Overview
Thost-Unna palmoplantar keratoderma (also called Thost-Unna disease, diffuse nonepidermolytic palmoplantar keratoderma, or NEPPK) is a rare inherited skin condition that causes the skin on the palms of the hands and soles of the feet to become very thick and tough. This thickening, called keratoderma, usually appears in the first few months or years of life and tends to get worse over time. The skin may look yellowish, feel leathery, and have a sharp border where the thickened skin meets normal skin. In some people, the thickening can spread slightly onto the sides of the hands and feet. The condition is caused by changes (mutations) in the KRT1 or KRT16 gene, which provide instructions for making proteins that help keep skin cells strong and connected. When these proteins do not work properly, skin cells build up faster than they should, leading to the characteristic thickening. Thost-Unna keratoderma does not usually affect internal organs, and most people have a normal life expectancy. There is no cure for Thost-Unna keratoderma, but symptoms can be managed. Treatments focus on softening and reducing the thick skin using moisturizers, keratolytic creams (products that help break down thickened skin), and careful physical removal of excess skin. With good daily skin care, many people are able to manage their symptoms and live active lives.
Key symptoms:
Very thick, tough skin on the palms and solesYellowish or ivory-colored skin on affected areasSharp, well-defined border between thick and normal skinSkin that feels leathery or waxy to the touchExcessive sweating of the hands and feet (hyperhidrosis)Unpleasant odor from the hands or feet due to sweatingDifficulty walking or standing for long periods due to thick sole skinPain or discomfort when walkingSkin that may crack or develop painful fissuresMild spread of thickening onto the sides of the hands and feet in some people
Autosomal dominant
Passed on from just one parent; each child has about a 50% chance of inheriting it
Infantile
Begins in infancy, roughly 1 month to 2 years old
Treatments
No FDA-approved treatments are currently listed for Thost-Unna palmoplantar keratoderma.
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Specialists
View all specialists →No specialists are currently listed for Thost-Unna palmoplantar keratoderma.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Thost-Unna palmoplantar keratoderma.
Community
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Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
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 is the best daily skin care routine for managing the thickening?,Are there prescription creams or medications that would help more than over-the-counter products?,What type of footwear or orthotics would you recommend to reduce pain when walking?,Are there any clinical trials or new treatments we should know about?,Should other family members be tested for this condition?,What signs of complications, such as skin infections, should prompt us to seek medical care quickly?
Common questions about Thost-Unna palmoplantar keratoderma
What is Thost-Unna palmoplantar keratoderma?
Thost-Unna palmoplantar keratoderma (also called Thost-Unna disease, diffuse nonepidermolytic palmoplantar keratoderma, or NEPPK) is a rare inherited skin condition that causes the skin on the palms of the hands and soles of the feet to become very thick and tough. This thickening, called keratoderma, usually appears in the first few months or years of life and tends to get worse over time. The skin may look yellowish, feel leathery, and have a sharp border where the thickened skin meets normal skin. In some people, the thickening can spread slightly onto the sides of the hands and feet. The
How is Thost-Unna palmoplantar keratoderma inherited?
Thost-Unna palmoplantar keratoderma follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Thost-Unna palmoplantar keratoderma typically begin?
Typical onset of Thost-Unna palmoplantar keratoderma is infantile. Age of onset can vary across affected individuals.