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(Q80.2) Lamellar ichthyosis
More details coming soon
16 951 in individuals diagnosis lamellar ichthyosis confirmed
1 607 deaths with diagnosis lamellar ichthyosis
10% mortality rate associated with the disease lamellar ichthyosis
Diagnosis lamellar ichthyosis is diagnosed Men are 19.34% more likely than Women
10 115
Men receive the diagnosis lamellar ichthyosis
995 (9.8 %)Died from this diagnosis.
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6 836
Women receive the diagnosis lamellar ichthyosis
612 (9.0 %)Died from this diagnosis.
Risk Group for the Disease lamellar 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 lamellar ichthyosis
Absence or low individual and public risk
Lamellar ichthyosis - what does this mean
Lamellar ichthyosis is a rare genetic skin disorder caused by a mutation in the tgm1 gene. it is characterized by thick, dry, scaly skin that forms hard, plate-like scales on the body. it usually appears at birth and is caused by a lack of the skin's natural oils. it can cause itching, cracking, and infection.
What happens during the disease - lamellar ichthyosis
Lamellar ichthyosis is a rare genetic disorder caused by a mutation in the tgm1 gene. this gene encodes for the enzyme transglutaminase-1, which is responsible for cross-linking proteins in the outermost layer of the skin. when the tgm1 gene is mutated, the outermost layer of skin does not form properly, resulting in the formation of thick, dry, and scaly patches of skin. this condition is usually present at birth and can be associated with other complications such as eye and ear infections, respiratory problems, and skin infections.
Clinical Pattern
More details coming soon
How does a doctor diagnose
- Physical Examination
- Skin Biopsy
- Blood Tests
- Genetic Testing
- Ultrasound
- CT Scan
- MRI
Treatment and Medical Assistance
Main Goal of the Treatment: To reduce symptoms and improve quality of life for those with Lamellar Ichthyosis.
- Moisturizing the skin with emollients several times a day
- Using topical medications to reduce itching and inflammation
- Applying topical retinoids to help reduce scaling
- Using topical antibiotics to reduce the risk of infection
- Using oral antibiotics to reduce the risk of infection
- Using systemic retinoids to help reduce scaling
- Using immunomodulators to help reduce inflammation
- Using systemic anti-inflammatory medications to reduce inflammation
- Using phototherapy to help reduce scaling
- Using laser therapy to help reduce scaling
- Using dietary supplements to help reduce inflammation
15 Days of Hospitalization Required
Average Time for Outpatient Care Not Established
Lamellar ichthyosis - Prevention
Lamellar ichthyosis is a genetic disorder that affects the skin, and there is currently no cure. however, preventive measures can be taken to reduce the risk of developing this condition. these include avoiding smoking, maintaining a healthy diet, using sunscreen, and avoiding excessive exposure to the sun. additionally, regular medical check-ups and genetic testing can help identify any potential risks and provide early intervention if necessary.