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(Q80.8) Other congenital ichthyosis
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16 951 in individuals diagnosis other congenital ichthyosis confirmed
1 607 deaths with diagnosis other congenital ichthyosis
10% mortality rate associated with the disease other congenital ichthyosis
Diagnosis other congenital ichthyosis is diagnosed Men are 19.34% more likely than Women
10 115
Men receive the diagnosis other congenital ichthyosis
995 (9.8 %)Died from this diagnosis.
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6 836
Women receive the diagnosis other congenital ichthyosis
612 (9.0 %)Died from this diagnosis.
Risk Group for the Disease other congenital 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 other congenital ichthyosis
Absence or low individual and public risk
Other congenital ichthyosis - what does this mean
Other congenital ichthyosis is a group of rare genetic skin disorders that cause dry, scaly skin from birth. it is caused by a mutation in one of several genes that affect the production of skin proteins and the development of the skin barrier. symptoms vary from mild to severe and can include thick, dry, scaly skin, hair loss, and an increased risk of infection.
What happens during the disease - other congenital ichthyosis
Other congenital ichthyosis is a rare skin disorder caused by a genetic mutation that affects the production of proteins needed for healthy skin growth and development. this mutation results in the buildup of an abnormal type of skin protein that causes the skin to become thick, scaly, and dry. in addition, the abnormal protein can block the skin's ability to sweat, which can lead to overheating and other complications.
Clinical Pattern
Other congenital ichthyosis is a rare skin disorder characterized by thick, dry, and scaly skin, which may appear as large, plate-like scales or small, fish-like scales. It typically begins at birth or in the first few months of life and can affect any area of the body, including the face, arms, and legs. Other symptoms may include itching, redness, and cracking of the skin.
How does a doctor diagnose
- Physical examination, including skin biopsy
- Complete blood count
- Genetic testing
- Imaging studies such as X-ray, MRI, or CT scan
- Liver function tests
- Urine tests
- Lipid profile
- Thyroid function tests
Treatment and Medical Assistance
Main goal of the treatment: To reduce the severity of symptoms and improve the quality of life of people with other congenital ichthyosis.
- Identifying and avoiding triggers that can worsen the symptoms.
- Using moisturizers and creams to keep the skin hydrated.
- Using topical steroids to reduce inflammation and improve skin texture.
- Using topical retinoids to reduce scaling.
- Using antibiotics to treat secondary bacterial infections.
- Using antifungal medications to treat secondary fungal infections.
- Using phototherapy to improve skin texture.
- Using systemic medications such as retinoids, immunosuppressants, and biologics to reduce inflammation and improve skin texture.
- Undergoing surgical procedures to remove thickened skin.
15 Days of Hospitalization Required
Average Time for Outpatient Care Not Established
Other congenital ichthyosis - Prevention
Other congenital ichthyosis is a genetic skin disorder that is not preventable. however, early detection and treatment can help reduce the symptoms and improve the quality of life of those affected. it is important to seek medical advice if any skin or scalp changes are noticed.