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 yr and 15 yr. 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 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.