November 8, 2014
Notes: Mattei, P L
Corey, K C
Kimball, A B
J Eur Acad Dermatol Venereol. 2014 Mar;28(3):333-7. doi: 10.1111/jdv.12106. Epub 2013 Feb 21.
Author Address: The Johns Hopkins Hospital, Baltimore, MD, USA.
Reference Type: Journal Article
Record Number: 4582Author: Maurer, M. and Parish, L. C.
Title: The dermatology view of hereditary angio-oedema: practical diagnostic and management considerations
Journal: J Eur Acad Dermatol Venereol
Short Title: The dermatology view of hereditary angio-oedema: practical diagnostic and management considerations
Alternate Journal: Journal of the European Academy of Dermatology and Venereology : JEADV
ISSN: 1468-3083 (Electronic)
Accession Number: 22568407
Keywords: Angioedemas, Hereditary/*diagnosis/*drug therapy/genetics/prevention & control
Complement C1/*antagonists & inhibitors
Complement C1 Inactivator Proteins/therapeutic use
Abstract: Hereditary angio-oedema (HAE) is characterized by recurrent, localized, non-pitting, non-pruritic, non-urticarial oedema. Nearly all patients experience skin swelling as a feature of HAE. There may be painful abdominal attacks, accompanied by nausea and vomiting. The disease is life-threatening should laryngeal oedema occur. HAE results from a deficiency or dysfunction of C1 inhibitor, a plasma protein with an important role in regulating the contact, complement and fibrinolytic systems. Effective management of HAE should include a plan for treatment of attacks, as well as routine and preprocedure prevention. Acute and prophylactic therapy with C1 inhibitor therapy for correcting the underlying deficiency in HAE is a valuable option.
Notes: Maurer, M
Parish, L C