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Palmoplantar Keratodermas

By James G. H. Dinulos, MD

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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 diffuse effects, with onset between ages 5 yr and 15 yr. Esophageal cancer may develop at a young age.

  • Unna-Thost disease and Vorner disease: These autosomal dominant forms have diffuse effects.

  • Papillon-Lefèvre syndrome: This autosomal recessive form causes manifestations before age 6 mo. 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.


  • 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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