Isolated diffuse palmoplantar keratoderma

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Overview

Isolated diffuse palmoplantar keratoderma (PPK) is a skin condition in which the skin on the palms of the hands and the soles of the feet becomes abnormally thick, hard, and sometimes yellowish. The word "diffuse" means the thickening covers most or all of the palms and soles rather than appearing in small patches. "Isolated" means this thickening occurs on its own, without other organ systems being affected. The condition is also sometimes called diffuse hereditary palmoplantar keratoderma or tylosis. The thickened skin develops because the outer layer of skin (the epidermis) produces too much of a tough protein called keratin. This can cause discomfort, cracking, pain when walking or gripping objects, and sometimes a bad odor or increased sweating of the affected areas. In some cases, the thickened skin can extend slightly beyond the palms and soles onto the tops of the hands or feet, which doctors call "transgrediens." There is currently no cure for isolated diffuse palmoplantar keratoderma. Treatment focuses on managing symptoms and improving quality of life. This typically includes regular use of moisturizers, keratolytic creams (which help soften and remove thickened skin), and sometimes oral retinoid medications in more severe cases. Mechanical removal of thickened skin by a dermatologist or podiatrist can also help. With consistent care, most people with this condition can lead active and fulfilling lives, though ongoing skin management is usually needed throughout life.

Also known as:

Key symptoms:

Thick, hard skin on the palms of the handsThick, hard skin on the soles of the feetYellowish discoloration of palms and solesPainful cracking or fissuring of thickened skinPain when walking or standing for long periodsDifficulty gripping objects due to stiff skinExcessive sweating of hands and feetUnpleasant odor from affected skinRedness around the edges of thickened skinSkin thickening that may extend to the backs of hands or feetCallus-like buildup that returns after removalBlistering in some forms (epidermolytic type)

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Isolated diffuse palmoplantar keratoderma.

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No actively recruiting trials found for Isolated diffuse palmoplantar keratoderma at this time.

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No specialists are currently listed for Isolated diffuse palmoplantar keratoderma.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Isolated diffuse palmoplantar keratoderma.

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Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.What specific type of palmoplantar keratoderma do I (or my child) have, and what gene is involved?,What is the best daily skin care routine to manage the thickened skin?,Would oral retinoid medication be appropriate, and what are the risks and benefits?,How often should I see a dermatologist or podiatrist for skin maintenance?,Is genetic testing recommended for other family members?,Are there any clinical trials or new treatments being studied for this condition?,What should I do if the skin becomes infected or very painful?

Common questions about Isolated diffuse palmoplantar keratoderma

What is Isolated diffuse palmoplantar keratoderma?

Isolated diffuse palmoplantar keratoderma (PPK) is a skin condition in which the skin on the palms of the hands and the soles of the feet becomes abnormally thick, hard, and sometimes yellowish. The word "diffuse" means the thickening covers most or all of the palms and soles rather than appearing in small patches. "Isolated" means this thickening occurs on its own, without other organ systems being affected. The condition is also sometimes called diffuse hereditary palmoplantar keratoderma or tylosis. The thickened skin develops because the outer layer of skin (the epidermis) produces too mu