Amyloidosis cutis dyschromia

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ORPHA:319635OMIM:617920E85.4+L99.0*
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Overview

Amyloidosis cutis dyschromia (ACD) is a very rare inherited skin condition in which abnormal proteins called amyloid build up in the skin. This leads to distinctive changes in skin color, creating a mottled or speckled pattern of dark (hyperpigmented) and light (hypopigmented) patches, often described as looking like freckle-like spots mixed with lighter areas. The condition typically appears in early childhood and mainly affects the arms, legs, and trunk. In addition to the color changes, some patients may experience mild itchiness, and the skin may appear slightly thickened or waxy in affected areas. Small blistering or skin fragility has also been reported in some cases. ACD is caused by mutations in specific genes and follows an autosomal recessive inheritance pattern, meaning a child must inherit a faulty copy of the gene from both parents to develop the condition. The disease has been most commonly reported in families from East Asian and Southeast Asian backgrounds, particularly in Japanese, Chinese, and Thai populations. While the skin changes can be cosmetically concerning, ACD generally does not affect internal organs, which distinguishes it from more serious systemic forms of amyloidosis. There is currently no cure for ACD, and treatment focuses on managing symptoms such as itching and protecting the skin. Dermatologists and clinical geneticists are the main specialists involved in care.

Also known as:

Key symptoms:

Mottled or speckled skin with dark and light patchesFreckle-like dark spots on the skinLight or pale patches of skin mixed with darker areasSkin color changes mainly on arms, legs, and trunkMild itching of affected skinSlightly thickened or waxy-feeling skinSkin changes that spread over timeSmall blisters or skin fragility in some casesShort stature in some patientsSkin changes that begin in early childhood

Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Amyloidosis cutis dyschromia.

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No actively recruiting trials found for Amyloidosis cutis dyschromia at this time.

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No specialists are currently listed for Amyloidosis cutis dyschromia.

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 Amyloidosis cutis dyschromia.

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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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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 for our family to confirm the diagnosis?,Are there any treatments that can slow down or stop the skin changes from spreading?,Should other family members be tested to see if they are carriers?,What can we do to manage itching and protect the skin?,Are there any clinical trials or new research for this condition?,Should we see a counselor or psychologist to help cope with the emotional impact?,Do we need any tests to make sure the amyloid is not affecting internal organs?

Common questions about Amyloidosis cutis dyschromia

What is Amyloidosis cutis dyschromia?

Amyloidosis cutis dyschromia (ACD) is a very rare inherited skin condition in which abnormal proteins called amyloid build up in the skin. This leads to distinctive changes in skin color, creating a mottled or speckled pattern of dark (hyperpigmented) and light (hypopigmented) patches, often described as looking like freckle-like spots mixed with lighter areas. The condition typically appears in early childhood and mainly affects the arms, legs, and trunk. In addition to the color changes, some patients may experience mild itchiness, and the skin may appear slightly thickened or waxy in affect

How is Amyloidosis cutis dyschromia inherited?

Amyloidosis cutis dyschromia follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Amyloidosis cutis dyschromia typically begin?

Typical onset of Amyloidosis cutis dyschromia is childhood. Age of onset can vary across affected individuals.