Palmoplantar Keratodermas

By James G. H. Dinulos, MD, Adjunct Associate Professor of Surgery (Dermatology), Dartmouth Medical School

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.