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Palmoplantar Keratodermas

By

James G. H. Dinulos

, MD, Geisel School of Medicine at Dartmouth

Last full review/revision May 2021| Content last modified May 2021
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Palmoplantar keratodermas are rare inherited disorders characterized by palmar and plantar hyperkeratosis.

Palmoplantar keratodermas encompass a broad range of inherited disorders. They may be categorized based on degree of skin involvement (eg, localized to the palms and soles, expanding to involve other cutaneous sites and adnexal structures) and on whether they are part of a recognizable syndrome that affects other organs, such as the eyes and ears. 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 entirety of the 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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