Striate palmoplantar keratoderma

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ORPHA:50942OMIM:148700Q82.8
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Overview

Striate palmoplantar keratoderma (also called striate PPK or SPPK) is a rare inherited skin condition that causes thick, hardened skin to develop on the palms of the hands and the soles of the feet. The word 'striate' refers to the streaky or linear pattern of thickened skin that often appears along the fingers and palms, while broader patches may form on the soles. This thickening happens because the skin produces too much of a tough protein called keratin, making it build up in certain areas. The condition usually becomes noticeable in early childhood, often when a child begins walking or using their hands more actively — pressure and friction seem to trigger or worsen the thickening. The skin can become painful, especially with physical activity, and may crack or peel. In some cases, the nails may also be affected. The condition is not life-threatening, but it can significantly affect comfort and daily activities. There is currently no cure for striate palmoplantar keratoderma. Treatment focuses on managing symptoms, mainly by softening and reducing the thickened skin using moisturizers, keratolytic creams (products that help shed excess skin), and sometimes oral retinoids prescribed by a dermatologist. With consistent care, many people are able to manage their symptoms and maintain a good quality of life.

Also known as:

Key symptoms:

Thick, hardened skin on the palms and solesStreaky or linear bands of thickened skin along the fingersBroad patches of thickened skin on the soles of the feetSkin pain or tenderness, especially with walking or grippingSkin cracking or fissuring in affected areasPeeling or shedding of thickened skinWorsening of skin thickening with pressure or frictionNail changes in some casesDifficulty with fine motor tasks due to thickened finger skinDiscomfort or difficulty walking

Clinical phenotype terms (3)— hover any for plain English
Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Infantile

Begins in infancy, roughly 1 month to 2 years old

Orphanet ↗OMIM ↗NORD ↗

Treatments

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

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

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No specialists are currently listed for Striate 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 Striate palmoplantar keratoderma.

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Community

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Caregiver Resources

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Social Security Disability

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Questions for your doctor

Bring these to your next appointment

  • Q1.Which gene is causing this condition in our family, and what does that mean for other family members?,What skin care routine do you recommend, and which products are most effective?,Are there any treatments beyond moisturizers that might help, such as prescription creams or oral medications?,What signs should I watch for that would mean the condition is getting worse or becoming infected?,Are there any activities or footwear types I should avoid to prevent worsening?,Should other family members be tested, and if so, how?,Are there any clinical trials or new treatments being studied that we should know about?

Common questions about Striate palmoplantar keratoderma

What is Striate palmoplantar keratoderma?

Striate palmoplantar keratoderma (also called striate PPK or SPPK) is a rare inherited skin condition that causes thick, hardened skin to develop on the palms of the hands and the soles of the feet. The word 'striate' refers to the streaky or linear pattern of thickened skin that often appears along the fingers and palms, while broader patches may form on the soles. This thickening happens because the skin produces too much of a tough protein called keratin, making it build up in certain areas. The condition usually becomes noticeable in early childhood, often when a child begins walking or u

How is Striate palmoplantar keratoderma inherited?

Striate palmoplantar keratoderma follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Striate palmoplantar keratoderma typically begin?

Typical onset of Striate palmoplantar keratoderma is infantile. Age of onset can vary across affected individuals.