(Q80.9) Congenital ichthyosis, unspecified

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16 951 in individuals diagnosis congenital ichthyosis, unspecified confirmed
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1 607 deaths with diagnosis congenital ichthyosis, unspecified
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10% mortality rate associated with the disease congenital ichthyosis, unspecified

Diagnosis congenital ichthyosis, unspecified is diagnosed Men are 19.34% more likely than Women

10 115

Men receive the diagnosis congenital ichthyosis, unspecified

995 (9.8 %)

Died from this diagnosis.

100
95
90
85
80
75
70
65
60
55
50
45
40
35
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15
10
5
0
6 836

Women receive the diagnosis congenital ichthyosis, unspecified

612 (9.0 %)

Died from this diagnosis.

Risk Group for the Disease congenital ichthyosis, unspecified - Men and Women aged 0

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In Men diagnosis is most often set at age 0-49, 60-64, 70-84
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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+
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In Women diagnosis is most often set at age 0-39, 50-59, 80-84

Disease Features congenital ichthyosis, unspecified

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Absence or low individual and public risk
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Congenital ichthyosis, unspecified - what does this mean

Congenital ichthyosis is a genetic skin disorder that is present at birth and is characterized by dry, thickened, and scaly skin. it is caused by mutations in genes that are responsible for the production of proteins that form the skin's protective barrier.

What happens during the disease - congenital ichthyosis, unspecified

Congenital ichthyosis is a genetic disorder that occurs when the outer layer of skin does not form properly, resulting in thick, dry, scaly skin. the underlying cause of this disorder is an abnormality in the production of proteins that are necessary for the formation of the skin barrier, resulting in an inability to retain adequate moisture and protect the skin from irritants. this leads to the formation of thick, dry, and scaly skin that can be itchy and uncomfortable.

Clinical Pattern

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How does a doctor diagnose

  • Physical examination of the skin
  • Evaluation of family history of skin disorders
  • Laboratory tests, such as a complete blood count and a skin biopsy
  • Genetic testing for mutations in genes associated with ichthyosis
  • Imaging tests, such as an X-ray or MRI scan, to rule out other conditions
Additional measures:
  • Photoprotection measures, such as the use of sunscreen and protective clothing
  • Treatment with topical moisturizers and emollients
  • Treatment with topical retinoids and vitamin D analogs
  • Systemic treatments, such as oral retinoids and immunosuppressants

Treatment and Medical Assistance

Main goal of the treatment: To improve the skin's appearance and reduce discomfort.
  • Regular application of moisturizers
  • Avoiding hot showers and baths
  • Use of topical retinoids or calcipotriol
  • Regular exfoliation with a loofah or scrub
  • Use of topical corticosteroids
  • Oral retinoids such as acitretin
  • Phototherapy with ultraviolet A (UVA) or ultraviolet B (UVB) light
  • Antibiotic creams or ointments
  • Surgery to remove thickened skin
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15 Days of Hospitalization Required
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Average Time for Outpatient Care Not Established

Congenital ichthyosis, unspecified - Prevention

The best way to prevent congenital ichthyosis, unspecified is to ensure that pregnant women are aware of the risks and receive regular prenatal care. women should also avoid smoking, alcohol and drug use during pregnancy, as well as any other potential risk factors that may increase the chance of a baby being born with congenital ichthyosis. additionally, genetic counseling should be considered for couples who have a family history of the condition.