Overview
Dyschromatosis symmetrica hereditaria (DSH), also known as reticulate acropigmentation of Dohi, is a rare inherited skin condition that causes patches of both darker and lighter skin to appear on the backs of the hands and feet. The name describes what you see: 'dyschromatosis' means abnormal coloring, 'symmetrica' means it appears on both sides of the body equally, and 'hereditaria' means it is passed down through families. The skin changes usually appear in early childhood and tend to stabilize by the time a person reaches adulthood. The face may also show small freckle-like spots, while the arms and legs are generally not affected. The condition is caused by changes (mutations) in a gene called ADAR1, which plays a role in how the body processes genetic information inside cells. DSH does not affect internal organs, and most people with this condition live a completely normal, healthy life. The main concern is cosmetic — the uneven skin coloring can sometimes cause emotional distress or self-consciousness, especially in children and teenagers. There is no cure for DSH, and treatment is focused on managing appearance. Options include camouflage makeup, sun protection to prevent worsening of the pigment changes, and in some cases laser therapy. Because the condition is benign and does not shorten lifespan, the main goal of care is supporting the emotional well-being of affected individuals.
Also known as:
Key symptoms:
Mixed patches of darker (hyperpigmented) and lighter (hypopigmented) skin on the backs of the hands and feetFreckle-like spots on the face, especially the cheeksSkin color changes that appear symmetrically on both sides of the bodySkin changes that first appear in infancy or early childhoodGradual spreading of skin patches during childhood that stabilizes in adulthoodNo pain or itching associated with the skin patchesNormal skin texture — the patches are only changes in color, not raised or rough
Clinical phenotype terms (4)— 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 Dyschromatosis symmetrica hereditaria.
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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 Dyschromatosis symmetrica hereditaria.
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.Is genetic testing recommended to confirm the diagnosis, and should other family members be tested?,What sun protection measures are most important for my child, and will sun exposure make the skin changes worse?,Are there any laser or cosmetic treatments that might help, and what results can realistically be expected?,Will the skin patches spread or change as my child gets older?,Are there any other health conditions associated with DSH that I should watch for?,Where can I find support groups or resources for families affected by rare skin conditions?,What should I tell my child's school or teachers about this condition?
Common questions about Dyschromatosis symmetrica hereditaria
What is Dyschromatosis symmetrica hereditaria?
Dyschromatosis symmetrica hereditaria (DSH), also known as reticulate acropigmentation of Dohi, is a rare inherited skin condition that causes patches of both darker and lighter skin to appear on the backs of the hands and feet. The name describes what you see: 'dyschromatosis' means abnormal coloring, 'symmetrica' means it appears on both sides of the body equally, and 'hereditaria' means it is passed down through families. The skin changes usually appear in early childhood and tend to stabilize by the time a person reaches adulthood. The face may also show small freckle-like spots, while the
How is Dyschromatosis symmetrica hereditaria inherited?
Dyschromatosis symmetrica hereditaria follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Dyschromatosis symmetrica hereditaria typically begin?
Typical onset of Dyschromatosis symmetrica hereditaria is childhood. Age of onset can vary across affected individuals.
Which specialists treat Dyschromatosis symmetrica hereditaria?
15 specialists and care centers treating Dyschromatosis symmetrica hereditaria are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.