(Q80) Congenital ichthyosis

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

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

10 115

Men receive the diagnosis congenital ichthyosis

995 (9.8 %)

Died from this diagnosis.

100
95
90
85
80
75
70
65
60
55
50
45
40
35
30
25
20
15
10
5
0
6 836

Women receive the diagnosis congenital ichthyosis

612 (9.0 %)

Died from this diagnosis.

Risk Group for the Disease congenital ichthyosis - 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

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Non-contagious
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Unspecified
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Absence or low individual and public risk

Congenital ichthyosis - what does this mean

Congenital ichthyosis is a rare skin disorder that is present at birth and is caused by a genetic mutation that affects the skin's ability to retain moisture, resulting in dry, scaly skin.

What happens during the disease - congenital ichthyosis

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Clinical Pattern

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How does a doctor diagnose

  • Physical examination
  • Medical history review
  • Skin biopsy
  • Genetic testing
  • Blood tests
  • Imaging tests
Additions:
  • Urine tests
  • Allergy testing

Treatment and Medical Assistance

Main goal of the treatment: To reduce the symptoms of Congenital Ichthyosis and improve the patient's skin condition.
  • Prescribe moisturizing creams and ointments.
  • Prescribe topical retinoids.
  • Prescribe antibiotics to treat secondary skin infections.
  • Prescribe systemic retinoids.
  • Prescribe topical calcineurin inhibitors.
  • Perform physical therapy to prevent skin contractures.
  • Perform skin grafts to improve skin appearance.
  • Provide emotional support to the patient and their family.
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15 Days of Hospitalization Required
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Average Time for Outpatient Care Not Established

Congenital ichthyosis - Prevention

Congenital ichthyosis is a rare skin condition that is often genetic in origin. prevention of this condition is not possible, however, early detection and management of the symptoms can help to reduce the severity of the condition. it is important for parents to be aware of the signs and symptoms of congenital ichthyosis so that they can seek appropriate medical care for their child if necessary.

Specified forms of the disease

(Q18.0) Sinus, fistula and cyst of branchial cleft
(Q18.1) Preauricular sinus and cyst
(Q18.2) Other branchial cleft malformations
(Q18.3) Webbing of neck
(Q18.4) Macrostomia
(Q18.5) Microstomia
(Q18.6) Macrocheilia
(Q18.7) Microcheilia
(Q18.8) Other specified congenital malformations of face and neck
(Q18.9) Congenital malformation of face and neck, unspecified