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Allergy Asthma Clin Immunol 2018 4;14:24. Epub 2018 Jun 4.

Western Springs Asthma and Allergy, Western Spring, IL USA.

Background: Angioedema secondary to acquired C1 inhibitor deficiency (AAE) is a rare disease. It usually is associated with lymphoproliferative disorders. We present a case of AAE in a patient with antiphospholipid syndrome (APS), a non-Hodgkin lymphoproliferative disorder (NHL) with undetectable levels of C2, C4, and an undetectable CH50. Read More

Rev Med Interne 2018 May 4. Epub 2018 May 4.

Service de dermatologie, centre hospitalier Le Mans, 194, avenue Rubillard, 72037 Le Mans, France.

Introduction: Episodes of acquired bradykinin-mediated angioedema due to angiotensin-converting enzyme (ACE) inhibitors may result in fatal outcomes. There is no consensus regarding emergency pharmacological management of these episodes. Treatment options include icatibant and C1INH concentrate. Read More

Clin Chem Lab Med 2018 May 5. Epub 2018 May 5.

The Binding Site Group Limited, Birmingham, UK.

Background: The measurement of complement components is clinically useful where a deficiency is suspected, or where excessive activation and consumption are present in disease. C2 deficiency carries an increased risk of developing systemic lupus erythematosus, recurrent infections and atherosclerosis. In this study, we have evaluated The Binding Site's Human Complement C2 SPAPLUS® assay. Read More

Orphanet J Rare Dis 2018 May 4;13(1):73. Epub 2018 May 4.

Department of Dermatology and Allergy, Charité - Universitätsmedizin Berlin, Berlin, Germany.

Background: Bradykinin-mediated angioedema (Bk-AE) can be life-threatening and requires specific targeted therapies. Knowledge of its epidemiology may help optimize its management.

Methods: We systematically searched the medical literature to identify abstracts of interest indexed between 1948 and March, 2016. Read More

J Allergy Clin Immunol Pract 2018 Apr 30. Epub 2018 Apr 30.

Hungarian Angioedema Center, 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary. Electronic address:

Background: The mechanism of idiopathic nonhistaminergic acquired angioedema (InH-AAE) has not yet been precisely elucidated. This condition is characterized by recurrent angioedema without wheals.

Objective: To study the clinical features of InH-AAE, and to make, for the first time, independent comparisons with hereditary angioedema of unknown origin (U-HAE), as well as with hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE). Read More

Ann Dermatol Venereol 2018 Apr 16. Epub 2018 Apr 16.

Dermatologie, CHRU Minjoz, 3, boulevard Alexandre-Fleming, 25030 Besançon, France.

Background: Acquired C1-esterase inhibitor (C1-INH) deficiency angioedema (C1-INH-AAE) is a form of bradykinin-mediated angioedema. This rare disorder is due to acquired consumption of C1-INH, hyperactivation of the classic pathway of human complement, and potentially fatal recurrent angioedema symptoms. Clinical symptoms of C1-INH-AAE are very similar to those of hereditary angioedema (HAE) but usually appear after the fourth decade of life and induce abdominal pain less frequently. Read More

Cutis 2018 02;101(2):E14-E16

Department of Dermatology, Penn State Hershey Medical Center, USA.

Case Rep Hematol 2018 10;2018:7809535. Epub 2018 Jan 10.

Department of Medicine, Reading Hospital, West Reading, PA, USA.

Acquired angioedema due to C1 inhibitor deficiency (C1INH-AAE) is a rare and potentially fatal syndrome of bradykinin-mediated angioedema characterized by episodes of angioedema without urticaria. It typically manifests with nonpitting edema of the skin and edema in the gastrointestinal (GI) tract mucosa or upper airway. Edema of the upper airway and tongue may lead to life-threatening asphyxiation. Read More

J Investig Allergol Clin Immunol 2018 ;28(1):62-64

Clínica Tambre, Madrid, Spain.

Anesth Pain Med 2017 Aug 22;7(4):e57801. Epub 2017 Jul 22.

Department of Anesthesiology and Perioperative Medicine, Drexel University College of Medicine, 245 N. 15th Street, Suite 7502, MS 310, Philadelphia, PA, 19102.

Introduction: An acquired Tracheoesophageal fistula (TEF) is commonly caused by a malignancy or trauma, with pulmonary infection or aspiration being the presenting symptom. However, in the critical care setting the presentation can be subtle and may present with difficult ventilation. High endotracheal tube cuff pressures can lead to tracheal erosions and thus increasing the chances for developing a TEF. Read More

Int Arch Allergy Immunol 2018 26;175(3):126-135. Epub 2018 Jan 26.

Department of Medicine, Division of Allergy and Clinical Immunology, University of Salerno, Baronissi, Italy.

Recurrent angioedema (AE) without wheals is increasingly recognized as a clinical entity and a frequent cause of admission to the emergency room. The Hereditary Angioedema Working Group (HAWK) classification allowed the scientific community to go beyond the semantic confusion that dominated this topic for decades. This classification distinguishes hereditary and acquired forms of AE, either related or unrelated to C1 inhibitor deficiency. Read More

Sci Rep 2018 Jan 17;8(1):977. Epub 2018 Jan 17.

