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Chapter 34: Hereditary Palmoplantar Keratodermas

Dan Vodo; Liat Samuelov; Eli Sprecher

HEREDITARY PALMOPLANTAR KERATODERMAS

Therapeutic Approach

Palmoplantar keratoderma (PPK) refers to a clinically and genetically heterogeneous group of disorders of cornification, characterized by abnormal thickening of the skin of the palms and soles. PPKs can be inherited or acquired as in psoriasis. Inherited PPKs are usually classified according to their morphology (diffuse, focal, punctate), mode of inheritance, and the presence or absence of extracutaneous features. This chapter discusses therapeutic options for hereditary PPKs, for which, unfortunately, no curative therapy is currently available. In the authors’ experience, initial therapy should include lifestyle adjustments as well as topical emollients and keratolytic agents, with first-line systemic therapy often being retinoids. Other treatment options should be based on the PPK subtype as well as clinical severity and medical history. See Table 34-1.

Table 34-1Hereditary Palmoplantar Keratodermas Treatment Table
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Table 34-1 Hereditary Palmoplantar Keratodermas Treatment Table
MEDICATION/INTERVENTION/PROCEDURE INDICATIONa MECHANISM OF ACTION DOSING ADVERSE EFFECTS SUGGESTED MONITORINGb LEVEL OF EVIDENCE
General measures
Insoles or orthotics All types Limit mechanical pressure PRN None None IV1
Wicking socks All types Absorb sweat PRN None None IV1
Mechanical trimming All types Mechanical keratolysis By pumice, grater, blade or scalpel Secondary infection None IV1
Emollients All types Softening PRN None None IV1
Antibiotics or antifungals All types Treatment of superinfection Topical or systemic treatment According to chosen drug As per drug IV1
Topical Therapy
Keratolytic agents All types Keratolysis, Softening Salicylic acid 5%-20%; propylene glycol 35%–70%; urea 10%-30%; lactic acid 5%-10%; sodium lactate 30%; apply QD-BID, usually under occlusion and with emollients Skin irritation, burning; extensive application of salicylic acid, especially in neonates/infants, can lead to salicylism None III2-6
Vitamin D analogues EPPK, PPPK, OLS Antiproliferative, promotes differentiation Calcipotriol 0.005% cream/ointment; apply BID Skin irritation; intense exposure can lead to hypercalcemia and nephrolithiasis None III7-9
Glucocorticoids PPPK, OLS Anti-inflammatory, epidermal thinning Usually in combination with keratolytics or calcipotriol; QD-BID Skin irritation, atrophy, striae, telangiectasias, hypopigmentation None III4,9
Retinoids PPPK, MDM Antiproliferative, anti-inflammatory, promotes differentiation Tazarotene 0.1% gel; retinoic acid 0.025%-0.05%; adapalene 0.1% apply QD Skin irritation, burning and stinging None III10,11
Sirolimus PC Anti-proliferative, inhibition of keratins expression (eg, KRT6, KRT16) 1% ointment, BID Skin irritation None III12
Fluorouracil (5-FU) PPPK Desquamation, cytotoxic effect 5% cream, QD Skin irritation None III13
Gentamycin Nagashima type PPK Readthrough of SERPINB7 nonsense mutations 0.1% gentamicin ointment BID Skin irritation, pruritic erythema, vesicles None IB14
Systemic Therapy (often used in conjunction with other therapeutic modalities)
Retinoids PPPK, VWS, MDM, PLS, CS, LOR, PC, EPPK, Huriez syndrome Antiproliferative, anti-inflammatory, promotes differentiation, can prevent pseudoainhum development

Alitretinoin 20-40 mg QD; Acitretin 0.25-0.50 mg/kg QD;

Isotretinoin 0.35-0.5 mg/kg QD

 Teratogenic, skin dryness, nausea, hair loss, increased lipids, increased liver enzymes, PTC, rhabdomyolysis; In PC and EPPK can induce blisters and worsen pain CBC, ...

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