Overview
Erythrokeratoderma 'en cocardes' is a very rare inherited skin disorder. The name comes from French and means 'erythrokeratoderma in cockades' — referring to the distinctive ring-shaped or target-like red, scaly patches that appear on the skin. It is also sometimes called Erythrokeratoderma figurata variabilis or referred to under the broader group of erythrokeratodermas. In this condition, the skin does not shed and renew itself properly, leading to a buildup of thick, rough, scaly skin (a process called hyperkeratosis) combined with redness (erythema). The patches typically appear in a circular or concentric ring pattern, somewhat like a bullseye or cockade rosette, which is what makes this form distinct from other erythrokeratodermas. These patches most commonly appear on the limbs, trunk, and face. The skin changes are usually present from early childhood or even infancy and tend to be stable or slowly progressive over time. There is currently no cure for this condition. Treatment focuses on managing symptoms, mainly by keeping the skin moisturized and using medications to reduce scaling and thickening. Retinoids (vitamin A-derived medicines) are sometimes used to help control severe skin changes. With proper skin care, most people with this condition can manage their symptoms and live a relatively normal life, though the skin changes are typically lifelong.
Also known as:
Key symptoms:
Red, scaly patches on the skin arranged in ring or bullseye patternsThickened, rough skin (hyperkeratosis) in affected areasSkin patches that may vary in size and shape over timeRedness around or within the scaly patchesPatches commonly found on the arms, legs, trunk, and faceDry, flaky skin that may crack or feel tightItching or discomfort in affected skin areasSkin changes that are usually present from infancy or early childhood
Clinical phenotype terms (5)— hover any for plain English
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 Erythrokeratoderma ''en cocardes''.
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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 Erythrokeratoderma ''en cocardes''.
Community
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Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
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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.What genetic tests should we do to confirm the diagnosis and understand the cause?,What skin care routine do you recommend for daily management?,Are there any medications, such as retinoids, that might help, and what are the risks?,Will this condition get worse over time, or is it likely to stay the same?,Are there any clinical trials or new treatments being studied for this condition?,Should other family members be tested or evaluated?,Can you refer us to a psychologist or support group to help with the emotional impact of this condition?
Common questions about Erythrokeratoderma ''en cocardes''
What is Erythrokeratoderma ''en cocardes''?
Erythrokeratoderma 'en cocardes' is a very rare inherited skin disorder. The name comes from French and means 'erythrokeratoderma in cockades' — referring to the distinctive ring-shaped or target-like red, scaly patches that appear on the skin. It is also sometimes called Erythrokeratoderma figurata variabilis or referred to under the broader group of erythrokeratodermas. In this condition, the skin does not shed and renew itself properly, leading to a buildup of thick, rough, scaly skin (a process called hyperkeratosis) combined with redness (erythema). The patches typically appear in a circ
How is Erythrokeratoderma ''en cocardes'' inherited?
Erythrokeratoderma ''en cocardes'' follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Erythrokeratoderma ''en cocardes'' typically begin?
Typical onset of Erythrokeratoderma ''en cocardes'' is infantile. Age of onset can vary across affected individuals.
Which specialists treat Erythrokeratoderma ''en cocardes''?
2 specialists and care centers treating Erythrokeratoderma ''en cocardes'' are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.