Palmoplantar porokeratosis of Mantoux

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Overview

Palmoplantar porokeratosis of Mantoux (also called porokeratosis palmaris et plantaris disseminata, or PPPD) is a rare inherited skin disorder that belongs to a group of conditions called porokeratoses. In this condition, the skin develops small, ring-shaped or pit-like lesions — most commonly on the palms of the hands and the soles of the feet, though they can spread to other parts of the body over time. These lesions form because skin cells do not grow and shed in a normal, orderly way. A raised, thread-like border called a 'cornoid lamella' surrounds each lesion, which is a hallmark feature seen under a microscope. The condition usually begins in adolescence or early adulthood and tends to slowly get worse over the years. The lesions can cause discomfort, itching, or a burning sensation, and some people find them cosmetically distressing. In rare cases, long-standing lesions may develop into skin cancer, so regular skin monitoring is important. There is currently no cure for palmoplantar porokeratosis of Mantoux. Treatment focuses on managing symptoms and reducing the appearance of lesions. Options include moisturizing creams, retinoids (vitamin A-based medicines), topical treatments to remove thickened skin, and sun protection. Regular check-ups with a dermatologist are strongly recommended to watch for any changes that might suggest cancer development.

Key symptoms:

Small, ring-shaped or pit-like lesions on the palms and solesRaised, thread-like border around each skin lesionItching or burning sensation in affected areasSkin lesions that may slowly spread to other body areas over timeThickened or rough skin patchesDiscomfort when walking if lesions are on the soles of the feetCosmetically noticeable skin changes on hands and feetPossible development of new lesions during sun exposure or illness

Inheritance

Autosomal dominant

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

Age of Onset

Juvenile

Begins in the teen years

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Palmoplantar porokeratosis of Mantoux.

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No actively recruiting trials found for Palmoplantar porokeratosis of Mantoux at this time.

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No specialists are currently listed for Palmoplantar porokeratosis of Mantoux.

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 Palmoplantar porokeratosis of Mantoux.

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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.How certain are you that this is palmoplantar porokeratosis of Mantoux, and should I have a skin biopsy to confirm?,Should I have genetic testing, and what does a positive result mean for my family members?,What is the risk that my lesions could become cancerous, and how often should I be checked?,What treatments do you recommend for my specific situation, and what are the side effects?,Are there any activities, foods, or environmental factors I should avoid to prevent my condition from getting worse?,Should my children or siblings be examined or tested for this condition?,Are there any clinical trials or new treatments I should know about?

Common questions about Palmoplantar porokeratosis of Mantoux

What is Palmoplantar porokeratosis of Mantoux?

Palmoplantar porokeratosis of Mantoux (also called porokeratosis palmaris et plantaris disseminata, or PPPD) is a rare inherited skin disorder that belongs to a group of conditions called porokeratoses. In this condition, the skin develops small, ring-shaped or pit-like lesions — most commonly on the palms of the hands and the soles of the feet, though they can spread to other parts of the body over time. These lesions form because skin cells do not grow and shed in a normal, orderly way. A raised, thread-like border called a 'cornoid lamella' surrounds each lesion, which is a hallmark feature

How is Palmoplantar porokeratosis of Mantoux inherited?

Palmoplantar porokeratosis of Mantoux follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Palmoplantar porokeratosis of Mantoux typically begin?

Typical onset of Palmoplantar porokeratosis of Mantoux is juvenile. Age of onset can vary across affected individuals.