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Palmoplantar Keratodermas

By

James G. H. Dinulos

, MD, Geisel School of Medicine at Dartmouth

Last full review/revision Jan 2020| Content last modified Jan 2020
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Topic Resources

Palmoplantar keratodermas are rare inherited disorders characterized by palmar and plantar hyperkeratosis.

Most palmoplantar keratodermas are not severe and are autosomal dominant. Secondary infections are common. Examples include the following:

  • Howel-Evans syndrome: This autosomal dominant form has extracutaneous manifestations, with onset between ages 5 years and 15 years. Esophageal cancer may develop at a young age.

  • Unna-Thost disease and Vorner disease: These are autosomal dominant forms.

  • Papillon-Lefèvre syndrome: This autosomal recessive form causes manifestations before age 6 months. Severe periodontitis can result in loss of teeth.

  • Vohwinkel syndrome: In this autosomal dominant form, patients may also develop digital autoamputation and high-frequency hearing loss.

  • Diffuse nonepidermolytic palmoplantar keratoderma: This autosomal dominant form develops in infancy and causes well-demarcated, symmetric keratoderma involving the entire palms and soles.

Treatment

  • Symptomatic treatment

Symptomatic measures can include emollients, keratolytics, and physical scale removal. Secondary infections require treatment with antimicrobials. Oral retinoids are sometimes used.

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