Ehlers Danlos Syndrome Dominant Type 4 C0268339
A genetic connective tissue disorder affecting blood vessels and organs, causing fragile skin, easy bruising, and risk of organ rupture. Primarily impacts those with a family history of the condition. Management involves lifelong monitoring and avoiding activities that increase injury or rupture risk.
Key facts at a glance
- ICD-10
- Q24.9
- ICD-11
- LA00.0
- Body system
- cardiovascular
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Chest pain
Chest discomfort or palpitations
(cardiovascular)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Dyspnea
(cardiovascular)Easy bruising
(cardiovascular)Palpitations
(cardiovascular)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Cardiovascular Screening and Surveillance
Regular echocardiograms, CT angiography, or MR angiography to monitor for arterial fragility, aneurysm formation, and other vascular complications characteristic of vascular EDS.
Disease management and monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Arterial Protection Protocol
Comprehensive lifestyle modifications including blood pressure monitoring, avoidance of contact sports and heavy lifting, and strenuous activities that increase vascular stress.
Additional treatment options
Vascular Intervention Planning
Elective surgical repair of aneurysms or arterial complications when indicated, performed by experienced surgical teams familiar with tissue fragility.
surgicalBlood Pressure Management
Careful blood pressure control using medications that minimize arterial stress while maintaining adequate perfusion, typically involving beta-blockers or similar agents.
medicationGenetic Counseling and Family Support
Genetic counseling for patients and families to understand inheritance patterns, implications for family members, and available reproductive options.
psychologicalWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Ehlers Danlos Syndrome Dominant Type 4 C0268339?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
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This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy