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Palmoplantar Keratodermas


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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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 of Palmoplantar Keratodermas

  • 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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