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(Q80.1) X-linked ichthyosis
More details coming soon
16 951 in individuals diagnosis x-linked ichthyosis confirmed
1 607 deaths with diagnosis x-linked ichthyosis
10% mortality rate associated with the disease x-linked ichthyosis
Diagnosis x-linked ichthyosis is diagnosed Men are 19.34% more likely than Women
10 115
Men receive the diagnosis x-linked ichthyosis
995 (9.8 %)Died from this diagnosis.
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6 836
Women receive the diagnosis x-linked ichthyosis
612 (9.0 %)Died from this diagnosis.
Risk Group for the Disease x-linked ichthyosis - Men and Women aged 0
In Men diagnosis is most often set at age 0-49, 60-64, 70-84
Less common in men the disease occurs at Age 50-59, 65-69, 85-95+Less common in women the disease occurs at Age 40-49, 60-79, 85-95+
In Women diagnosis is most often set at age 0-39, 50-59, 80-84
Disease Features x-linked ichthyosis
Absence or low individual and public risk
X-linked ichthyosis - what does this mean
X-linked ichthyosis is an inherited genetic disorder caused by a mutation in the sts gene on the x chromosome. this mutation results in an abnormal production of the enzyme steroid sulfatase, which is responsible for breaking down certain fats in the skin. this leads to an accumulation of these fats, resulting in dry, scaly skin and an increased sensitivity to heat.
What happens during the disease - x-linked ichthyosis
X-linked ichthyosis is caused by mutations in the gene responsible for the production of the enzyme steroid sulfatase (sts). this enzyme is responsible for the breakdown of cholesterol sulfate, which is essential for the formation of the protective outer layer of the skin. without sts, the outer layer of the skin cannot be formed properly, leading to the formation of dry, scaly patches on the skin.
Clinical Pattern
More details coming soon
How does a doctor diagnose
- Physical examination
- Skin biopsy
- Genetic testing for X-linked ichthyosis
- Blood tests to check for electrolyte imbalances
- Urine tests to check for kidney problems
- Imaging tests such as X-ray or MRI scan
- Ultrasound to check for liver or gallbladder abnormalities
Treatment and Medical Assistance
Main goal of the Treatment: To reduce the scaling, dryness, and itching of X-linked ichthyosis.
- Moisturize the skin regularly.
- Use gentle soaps and cleansers.
- Avoid hot water and long baths.
- Use topical creams and ointments to reduce itching and scaling.
- Take oral medications such as retinoids or antihistamines to reduce inflammation.
- Use a humidifier in the home.
- Avoid harsh soaps and detergents.
- Wear loose-fitting clothing.
- Avoid scratching and picking at the skin.
- Use sunscreen to protect the skin from UV radiation.
15 Days of Hospitalization Required
Average Time for Outpatient Care Not Established
X-linked ichthyosis - Prevention
X-linked ichthyosis can be prevented by avoiding exposure to environmental triggers, such as extreme cold and heat, and using moisturizers to keep the skin hydrated. additionally, genetic counseling can help identify carriers of the disease and enable early diagnosis and treatment.