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Treatment:
Topical keratolytics
Oral retinoids.
Topical calcipotriol.
Progressive Palmoplantar Keratoderma
(Also known as PPPK, Greither disease, PPK transgrediens et progrediens)
Progressive PPK is transmitted through an autosomal dominant inheritance.
Onset of clinical features usually appears between ages 8 and 10.
Clinical features:
The widespread thickened skin spreads from the palms and the soles to the tops of the hands and feet and
up the Achilles tendon (back of the heel).
Excessive perspiration and variations in signs and symptoms between affected family members are
common.
Signs and symptoms tend to be worse during childhood, static after puberty, and improve in middle age.
Treatment:
Topical keratolytics, such as 6% salicylic acid in white soft paraffin, or a gel of 6% salicylic acid in 70%
propylene glycol.
Benzoic acid compounds.
Oral retinoids.
Symmetrical, sharply defined palmoplantar keratoderma surrounded by reddened skin and deformities of
the joints that lead to constriction and spontaneous amputation.
Horny growths around the eyes and mouth.
Nail abnormalities.
White thickened patches of skin around the anus and in the mouth.
Sparse hair.
Treatment:
Oral retinoids.
Topical retinoids.
Palmoplantar Keratoderma with sclerodactyly (hardening and thickening of the connective tissues of the fingers and
toes).
(Also known as Huriez syndrome)
A rare disorder transmitted through an autosomal dominant inheritance. Clinical symptoms are visible in infancy.
Clinical features:
Sclerodactyly scleroderma or hardening and thickening of the connective tissues of the fingers and toes.
Widespread thickened skin more marked on the soles than on the palms.
Nail abnormalities.
Decreased sweating.
Associated with squamous cell carcinoma.
Treatment:
Dental care and appropriate antibiotic therapy for periodontitis.
Oral retinoids.
Treatment of all types of hereditary and nonhereditary keratodermas can be difficult. Most treatment options only
result in short-term improvement and are compounded frequently by side effects. Treatment tends to address the
symptoms and can vary from saltwater soaks and paring down of the thickened skin, to topical keratolytics, oral
retinoids, or reconstructive surgery of the hyperkeratotic skin followed by grafting. Common treatments include:
Topical keratolytics including 5 10% salicylic acid, 10% lactic acid, or 10% urea in a neutral base.
Topical retinoids, such as tretinoin (0.05% gel and 0.1% cream), are effective, but treatment can cause
irritation of the surrounding skin.
Topical steroids can be considered, with or without keratolytics, in conditions where there is an
inflammatory component.
Treatment with 5% 5-fluorouracil has produced dramatic results in spiny keratoderma, but its use in other
keratodermas has not been evaluated.
Oral retinoids are effective, especially in some hereditary PPKs such as Mal de Meleda, Papillon-Lefevre
syndrome, and erythrokeratodermia variabilis (a form of ichthyosis that often includes palmoplantar
keratoderma). Most hereditary PPKs require long-term treatment. Caution is advised if the patient has a
blistering form, since large erosions may occur with oral retinoids therapy. Patients should be started on a
low dose, and dosage should be increased should be carefully increased to avoid flaring the disease and/or
causing erosions.
Regular foot care, careful selection of footwear, and treatment of fungal infections are important.
Dermabrasion may help topical agents penetrate, and carbon dioxide laser treatment may be beneficial in
limited keratodermas.
For severe, difficult to treat keratoderma, surgery may need to be considered. Total excision of
hyperkeratotic skin followed by grafts has been successful in a number of cases.
Adapted from eMedicine.com, Keratosis Palmaris et Plantaris. Copyright 2002, eMedicine.com, Inc.