Disease with focal palmoplantar keratoderma as a major feature

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Overview

Disease with focal palmoplantar keratoderma as a major feature refers to a group of rare genetic skin conditions where the main problem is thickened skin on specific areas of the palms of the hands and soles of the feet. The term "focal" means the thickening does not cover the entire palm or sole but instead appears in distinct patches or spots, often at pressure points or areas of repeated friction. Palmoplantar keratoderma (PPK) means abnormal thickening of the outer layer of skin (the epidermis) on the palms and soles. This group of conditions can range from mild cosmetic concerns to more significant problems that affect walking, gripping objects, and daily comfort. The thickened skin can crack and become painful, and in some cases may be associated with other features depending on the specific underlying genetic cause. Some forms may also involve nail changes, excessive sweating, or skin changes in other body areas. Treatment is mainly focused on managing symptoms. This includes regular use of moisturizers, keratolytic creams (which help soften and remove thickened skin), and sometimes careful trimming of calluses by a dermatologist. In more severe cases, oral retinoid medications may be considered. There is currently no cure for these genetic forms of keratoderma, but ongoing skin care can significantly improve comfort and quality of life. Genetic counseling is recommended for affected families to understand the inheritance pattern and recurrence risk.

Also known as:

Key symptoms:

Thickened skin patches on the palms of the handsThickened skin patches on the soles of the feetPainful calluses at pressure points on hands and feetCracking or fissuring of thickened skinDifficulty walking due to painful solesDifficulty gripping objects due to thickened palmsBlistering at sites of frictionNail thickening or abnormal nailsExcessive sweating of hands and feetRedness around thickened skin areasSkin lesions that worsen with physical activity or pressure

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 Disease with focal palmoplantar keratoderma as a major feature.

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No actively recruiting trials found for Disease with focal palmoplantar keratoderma as a major feature at this time.

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No specialists are currently listed for Disease with focal palmoplantar keratoderma as a major feature.

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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 Disease with focal palmoplantar keratoderma as a major feature.

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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 focal palmoplantar keratoderma do I or my child have?,Should we pursue genetic testing to identify the exact gene involved?,What is the best daily skin care routine for managing the thickened skin?,Are oral retinoids appropriate in my case, and what are the risks?,How can I prevent painful cracking and skin infections?,Is this condition likely to get worse over time?,What is the chance of passing this condition to my children?

Common questions about Disease with focal palmoplantar keratoderma as a major feature

What is Disease with focal palmoplantar keratoderma as a major feature?

Disease with focal palmoplantar keratoderma as a major feature refers to a group of rare genetic skin conditions where the main problem is thickened skin on specific areas of the palms of the hands and soles of the feet. The term "focal" means the thickening does not cover the entire palm or sole but instead appears in distinct patches or spots, often at pressure points or areas of repeated friction. Palmoplantar keratoderma (PPK) means abnormal thickening of the outer layer of skin (the epidermis) on the palms and soles. This group of conditions can range from mild cosmetic concerns to more