Overview
Anonychia with flexural pigmentation is a very rare inherited skin condition. The name describes its two main features: 'anonychia' means being born without fingernails or toenails (or having very underdeveloped nails), and 'flexural pigmentation' refers to dark patches of skin that appear in the body's fold areas — such as the armpits, groin, and behind the knees. This condition is sometimes referred to simply by its Orphanet code or grouped under congenital nail disorders. People with this condition are typically born with absent or severely underdeveloped nails on some or all fingers and toes. The darkened skin patches in the skin folds usually become noticeable in early childhood or infancy. Beyond these two main features, the condition does not appear to affect internal organs or cause intellectual disability, meaning most people can live full and healthy lives. There is currently no cure for anonychia with flexural pigmentation. Treatment focuses on managing symptoms — for example, protecting the fingertips where nails are absent and using cosmetic or dermatological approaches for the skin pigmentation. Because this condition is so rare, most of what is known comes from a very small number of reported cases in the medical literature.
Key symptoms:
Absent or severely underdeveloped fingernailsAbsent or severely underdeveloped toenailsDark patches of skin in body fold areas (armpits, groin, behind knees)Sensitive or exposed fingertips due to missing nailsPossible skin thickening in affected fold areas
Clinical phenotype terms (13)— hover any for plain English
Autosomal recessive
Passed on when both parents carry the same gene change; often skips generations
Neonatal
Begins at or shortly after birth (first 4 weeks)
Treatments
No FDA-approved treatments are currently listed for Anonychia with flexural pigmentation.
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Specialists
View all specialists →No specialists are currently listed for Anonychia with flexural pigmentation.
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 Anonychia with flexural pigmentation.
Community
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Caregiver Resources
NORD Caregiver Resources
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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 find the cause?,Are there any other health problems we should watch for with this condition?,What can we do to protect my child's fingertips where nails are missing?,Are there any treatments to reduce the dark skin patches in the fold areas?,What is the chance that other family members or future children could be affected?,Should we see any other specialists, such as a psychologist or occupational therapist?,Are there any research studies or registries we can join to help advance understanding of this condition?
Common questions about Anonychia with flexural pigmentation
What is Anonychia with flexural pigmentation?
Anonychia with flexural pigmentation is a very rare inherited skin condition. The name describes its two main features: 'anonychia' means being born without fingernails or toenails (or having very underdeveloped nails), and 'flexural pigmentation' refers to dark patches of skin that appear in the body's fold areas — such as the armpits, groin, and behind the knees. This condition is sometimes referred to simply by its Orphanet code or grouped under congenital nail disorders. People with this condition are typically born with absent or severely underdeveloped nails on some or all fingers and t
How is Anonychia with flexural pigmentation inherited?
Anonychia with flexural pigmentation follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Anonychia with flexural pigmentation typically begin?
Typical onset of Anonychia with flexural pigmentation is neonatal. Age of onset can vary across affected individuals.