Overview
Acrokeratoelastoidosis of Costa (also called AKE or acrokeratoelastoidosis) is a rare inherited skin condition that mainly affects the hands and feet. The name comes from Greek and Latin words meaning 'hardening of the skin at the extremities with loss of elastic fibers.' It was first described by the dermatologist Costa in 1953. In this condition, the skin develops small, firm, yellowish or skin-colored bumps (called papules) along the edges of the palms and soles, as well as on the fingers and toes. These bumps form because the elastic fibers in the skin break down and the outer layer of skin thickens abnormally. The condition is not life-threatening and does not affect internal organs. It tends to appear in childhood or early adulthood and usually stays stable over time, meaning it does not dramatically worsen. There is no cure, but treatments can help manage the appearance and discomfort of the skin changes. Moisturizers, keratolytic creams (which soften thickened skin), and retinoids (vitamin A-based medicines) are the main options used by dermatologists to help manage symptoms.
Also known as:
Key symptoms:
Small, firm, yellowish or skin-colored bumps on the edges of the palms and solesBumps along the sides of the fingers and toesThickened skin on the hands and feetMild itching or discomfort in affected areasDry, rough skin texture on the palms and solesBumps that may slowly increase in number over timeNo blistering or open soresCosmetic concern due to visible skin changes
Clinical phenotype terms (12)— hover any for plain English
Autosomal dominant
Passed on from just one parent; each child has about a 50% chance of inheriting it
Childhood
Begins in childhood, roughly ages 1 to 12
Treatments
No FDA-approved treatments are currently listed for Acrokeratoelastoidosis of Costa.
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Specialists
View all specialists →No specialists are currently listed for Acrokeratoelastoidosis of Costa.
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 Acrokeratoelastoidosis of Costa.
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Caregiver Resources
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Family & Caregiver Grants
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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.Is my diagnosis confirmed by skin biopsy, and what did the biopsy show?,Should other family members be examined or tested for this condition?,What skin care routine and treatments do you recommend for me specifically?,Are there any clinical trials or research studies I could participate in?,Should I see a clinical geneticist or genetic counselor to understand the inheritance in my family?,What signs should prompt me to come back sooner between scheduled appointments?,Will this condition affect other parts of my body over time?
Common questions about Acrokeratoelastoidosis of Costa
What is Acrokeratoelastoidosis of Costa?
Acrokeratoelastoidosis of Costa (also called AKE or acrokeratoelastoidosis) is a rare inherited skin condition that mainly affects the hands and feet. The name comes from Greek and Latin words meaning 'hardening of the skin at the extremities with loss of elastic fibers.' It was first described by the dermatologist Costa in 1953. In this condition, the skin develops small, firm, yellowish or skin-colored bumps (called papules) along the edges of the palms and soles, as well as on the fingers and toes. These bumps form because the elastic fibers in the skin break down and the outer layer of ski
How is Acrokeratoelastoidosis of Costa inherited?
Acrokeratoelastoidosis of Costa follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Acrokeratoelastoidosis of Costa typically begin?
Typical onset of Acrokeratoelastoidosis of Costa is childhood. Age of onset can vary across affected individuals.