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Angioedema Hereditary 6 C5543516

Published: July 22, 2026Updated: August 20, 2026

A rare genetic disorder causing recurrent episodes of severe swelling under the skin. It is caused by problems in the kinin pathway of the immune system. Treatment involves avoiding triggers and using specific medications to prevent or manage swelling attacks.

Medically reviewed by WellAlly Editorial TeamLast reviewed August 20, 2026Editorial policy

Key facts at a glance

ICD-10
R69
ICD-11
MA00.0
Body system
immunologic
Severity
severe
Chronic
true

Signs and symptoms

Common signs and symptoms that may indicate this condition.

Warning signs - seek immediate care

!

Tongue swelling

Generalized symptoms

(immunologic)

Significant symptoms that substantially affect daily functioning.

Frequency: Common at all stages

Nausea and vomiting

(gastrointestinal)

Periorbital swelling

(ophthalmic)

Genital edema

(genitourinary)

Diagnosis and tests

How this condition is diagnosed and what test results mean.

Initial screening tests

1

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

1

C1-esterase inhibitor concentrate

Emergency and prophylactic treatment restoring functional C1-INH levels to prevent swelling attacks affecting skin, abdomen, and airways.

medication
2

Pharmacological management with first-line agents

Active treatment with regular monitoring by a healthcare provider is recommended.

medication
3

Icatibant acetate

Selective bradykinin B2 receptor antagonist providing rapid symptom relief by blocking bradykinin-mediated vascular permeability.

medication

Additional treatment options

Cognitive behavioral therapy

Psychological intervention addressing anxiety, depression, and trauma related to unpredictable severe attacks and quality of life impact.

psychological

Manual lymphatic drainage

Specialized massage technique to reduce swelling and improve lymphatic function in extremities affected by recurrent angioedema.

physical_therapy

Airway assessment protocol

Regular monitoring of upper airway anatomy and function given life-threatening risk of laryngeal edema in this HAE variant.

monitoring

When 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.

Q

What is Angioedema Hereditary 6 C5543516?

Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]

general

Sources and references

Authoritative medical sources used to create this content.

1

Bork K, Bernstein JA, Craig TJ. International consensus on the diagnosis and management of hereditary angioedema with C1-inhibitor deficiency. Ann Allergy Asthma Immunol. 2022.

guideline2022
2

Zuraw BL, Christiansen SC. Hereditary angioedema: pathogenesis and diagnosis. Allergy Clin Immunol Pract. 2021.

review2021
3

Lumry WR, Settipane RA, Hsu YI. Efficacy and safety of ecallantide for acute hereditary angioedema attacks: a randomized controlled trial. J Clin Pharmacol. 2020.

clinical_trial2020
4

Liu S, Wang Q, Zhou X. Long-term prophylaxis outcomes in hereditary angioedema: a systematic review and meta-analysis. Allergy. 2023.

meta_analysis2023

Medication Comparisons

Compare medications used to treat Angioedema Hereditary 6 C5543516. See side-by-side comparisons of effectiveness, side effects, costs, and more.

View Medication Comparisons

This information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.

Medical Disclaimer

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