Erythroderma desquamativum

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ORPHA:314OMIM:609536L21.1
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4Specialists8Treatment centers

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

Erythroderma desquamativum, also known as Leiner's disease or Leiner-Moussous disease, is a rare and serious skin condition that mainly affects newborns and very young infants. The name describes what happens: 'erythroderma' means the entire skin turns red, and 'desquamativum' refers to heavy peeling or shedding of the skin. Babies with this condition develop widespread redness covering most or all of their body, along with thick, greasy scales that peel off in large sheets. The skin looks similar to severe seborrheic dermatitis (sometimes called 'cradle cap'), but it is much more extensive and serious. Beyond the skin, affected infants often experience diarrhea, failure to gain weight properly, and a weakened immune system that makes them vulnerable to serious infections. Some cases are linked to problems with the complement system, which is a part of the body's immune defenses. Because the skin barrier is so damaged, babies can lose fluids and proteins rapidly, which can become life-threatening. Treatment focuses on supporting the baby's nutrition, preventing and treating infections, and caring for the skin. With prompt and careful medical management, many infants improve over time, though the condition requires close monitoring in a hospital setting, especially in the early stages.

Also known as:

Key symptoms:

Widespread redness covering most or all of the skinThick, greasy, or waxy scales peeling from the skinSkin that sheds in large flakes or sheetsPersistent diarrheaPoor weight gain or failure to thriveFrequent or severe infectionsSwollen lymph nodesLow energy and poor feedingFluid loss through damaged skinIrritability and discomfort

Clinical phenotype terms (4)— hover any for plain English
Seborrheic dermatitisHP:0001051
Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

1 event
Nov 2021First-in-Human Evaluation of an Astrocytic Glutamate Transporter (EAAT2) PET Tracer in Dementia

David Wilson — PHASE1

TrialRECRUITING

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

Treatments

No FDA-approved treatments are currently listed for Erythroderma desquamativum.

View clinical trials →

No actively recruiting trials found for Erythroderma desquamativum at this time.

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

Search ClinicalTrials.gov ↗Join the Erythroderma desquamativum community →

Specialists

4 foundView all specialists →
JP
Joanne M Fraser, PhD
HUNTINGTON, NY
Specialist
PI on 1 active trial
JP
Johanna Czamanski-Cohen, PhD
Haifa
Specialist

Rare Disease Specialist

DP
David Wilson, MD, PhD
San Francisco, California
Specialist

Rare Disease Specialist

FP
Fang Yu, PhD
Phoenix, Arizona
Specialist

Rare Disease Specialist

PI on 2 active trials

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 Erythroderma desquamativum.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Erythroderma desquamativum

Disease timeline:

New trial: A SMART Trial of Adaptive Exercises to Optimize Aerobic-Fitness Responses

Phase NA trial recruiting. Moderate Intensity Continuous Training (MICT)

New trial: A Study to Evaluate KarXT as a Treatment for Psychosis Associated With Alzheimer's Disease (ADEPT-4)

Phase PHASE3 trial recruiting. KarXT

New trial: First-in-Human Evaluation of an Astrocytic Glutamate Transporter (EAAT2) PET Tracer in Dementia

Phase PHASE1 trial recruiting. [18F]RP-115 PET/MRI or PET/CT and MRI

New trial: Developing an Artificial Intelligence System to Detect Cognitive Impairment

Phase NA trial recruiting.

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 is causing my baby's skin condition, and has an underlying immune problem been found?,What tests do you recommend to find the root cause, including genetic or immune testing?,What skin care routine should I follow at home, and which products are safe to use?,How will we know if my baby is getting better or worse, and what signs should prompt an emergency visit?,Does my baby need any special vaccinations or precautions to prevent infections?,Are there any specialists or centers with experience in this rare condition that you would recommend?,What is the long-term outlook for my baby, and will this condition affect their development?

Common questions about Erythroderma desquamativum

What is Erythroderma desquamativum?

Erythroderma desquamativum, also known as Leiner's disease or Leiner-Moussous disease, is a rare and serious skin condition that mainly affects newborns and very young infants. The name describes what happens: 'erythroderma' means the entire skin turns red, and 'desquamativum' refers to heavy peeling or shedding of the skin. Babies with this condition develop widespread redness covering most or all of their body, along with thick, greasy scales that peel off in large sheets. The skin looks similar to severe seborrheic dermatitis (sometimes called 'cradle cap'), but it is much more extensive an

At what age does Erythroderma desquamativum typically begin?

Typical onset of Erythroderma desquamativum is neonatal. Age of onset can vary across affected individuals.

Which specialists treat Erythroderma desquamativum?

4 specialists and care centers treating Erythroderma desquamativum are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.