Palmoplantar Keratodermas (PPK) Fact Sheet

Palmoplantar keratodermas (PPK)

Palmoplantar keratodermas are a group of disorders characterized by thickening of the skin on the palms of the hands and soles of the feet of affected individuals. The various forms of PPK can be divided into hereditary forms with only skin problems, hereditary syndromes with PPK as an associated feature, and acquired forms. The more commonly seen hereditary PPKs are discussed here.

Hereditary forms may be localized to the hands and feet, or they may be associated with a more generalized skin disorder. Classification of hereditary PPK is frequently confusing. A simple classification incorporates 3 factors:

  1. specific form and structure and distribution of the palmoplantar keratosis,
  2. presence of associated skin and other tissue (such as nails, hair, mucous membranes) disease in sites other than the palms and the soles, and
  3. presence or absence of fragile or blistering skin. 

 

Hereditary PPK’s

Diffuse Hereditary PPK without associated features:

Diffuse non-epidermolytic palmoplantar keratoderma
(also know as diffuse NEPPK, or PPK diffusa circumscripta).

Mode of inheritance: Diffuse non-epidermolytic palmoplantar keratoderma is an autosomal dominantly inherited condition traced to KRT1 and KRT16 keratins.
Onset of clinical features usually presents within the first two years of life.

Clinical features:

Treatment:


Diffuse epidermolytic palmoplantar keratoderma
(Also known as diffuse EPPK, Vorner disease, PPK cum degenerations granulose)
Diffuse epidermolytic PPK is the most common type of hereditary PPK. It has an autosomal dominant inheritance traced to KRT9 keratin.
Onset of clinical features usually takes place within the first year.

Clinical features:

Treatment:


Progressive Palmoplantar Keratoderma
(Also known as PPPK, Greither disease, PPK transgrediens et progrediens)

Progressive PPK is transmitted through an autosomal dominant inheritance.
Onset of clinical features usually appears between ages 8 and 10.

Clinical features:

Treatment:


Diffuse Hereditary PPK with associated features
(Diffuse PPK is associated with extra palmoplantar skin involvement in several inherited disorders of cornification. The more common conditions are outlined below.)

Mal de Meleda
(Also know as Keratosis extremitatum hereditaria transgrediens et progrediens)
Mal de Meleda is a rare disorder seen in approximately 1 in 100,000 people. It was initially observed in inhabitants of the Adriatic island of Meleda (Miljet). It is transmitted through an autosomal recessive inheritance. Clinical features of the disorder usually appear in early infancy. Palmoplantar keratoderma is often the only manifestation.

Clinical features:

Treatment:


PPK Mutilans Vohwinkel
(Also know as mutilating keratoderma, Vohwinkel syndrome, and palmoplantar keratoderma mutilans)
PPK Mutilans Vohwinkel is a rare disorder that can be transmitted through an autosomal dominant inheritance or an autosomal recessive inheritance. The genetic defect has been traced to the GJB2 gene and connexin 26. Clinical features usually appear in infancy.

Clinical features:

Treatment:


Mutilating Palmoplantar keratoderma with periorificial keratotic plaques
(Also known as Olmstead Syndrome)
A rare disorder transmitted through an autosomal dominant inheritance. Clinical features usually appear within the first year of life.

Clinical features:

Treatment:


Palmoplantar Keratoderma with sclerodactyly (hardening and thickening of the connective tissues of the fingers and toes).
(Also known as Huriez syndrome)
A rare disorder transmitted through an autosomal dominant inheritance. Clinical symptoms are visible in infancy.

Clinical features:


Palmoplantar Keratoderma with peridontitis (inflammation of the gums)
(Also known as Papillon-Lefevre Syndrome)
A rare disease transmitted through an autosomal recessive inheritance. The disorder results from mutations in cathepsin C. It occurs equally among males and females. Clinical features usually appear within the first and fifth years of life.

Clinical features:

Treatment:


Treatment of all types of hereditary and nonhereditary keratodermas can be difficult. Most treatment options only result in short-term improvement and are compounded frequently by side effects. Treatment tends to address the symptoms and can vary from saltwater soaks and paring down of the thickened skin, to topical keratolytics, oral retinoids, or reconstructive surgery of the hyperkeratotic skin followed by grafting. Common treatments include:

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Adapted from eMedicine.com, Keratosis Palmaris et Plantaris.   Copyright 2002, eMedicine.com, Inc. 


This information is provided as a service to patients and parents of patients who have ichthyosis.  It is not intended to supplement appropriate medical care, but instead to complement that care with guidance in practical issues facing patients and parents.  Neither FIRST, its Board of Directors, Medical & Scientific Advisory Board, Board of Medical Editors nor Foundation staff and officials endorse any treatments or products reported here.  All issues pertaining to the care of patients with ichthyosis should be discussed with a dermatologist experienced in the treatment of their skin disorder.



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