laser B
image5-Fluorouracil E
imageTyrosine-restricted diet in oculocutaneous keratoderma E

[/level-membership-for-dermatology-category][not-level-membership-for-dermatology-category]

Palmoplantar keratoderma

Ravi Ratnavel

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

image

Palmoplantar keratodermas (PPKs) consist of a heterogeneous group of disorders characterized by thickening of the palms and soles. The condition may be subdivided into hereditary keratodermas, acquired forms, and conditions in which PPK is an associated feature of a specific dermatosis.

Management strategy

PPK may be localized to the hands and feet, or develop as part of a more generalized skin disorder. It is important when making a diagnosis to establish its morphology and the presence of any associated ectodermal disease at sites other than the palms and soles. Biopsy may be necessary to distinguish between some hereditary forms of PPK. PPK can be associated with infections (dermatophytes, human papillomavirus, HIV, syphilis, and scabies), drugs (arsenic exposure), and internal malignancy, or may be a cutaneous manifestation of systemic disease (myxedema, diabetes mellitus, or cutaneous T-cell lymphoma). Hyperkeratosis of the palms and soles can also be a feature of eczema, psoriasis, and cutaneous T-cell lymphoma.

The treatment of PPK is difficult. Most therapeutic options produce only short-term improvement and are frequently complicated by unwanted adverse effects. Treatment options range from simple measures such as salt-water soaks with paring and use of topical keratolytics, to systemic retinoids and reconstructive surgery with total excision of the hyperkeratotic skin followed by grafting.

In patients with limited disease, topical keratolytics containing salicylic acid, lactic acid, or urea in a suitable base may be tried. Examples include 5–10% salicylic acid, 10–40% propylene glycol, or 10% lactic acid in aqueous cream or a combination therapy using 10% urea and 5% lactic acid in aqueous cream to be applied twice daily. These formulations can be made up on an individual basis, or the closest proprietary product prescribed. The efficacy of these agents may be increased by occlusion at night. Topical retinoids such as tretinoin (0.01% gel and 0.1% cream) may also be tried; treatment is however often limited by skin irritation. Potent topical corticosteroids, such as clobetasol propionate 0.05%, with or without keratolytics, are occasionally of value in the management of inflammatory PPK. 5-Fluorouracil 5% has produced dramatic results in spiny keratoderma, but its use in other keratodermas has not been evaluated.

The efficacy of the oral retinoids in keratoderma is well established. Good responses have been seen in mal de Meleda, Papillon–Lefèvre syndrome, and erythrokeratoderma variabilis. In some types of PPK, particularly epidermolytic forms, hyperesthesia may limit the usefulness or practicality of treatment with retinoids. The potential risk of bone toxicity should also be assessed in patients on long-term therapy, although the risks are small. Periodic radiologic bone monitoring and, when possible, prescription of pulsed (intermittent) therapy are recommended. The optimal dosage of acitretin is 25–35 mg daily in adults or 0.7 mg/kg daily in children, which may be adjusted after 4 weeks of therapy.

Psoralen plus UVA (PUVA) therapy or re-PUVA (a synergistic combination of oral retinoids and PUVA) may be effective in PPK secondary to psoriasis or eczema. In oculocutaneous tyrosinemia (an autosomal recessive condition characterized by focal palmoplantar keratosis, corneal ulceration, and mental retardation), dietary restriction of phenylalanine and tyrosine has led to resolution of PPK. Oral administration of 1α,25-dihydroxyvitamin D3 and topical calcipotriol ointment has been reported to be effective. Regular podiatry, careful selection of footwear, and treatment of secondary fungal infections is an integral part of management of all PPK. Regular intermittent use of terbinafine cream and other topical antifungals can reduce skin maceration and improve comfort. Surgical or laser dermabrasion is an option for some patients, with potential amelioration of symptoms and improved penetration of topical agents.

For severe refractory PPK excision and skin grafting may be considered. Excision should remove hyperkeratotic skin, including dermis, epidermis, and subcutis, to prevent any risk of recurrence.

Specific investigations

Palmoplantar keratoderma in association with myxoedema.
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