About Epidermolytic palmoplantar keratoderma

What is Epidermolytic palmoplantar keratoderma?

Epidermolytic palmoplantar keratoderma (EPPK) is a rare genetic skin disorder that affects the palms and soles of the feet. It is characterized by thickening of the skin on the palms and soles, which can lead to pain, itching, and cracking. It is caused by mutations in the genes that control the production of keratin, a protein that helps form the outer layer of the skin. Treatment typically involves topical medications and lifestyle modifications.

What are the symptoms of Epidermolytic palmoplantar keratoderma?

The main symptom of epidermolytic palmoplantar keratoderma is thick, scaly skin on the palms of the hands and soles of the feet. Other symptoms may include:

• Thick, yellowish-brown patches of skin on the palms and soles

• Fissures (cracks) in the skin

Blisters or ulcers on the palms and soles

Itching or Burning sensation

• Thickening of the nails

• Thickening of the skin on the fingers and toes

• Thickening of the skin on the elbows and knees

• Thickening of the skin on the face, neck, and scalp

What are the causes of Epidermolytic palmoplantar keratoderma?

Epidermolytic palmoplantar keratoderma is caused by mutations in the genes encoding keratin 1 and keratin 10, which are proteins that form the structural framework of the skin. These mutations cause the skin to become thick and scaly, and can lead to other skin problems such as blisters, calluses, and infections.

What are the treatments for Epidermolytic palmoplantar keratoderma?

1. Topical treatments: These include topical corticosteroids, topical retinoids, topical calcineurin inhibitors, and topical keratolytics.

2. Systemic treatments: These include oral retinoids, oral calcineurin inhibitors, and oral antibiotics.

3. Phototherapy: This involves exposing the affected areas to ultraviolet light.

4. Surgery: This may be necessary to remove thickened skin or to reduce the size of the affected areas.

5. Other treatments: These include topical emollients, topical moisturizers, and topical antifungal agents.

What are the risk factors for Epidermolytic palmoplantar keratoderma?

1. Genetic predisposition: Epidermolytic palmoplantar keratoderma is an inherited disorder caused by mutations in the KRT1 or KRT10 genes.

2. Age: The condition is usually present at birth or develops in early childhood.

3. Gender: Epidermolytic palmoplantar keratoderma is more common in males than females.

4. Ethnicity: The condition is more common in people of Mediterranean, Middle Eastern, and North African descent.

Is there a cure/medications for Epidermolytic palmoplantar keratoderma?

Yes, there are treatments available for epidermolytic palmoplantar keratoderma. Treatment options include topical medications such as corticosteroids, retinoids, and calcipotriene, as well as systemic medications such as acitretin, methotrexate, and cyclosporine. Additionally, laser therapy and photodynamic therapy may be used to reduce the appearance of lesions.