Autosomal dominant focal non-epidermolytic palmoplantar keratoderma with plantar blistering

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ORPHA:402003OMIM:615735Q82.8
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Overview

Autosomal dominant focal non-epidermolytic palmoplantar keratoderma with plantar blistering is a very rare inherited skin condition that primarily affects the skin on the palms of the hands and the soles of the feet. The term "palmoplantar keratoderma" means thickening of the skin (keratoderma) on the palms (palmo-) and soles (plantar). In this specific form, the thickening occurs in a focal pattern, meaning it develops in certain pressure-bearing areas rather than covering the entire palm or sole. A distinguishing feature of this condition is the development of blisters on the soles of the feet, which can be painful and may interfere with walking and daily activities. The term "non-epidermolytic" refers to the fact that, when examined under a microscope, the skin cells do not show a specific type of breakdown pattern seen in some other forms of keratoderma. Because this is an autosomal dominant condition, only one copy of the altered gene (inherited from one parent) is needed to cause the disease. Symptoms typically develop in childhood or early adulthood and may worsen with physical activity or friction on the feet. Treatment is mainly supportive and focuses on managing symptoms. This includes regular use of moisturizers, keratolytic agents (creams that help soften and remove thickened skin), protective footwear, and careful wound care for blisters. There is currently no cure for this condition, and management is lifelong.

Key symptoms:

Thickened skin on the palms of the handsThickened skin on the soles of the feetBlisters on the soles of the feetPain when walking or standingCallus-like patches on pressure points of the feetSkin tenderness on the solesDifficulty walking due to foot painWorsening of symptoms with physical activity or frictionCracking or fissuring of thickened skinDiscomfort when wearing shoes

Inheritance

Autosomal dominant

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

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Autosomal dominant focal non-epidermolytic palmoplantar keratoderma with plantar blistering.

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No actively recruiting trials found for Autosomal dominant focal non-epidermolytic palmoplantar keratoderma with plantar blistering at this time.

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No specialists are currently listed for Autosomal dominant focal non-epidermolytic palmoplantar keratoderma with plantar blistering.

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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 Autosomal dominant focal non-epidermolytic palmoplantar keratoderma with plantar blistering.

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Community

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Latest news about Autosomal dominant focal non-epidermolytic palmoplantar keratoderma with plantar blistering

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Questions for your doctor

Bring these to your next appointment

  • Q1.What specific type of palmoplantar keratoderma does my family member have, and how was this determined?,Is genetic testing recommended, and what would the results mean for other family members?,What is the best daily skin care routine to manage the thickening and prevent blisters?,Are there any medications that could help reduce the severity of symptoms?,How should I care for blisters at home, and when should I seek medical attention for them?,What type of footwear or orthotics do you recommend?,Are there any clinical trials or new treatments being studied for this condition?

Common questions about Autosomal dominant focal non-epidermolytic palmoplantar keratoderma with plantar blistering

What is Autosomal dominant focal non-epidermolytic palmoplantar keratoderma with plantar blistering?

Autosomal dominant focal non-epidermolytic palmoplantar keratoderma with plantar blistering is a very rare inherited skin condition that primarily affects the skin on the palms of the hands and the soles of the feet. The term "palmoplantar keratoderma" means thickening of the skin (keratoderma) on the palms (palmo-) and soles (plantar). In this specific form, the thickening occurs in a focal pattern, meaning it develops in certain pressure-bearing areas rather than covering the entire palm or sole. A distinguishing feature of this condition is the development of blisters on the soles of the fe

How is Autosomal dominant focal non-epidermolytic palmoplantar keratoderma with plantar blistering inherited?

Autosomal dominant focal non-epidermolytic palmoplantar keratoderma with plantar blistering follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.