Dermatologic Manifestations of Hereditary Angioedema Medication

  • Author: Warren R Heymann, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jan 6, 2012
 

Medication Summary

The goals of pharmacotherapy for hereditary angioedema (HAE) are to reduce morbidity and to prevent complications.

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Antigonadotropic agents

Class Summary

These agents may be used at doses that prevent attacks.

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Danazol (Danocrine)

 

Increases levels of C4 component of complement and reduces attacks associated with angioedema. In HAE, danazol increases level of deficient C1 esterase inhibitor.

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Antifibrinolytic agents

Class Summary

Act through the inhibition of plasmin.

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Epsilon-aminocaproic acid (Amicar)

 

Lysine analog that inhibits fibrinolysis via inhibition of plasminogen activator substances and, to a lesser degree, through antiplasmin activity.

Widely distributed. Half-life is 1-2 h. Peak effect occurs within 2 h. Hepatic metabolism is minimal. Can be used PO/IV.

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Tranexamic acid (Cyklokapron)

 

Alternative to aminocaproic acid. Inhibits fibrinolysis by displacing plasminogen from fibrin.

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Serine Proteinase Inhibitors (serpins)

Class Summary

Used for routine prophylaxis against angioedema attacks and for treatment of acute attacks.

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C1 inhibitor, human (Cinryze)

 

C1 inhibitor is a normal constituent of human blood and is one of the serine proteinase inhibitors (serpins). Regulates activation of pathways for complement and intrinsic coagulation. Also regulates fibrinolytic system. Available as a sterile, lyophilized preparation derived from human plasma. Specific activity is 4-9 U/mg protein. One unit corresponds to the mean quantity of C1 inhibitor present in 1 mL of normal fresh plasma. Indicated for routine prophylaxis against angioedema attacks in adolescents and adults with hereditary angioedema

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C1 esterase inhibitor, human (Berinert)

 

Serine proteinase inhibitor found in human blood that regulates activation of the complement pathway, intrinsic coagulation system, and fibrinolytic system. Binds to and neutralizes substrates that activate these systems, thereby suppressing activity. Available as a pasteurized, lyophilized preparation derived from purified human plasma. One unit corresponds to the mean quantity of C1 inhibitor present in 1 mL of normal fresh plasma. Indicated for acute laryngeal, abdominal, and facial angioedema attacks in adolescents and adults with HAE.

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Kallikrein Inhibitor

Class Summary

Elicits specific kallikrein inhibitor activity resulting in bradykinin reduction. Useful for treating acute episodic attacks.

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Ecallantide (Kalbitor)

 

Human plasma kallikrein inhibitor. Binds to plasma kallikrein and blocks its binding site. Reduces conversion of kininogen to bradykinin. Indicated for acute attacks of hereditary angioedema. Available as injectable solution; 10 mg/mL per single-use vial.

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Bradykinin Receptor Antagonists

Class Summary

Bradykinin receptor antagonists such as icatibant inhibit bradykinin from binding the B2 receptor and thereby treat the clinical symptoms of an acute attack. Recommended dose of icatibant is 30 mg SC in the abdominal area. It is available as a single-use, prefilled syringe, which delivers a dose of 30 mg (10 mg/mL).

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Icatibant (Firazyr)

 

Bradykinin B2 receptor antagonist indicated for acute attacks of hereditary angioedema (HAE).

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Contributor Information and Disclosures
Author

Warren R Heymann, MD  Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Amanda T Moon, MD  Resident Physician, Department of Dermatology, University of Rochester, Strong Memorial Hospital

Amanda T Moon, MD, is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Medical Student Association/Foundation, and Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Paul Krusinski, MD  Director of Dermatology, Fletcher Allen Health Care; Professor, Department of Internal Medicine, University of Vermont College of Medicine

Paul Krusinski, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Kathleen M. Rossy, MD, to the development and writing of this article.

References

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