Palmoplantar keratodermas are rare inherited or acquired disorders characterized by palmar and plantar hyperkeratosis.
Topic Resources
Palmoplantar keratodermas encompass a broad range of inherited or acquired disorders. They may be categorized based on the degree of skin involvement (eg, localized to the palms and soles, or 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. Hyperkeratosis of the palmoplantar regions is also known as tylosis.
Examples of inherited palmoplantar keratodermas 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 Vörner disease: These are autosomal dominant forms.
Papillon-Lefèvre syndrome: This autosomal recessive form causes manifestations before the age of 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.
Layers of the Skin
In this figure, the 5 layers of the epidermis along with its basement membrane and the dermis can be seen. Note that the stratum lucidum is present over the palms and soles only. The basement membrane is disproportionately enlarged to display its layers. The sublamina densa anchors the dermis to the upper layers of the basement membrane. |
Acquired forms of palmoplantar keratodermas are caused by chronic contact dermatitis, certain infections including Norwegian scabies, or as sequelae of chronic conditions such as myxedema and or chronic lymphedema. Acquired palmoplantar keratotic thickening may occur as a paraneoplastic symptom of various parenchymal cancers, including bladder, breast, colon, esophageal, and sometimes lung cancer.
Treatment of Palmoplantar Keratodermas
Symptomatic treatment
Symptomatic measures can include emollients, keratolytics, and physical scale removal.
Secondary infections require treatment with antimicrobials.
Topical and oral retinoids are sometimes used.
