Exfoliative ichthyosis

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:289586OMIM:607936Q80.8
Who is this for?
Show terms as
1FDA treatments1Specialists8Treatment centers

Where are you in your journey?

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

Overview

Exfoliative ichthyosis is a rare genetic skin disorder that belongs to a group of conditions called ichthyoses, which are characterized by dry, thickened, and scaly skin. In exfoliative ichthyosis, the skin tends to peel and shed in sheets or flakes, particularly on the palms of the hands and soles of the feet, though other body areas can also be affected. The condition is sometimes also referred to as "peeling skin" type ichthyosis or ichthyosis exfoliativa. The disease is caused by mutations in genes that play a role in maintaining the skin's outer barrier. Because the skin barrier does not function properly, the skin becomes dry, fragile, and prone to peeling. Symptoms are usually present from birth or early infancy. Affected individuals may notice redness, dryness, and superficial peeling of the skin that can worsen with heat, humidity, or friction. The severity can vary from mild peeling limited to the hands and feet to more widespread skin involvement. There is currently no cure for exfoliative ichthyosis. Treatment focuses on managing symptoms and protecting the skin. This typically includes regular use of moisturizers and emollients to keep the skin hydrated, gentle exfoliation, and avoiding triggers that worsen peeling. In some cases, keratolytic agents (creams that help soften and remove thickened skin) may be recommended. With proper skin care, most individuals can manage their symptoms effectively, though the condition is lifelong and requires ongoing attention.

Also known as:

Key symptoms:

Peeling or shedding of skin, especially on palms and solesDry, flaky skinRedness of the skinThickened skin on hands and feetSkin that blisters easilyItching or discomfortSkin sensitivity to heat and frictionCracking of the skinMild scaling on the bodySkin fragility

Inheritance

Autosomal recessive

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

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

Treatments

1 available

Dyural 80-Lm

METHYLPREDNISOLONE ACETATE, LIDOCAINE HYDROCHLORIDE, BUPIVACAINE HYDROCHLORIDE, POVIDINE IODINE, ISOPROPYL ALCOHOL· Advanced Rx Pharmacy of Tennessee, LLC■ Boxed Warning

exfoliative dermatitis

No actively recruiting trials found for Exfoliative ichthyosis at this time.

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

Search ClinicalTrials.gov ↗Join the Exfoliative ichthyosis community →

Specialists

1 foundView all specialists →

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 Exfoliative ichthyosis.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Exfoliative ichthyosisForum →

No community posts yet. Be the first to share your experience with Exfoliative ichthyosis.

Start the conversation →

Latest news about Exfoliative ichthyosis

No recent news articles for Exfoliative ichthyosis.

Follow this condition to be notified when news becomes available.

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 specific gene mutation is causing my (or my child's) condition?,What is the best daily skin care routine to manage symptoms?,Are there any prescription creams or medications that could help?,How can I tell if a skin infection is developing, and when should I seek urgent care?,Are there any activities or environmental factors I should avoid?,Should other family members be tested for this condition?,Are there any clinical trials or new treatments being studied for this condition?

Common questions about Exfoliative ichthyosis

What is Exfoliative ichthyosis?

Exfoliative ichthyosis is a rare genetic skin disorder that belongs to a group of conditions called ichthyoses, which are characterized by dry, thickened, and scaly skin. In exfoliative ichthyosis, the skin tends to peel and shed in sheets or flakes, particularly on the palms of the hands and soles of the feet, though other body areas can also be affected. The condition is sometimes also referred to as "peeling skin" type ichthyosis or ichthyosis exfoliativa. The disease is caused by mutations in genes that play a role in maintaining the skin's outer barrier. Because the skin barrier does not

How is Exfoliative ichthyosis inherited?

Exfoliative ichthyosis follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Exfoliative ichthyosis typically begin?

Typical onset of Exfoliative ichthyosis is neonatal. Age of onset can vary across affected individuals.

Which specialists treat Exfoliative ichthyosis?

1 specialists and care centers treating Exfoliative ichthyosis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.