Osteosclerosis-ichthyosis-premature ovarian failure syndrome

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ORPHA:75325OMIM:609993M85.8
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Overview

Osteosclerosis-ichthyosis-premature ovarian failure syndrome is an extremely rare condition that affects multiple parts of the body at the same time. The name describes its three main features: osteosclerosis (bones that become abnormally dense and hard), ichthyosis (dry, scaly, thickened skin), and premature ovarian failure (when the ovaries stop working normally before age 40, leading to early menopause and fertility problems in women). This condition is sometimes referred to by the abbreviation OIPOF syndrome. The bone changes in this syndrome mean that the skeleton becomes overly dense, which sounds like it might make bones stronger, but it can actually cause problems including pain, increased fracture risk in some areas, and pressure on nerves. The skin in people with this condition tends to be rough, scaly, and thickened, similar to other forms of ichthyosis. Women with this syndrome often experience irregular or absent periods at a young age, reduced fertility, and symptoms of early menopause such as hot flashes. Because this syndrome is so rare, treatment focuses on managing each symptom separately rather than treating the underlying cause. Skin care routines, hormone replacement therapy for ovarian failure, and monitoring of bone health are the main approaches used. There is currently no cure, and care is provided by a team of specialists working together.

Also known as:

Key symptoms:

Abnormally dense, hardened bones (osteosclerosis)Dry, scaly, thickened skin (ichthyosis)Ovaries stopping work before age 40 (premature ovarian failure)Irregular or absent menstrual periods in young womenReduced or absent fertility in womenEarly menopause symptoms such as hot flashes and night sweatsBone pain or discomfortPossible nerve compression due to dense bonesSkin that may crack or become inflamed

Clinical phenotype terms (4)— hover any for plain English
Premature ovarian insufficiencyHP:0008209Increased bone mineral densityHP:0011001
Inheritance

Autosomal recessive

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

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Osteosclerosis-ichthyosis-premature ovarian failure syndrome.

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No actively recruiting trials found for Osteosclerosis-ichthyosis-premature ovarian failure syndrome at this time.

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No specialists are currently listed for Osteosclerosis-ichthyosis-premature ovarian failure 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 Osteosclerosis-ichthyosis-premature ovarian failure syndrome.

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Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.What genetic tests should I have to find the cause of my condition?,What hormone replacement therapy options are available for premature ovarian failure, and what are the risks and benefits?,How often should I have bone density scans, and what should I do to protect my bones?,Are there any fertility preservation options I should consider?,What skin care products or treatments do you recommend for my type of ichthyosis?,Are there any clinical trials or research studies I could participate in?,Should other family members be tested or screened for this condition?

Common questions about Osteosclerosis-ichthyosis-premature ovarian failure syndrome

What is Osteosclerosis-ichthyosis-premature ovarian failure syndrome?

Osteosclerosis-ichthyosis-premature ovarian failure syndrome is an extremely rare condition that affects multiple parts of the body at the same time. The name describes its three main features: osteosclerosis (bones that become abnormally dense and hard), ichthyosis (dry, scaly, thickened skin), and premature ovarian failure (when the ovaries stop working normally before age 40, leading to early menopause and fertility problems in women). This condition is sometimes referred to by the abbreviation OIPOF syndrome. The bone changes in this syndrome mean that the skeleton becomes overly dense, w

How is Osteosclerosis-ichthyosis-premature ovarian failure syndrome inherited?

Osteosclerosis-ichthyosis-premature ovarian failure syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.