Limb-mammary syndrome

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ORPHA:69085OMIM:603543Q82.4
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2Specialists8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Limb-mammary syndrome (LMS) is a very rare inherited condition that mainly affects the development of the hands, feet, and breasts. It belongs to a group of disorders called ectodermal dysplasias, which involve problems with tissues that form the outer layer of the body during early development — including skin, nails, hair, teeth, sweat glands, and limbs. The condition is also sometimes referred to as LMS or as part of the broader TP63-related disorder spectrum. People with limb-mammary syndrome are typically born with missing or underdeveloped fingers and toes, a condition called ectrodactyly (sometimes called 'split hand/split foot'). The breasts and nipples are often absent or very underdeveloped, and the mammary glands may not function. Some individuals also have cleft palate, sparse or absent eyelashes and eyebrows, dry eyes, and problems with sweat glands. In females, the uterus and other reproductive organs may also be affected. There is currently no cure for limb-mammary syndrome. Treatment focuses on managing individual symptoms. This may include surgery to improve hand and foot function, dental care, eye drops for dry eyes, and hormone therapy or fertility support for those with reproductive organ involvement. A team of specialists is usually needed to provide the best care. With the right support, many people with LMS can lead full and active lives.

Also known as:

Key symptoms:

Missing or fused fingers and/or toes (split hand or split foot appearance)Absent or very underdeveloped breasts and nipplesNon-functioning mammary glands (unable to breastfeed)Cleft palate (opening in the roof of the mouth)Sparse or absent eyelashes and eyebrowsDry eyes due to reduced tear productionAbsent or underdeveloped sweat glands, causing difficulty regulating body temperatureUnderdeveloped or absent uterus in femalesSparse scalp hairNail abnormalities such as thin or absent nailsDental problems including missing or abnormally shaped teeth

Clinical phenotype terms (32)— hover any for plain English
Lacrimal duct atresiaHP:0000564Absent lacrimal punctumHP:0001092Hypoplastic nipplesHP:0002557Absent nippleHP:0002561Bilateral breast hypoplasiaHP:0012814Breast aplasiaHP:0100783BlepharitisHP:0000498Nail dysplasiaHP:0002164Chronic irritative conjunctivitisHP:0007717Submucous cleft soft palateHP:00118193-4 finger cutaneous syndactylyHP:0011939OligodactylyHP:0012165
Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Limb-mammary syndrome.

View clinical trials →

No actively recruiting trials found for Limb-mammary syndrome at this time.

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Search ClinicalTrials.gov ↗Join the Limb-mammary syndrome community →

Specialists

2 foundView all specialists →
CM
Caroline Lilja, MD
Specialist
PI on 1 active trial
Yeliz Bahar Özdemir
Specialist
PI on 2 active trials1 Limb-mammary syndrome publication

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 Limb-mammary syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Limb-mammary 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.Which specific change in the TP63 gene does my child have, and what does that mean for their symptoms and outlook?,What surgeries might help improve hand and foot function, and when is the best time to consider them?,How do we protect my child from overheating, and what warning signs should I watch for?,Will my child's fertility be affected, and are there options to preserve it?,Are there other family members who should be tested for this condition?,What therapies — such as occupational or physical therapy — would help most right now?,Are there any clinical trials or research studies we should know about?

Common questions about Limb-mammary syndrome

What is Limb-mammary syndrome?

Limb-mammary syndrome (LMS) is a very rare inherited condition that mainly affects the development of the hands, feet, and breasts. It belongs to a group of disorders called ectodermal dysplasias, which involve problems with tissues that form the outer layer of the body during early development — including skin, nails, hair, teeth, sweat glands, and limbs. The condition is also sometimes referred to as LMS or as part of the broader TP63-related disorder spectrum. People with limb-mammary syndrome are typically born with missing or underdeveloped fingers and toes, a condition called ectrodacty

How is Limb-mammary syndrome inherited?

Limb-mammary syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Limb-mammary syndrome typically begin?

Typical onset of Limb-mammary syndrome is neonatal. Age of onset can vary across affected individuals.

Which specialists treat Limb-mammary syndrome?

2 specialists and care centers treating Limb-mammary syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.