Hypo- and hypermelanotic cutaneous macules-retarded growth-intellectual disability syndrome

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ORPHA:2435L81.8
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Overview

Hypo- and hypermelanotic cutaneous macules-retarded growth-intellectual disability syndrome (also sometimes referred to by its Orphanet code ORPHA:2435) is a very rare condition that affects the skin, growth, and brain development. The name describes its three main features: unusual skin spots, slower-than-normal growth, and intellectual disability. The skin spots can appear either lighter (hypomelanotic) or darker (hypermelanotic) than the surrounding skin, meaning the skin cells either make too little or too much of the pigment called melanin in certain areas. These patches are usually harmless on their own but are an important clue for diagnosis. Children with this syndrome typically grow more slowly than their peers and may be shorter than expected for their age. Intellectual disability means that learning, reasoning, and daily problem-solving skills develop more slowly and may remain limited throughout life. The degree of intellectual disability can vary from mild to more significant. Some individuals may also have other features affecting the nervous system or other body systems, though the condition is so rare that the full picture is not yet completely understood. There is currently no cure for this syndrome. Care focuses on supporting development through early intervention programs, special education, speech therapy, occupational therapy, and physical therapy. Regular monitoring by a team of specialists helps manage symptoms and improve quality of life. Genetic counseling is recommended for affected families.

Also known as:

Key symptoms:

Light-colored (hypopigmented) skin patchesDark-colored (hyperpigmented) skin patchesSlower growth and shorter height than peersIntellectual disability (difficulty with learning and reasoning)Delayed speech and language developmentDelayed motor milestones such as sitting or walking later than usualPossible behavioral challenges

Clinical phenotype terms (8)— hover any for plain English
Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Infantile

Begins in infancy, roughly 1 month to 2 years old

Orphanet ↗NORD ↗

FDA & Trial Timeline

1 event
Dec 1991

Triostat: FDA approved

Treatment of myxedema coma/precoma.

FDAcompleted

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

1 available

Triostat

Liothyronine sodium injection· SmithKline Beecham PharmaceuticalsOrphan Drug

Treatment of myxedema coma/precoma.

No actively recruiting trials found for Hypo- and hypermelanotic cutaneous macules-retarded growth-intellectual disability syndrome at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Hypo- and hypermelanotic cutaneous macules-retarded growth-intellectual disability syndrome community →

No specialists are currently listed for Hypo- and hypermelanotic cutaneous macules-retarded growth-intellectual disability syndrome.

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 Hypo- and hypermelanotic cutaneous macules-retarded growth-intellectual disability syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Hypo- and hypermelanotic cutaneous macules-retarded growth-intellectual disability syndrome

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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.What genetic tests should we do to find the cause of my child's condition?,What therapies (speech, physical, occupational) should we start right away, and how do we access them?,Should my child see an endocrinologist about their growth, and could growth hormone therapy help?,How often should the skin spots be checked, and are there any warning signs we should watch for?,What level of independence might my child achieve as an adult, and what support services are available?,Is there a risk that other family members or future children could be affected?,Are there any research studies or registries for this condition that we could participate in?

Common questions about Hypo- and hypermelanotic cutaneous macules-retarded growth-intellectual disability syndrome

What is Hypo- and hypermelanotic cutaneous macules-retarded growth-intellectual disability syndrome?

Hypo- and hypermelanotic cutaneous macules-retarded growth-intellectual disability syndrome (also sometimes referred to by its Orphanet code ORPHA:2435) is a very rare condition that affects the skin, growth, and brain development. The name describes its three main features: unusual skin spots, slower-than-normal growth, and intellectual disability. The skin spots can appear either lighter (hypomelanotic) or darker (hypermelanotic) than the surrounding skin, meaning the skin cells either make too little or too much of the pigment called melanin in certain areas. These patches are usually harml

At what age does Hypo- and hypermelanotic cutaneous macules-retarded growth-intellectual disability syndrome typically begin?

Typical onset of Hypo- and hypermelanotic cutaneous macules-retarded growth-intellectual disability syndrome is infantile. Age of onset can vary across affected individuals.

What treatment and support options exist for Hypo- and hypermelanotic cutaneous macules-retarded growth-intellectual disability syndrome?

1 patient support program are currently tracked on UniteRare for Hypo- and hypermelanotic cutaneous macules-retarded growth-intellectual disability syndrome. See the treatments and support programs sections for copay assistance, eligibility, and contact details.