Type IV Ehlers-Danlos Syndrome

A subtype of Ehlers-Danlos syndrome (EDS) characterized by vascular pathologies, e.g., AORTIC DISSECTION in addition to common EDS findings, e.g., hyperextensible skin and joints, skin fragility and reduced wound healing capability. It is associated with mutations in collagen type III alpha 1 chain gene (COLLAGEN TYPE III).

Also Known As:

Ehlers-Danlos Syndrome, Type IV; EDS IV; Ehlers Danlos Syndrome Type 4, Autosomal Dominant; Ehlers Danlos Syndrome, Arterial Type; Ehlers Danlos Syndrome, Ecchymotic Type; Ehlers Danlos Syndrome, Sack-Barabas Type; Ehlers-Danlos Syndrome, Arterial Type; Ehlers-Danlos Syndrome, Ecchymotic Type; Ehlers-Danlos Syndrome, Sack-Barabas Type; Ehlers-Danlos Syndrome, Type IV, Autosomal Dominant; Ehlers-Danlos Syndrome, Vascular Type; Ehlers Danlos Syndrome, Sack Barabas Type; Ehlers-Danlos Syndrome, Vascular; Ehlers-Danlos Syndromes, Vascular; Syndrome, Vascular Ehlers-Danlos; Syndromes, Vascular Ehlers-Danlos; Type IV Ehlers Danlos Syndrome; Vascular Ehlers Danlos Syndrome; Vascular Ehlers-Danlos Syndromes; Vascular Ehlers-Danlos Syndrome; Vascular Type Ehlers-Danlos Syndrome

Cardiovascular Diseases: 91362
- Vascular Diseases: 15138
  - Aneurysm: 24506
    - Blood Vessel Dissection: 480
      - Aortic Dissection: 5412
        Type IV Ehlers-Danlos Syndrome: 267
  - Hemostatic Disorders: 250
    - Ehlers-Danlos Syndrome: 799
      - Type IV Ehlers-Danlos Syndrome: 267
Hemic and Lymphatic Diseases: 6
- Hematologic Diseases: 3366
  - Hemorrhagic Disorders: 702
    - Hemostatic Disorders: 250
      - Ehlers-Danlos Syndrome: 799
        Type IV Ehlers-Danlos Syndrome: 267
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
- Congenital Abnormalities: 25723
  - Skin Abnormalities: 159
    - Ehlers-Danlos Syndrome: 799
      - Type IV Ehlers-Danlos Syndrome: 267
- Inborn Genetic Diseases: 11939
  - Genetic Skin Diseases: 47
    - Ehlers-Danlos Syndrome: 799
      - Type IV Ehlers-Danlos Syndrome: 267
Skin and Connective Tissue Diseases
- Skin Diseases: 13127
  - Skin Abnormalities: 159
    - Ehlers-Danlos Syndrome: 799
      - Type IV Ehlers-Danlos Syndrome: 267
  - Genetic Skin Diseases: 47
    - Ehlers-Danlos Syndrome: 799
      - Type IV Ehlers-Danlos Syndrome: 267
- Connective Tissue Diseases: 3269
  - Collagen Diseases: 754
    - Ehlers-Danlos Syndrome: 799
      - Type IV Ehlers-Danlos Syndrome: 267

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