Palmoplantar keratodermas

Palmoplantar keratodermas (PPK) are a heterogeneous group of disorders characterized by abnormal thickening of the skin on the palms of the hands and soles of the feet. PPK can be acquired or inherited, and the inherited forms are further divided into diffuse, focal, punctate, and striate types.

Etiology

The exact cause of PPK is not known, but it is believed to be due to mutations in the genes that regulate skin growth and differentiation. Some forms of PPK are associated with mutations in the keratin genes, while others are associated with mutations in the connexin genes. In some cases, PPK may be associated with other systemic diseases, such as cancer, heart disease, or diabetes.

Clinical Features

The main symptom of PPK is thickened, rough skin on the palms and soles. The severity of the skin thickening can vary from mild to severe. Other symptoms may include pain, difficulty walking, and cracks in the skin. In some cases, PPK may also affect the nails, causing them to become thick and brittle.

Diagnosis

Diagnosis of PPK is based on the clinical features and family history. In some cases, a skin biopsy may be needed to confirm the diagnosis. Genetic testing may also be done to identify the specific mutation causing the disease.

Treatment

There is no cure for PPK, but treatments are available to manage the symptoms. These may include topical creams and ointments to soften the skin, oral retinoids to slow skin growth, and physical therapies such as paring and debridement. In severe cases, surgery may be needed to remove the thickened skin.

Prognosis

The prognosis for individuals with PPK varies depending on the severity of the disease and the presence of associated systemic diseases. With appropriate management, most individuals with PPK can lead normal lives.

See Also

• Keratoderma
• Skin disease
• Genetic disorder