"L. Sacco" Department of Biomedical and Clinical Sciences, University of Milan, via GB Grassi 74, 20157, Milan, Italy.

C1-inhibitor is a serine protease inhibitor (serpin) controlling complement and contact system activation. Gene mutations result in reduced C1-inhibitor functional plasma level causing hereditary angioedema, a life-threatening disorder. Despite a stable defect, the clinical expression of hereditary angioedema is unpredictable, and the molecular mechanism underlying this variability remains undisclosed. Read More

Acute Med Surg 2017 10 18;4(4):462-466. Epub 2017 Aug 18.

Department of Emergency and Critical Care Medicine Japanese Red Cross Society Kyoto Daini Hospital Kyoto Japan.

Case: "Cannot intubate, cannot oxygenate" (CICO) is a rare, life-threatening situation. We describe a pediatric case of CICO and highlight some educational points.A 3-year-old boy who collapsed in the bathtub came to our emergency department. Read More

Pediatr Allergy Immunol 2017 12 24;28(8):850-852. Epub 2017 Oct 24.

Dermatology and Venereology, Medical University of Plovdiv, Plovdiv, Bulgaria.

Ann Allergy Asthma Immunol 2017 Oct 31;119(4):380-382. Epub 2017 Aug 31.

Division of Allergy and Clinical Immunology, Icahn School of Medicine, Mount Sinai, New York, New York.

Clin Transl Allergy 2017 31;7:27. Epub 2017 Aug 31.

Department of Clinical Immunology, University Hospital Zurich, Gloriastrasse 23, 8091 Zurich, Switzerland.

Background: Idiopathic non-histaminergic acquired angioedema (InH-AAE) is a rare disease for which there are no available laboratory parameters to clearly define the disorder. Therapy is often difficult and various treatment options have been proposed. In this paper, we have evaluated the most effective therapies for InH-AAE on the basis of current literature and report the therapeutic effect of omalizumab in three patients with InH-AAE. Read More

Dtsch Arztebl Int 2017 Jul;114(29-30):489-496

Department of Otorhinolaryngology, Ulm University Hospital; Department of Anaesthesiology, Ulm University Hospital.

Background: Acute angioedema of the upper airways can be life-threatening. An important distinction is drawn between mast-cell-mediated angioedema and bradykinin-mediated angioedema; the treatment of these two entities is fundamentally different.

Methods: This review is based on pertinent articles retrieved by a selective search in PubMed and on guidelines concerning the treatment of angioedema. Read More

Immunol Allergy Clin North Am 2017 08 15;37(3):497-511. Epub 2017 May 15.

Department of Medicine, Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Cox 201 Allergy Associates, Boston, MA 02114, USA.

Acquired angioedema due to C1-INH deficiency (C1-INH-AAE) can occur when there are acquired (not inherited) deficiencies of C1-INH. A quantitative or functional C1-INH deficiency with negative family history and low C1q is diagnostic of C1-INH-AAE. The most common conditions associated with C1-INH-AAE are autoimmunity and B-cell lymphoproliferative disorders. Read More

SAGE Open Med Case Rep 2017 8;5:2050313X17713912. Epub 2017 Jun 8.

Department of Anesthesiology and Critical Care Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.

Objective: To describe the perioperative management of a patient with acquired angioedema (AAE).

Methods: A 66-year-old Caucasian male presented from an outside hospital with a history of acquired angioedema and gastrointestinal stromal tumor-related intractable urticaria and mastocytosis. He was admitted for urgent laparoscopic partial gastrectomy, secondary to gastric outlet obstruction symptomatology. Read More

Allergy Asthma Clin Immunol 2017 13;13:28. Epub 2017 Jun 13.

Division of Allergy and Clinical Immunology, Department of Medicine, St. Michael's Hospital, Toronto, Canada.

Rationale: Angioedema without co-existent urticaria is due to a limited number of causes, including hereditary and acquired C1 esterase inhibitor deficiency, drug-induced angioedema or idiopathic histaminergic or non-histaminergic angioedema. We describe a cohort of patients with recurrent angioedema whose clinical features and response to medications are distinct from the causes above.

Methods: Patients were accrued retrospectively from an academic allergy practice between 2007 and 2014. Read More

Int J Emerg Med 2017 Dec 13;10(1):15. Epub 2017 Apr 13.

Department of Emergency Medicine, University Hospital, Aintree, Liverpool, UK.

Background: Angioedema is a common presentation in the emergency department (ED). Airway angioedema can be fatal; therefore, prompt diagnosis and correct treatment are vital.

Objective Of The Review: Based on the findings of two expert panels attended by international experts in angioedema and emergency medicine, this review aims to provide practical guidance on the diagnosis, differentiation, and management of histamine- and bradykinin-mediated angioedema in the ED. Read More

Dtsch Arztebl Int 2017 03;114(10):178

J Stomatol Oral Maxillofac Surg 2017 Apr 22;118(2):109-114. Epub 2017 Feb 22.

Service d'odontologie, hôpital Saint-Julien, CHU de Rouen, 76031 Rouen, France.

Bradykinin-mediated angioedema (AE) is a rare disease characterized by recurrent cutaneous or mucosal angioedema. This hereditary or acquired disease is of rapid installation, non-pruritic, usually painless and can affect the face, lips, larynx, gastrointestinal tract or extremities. When the affected area involves the upper respiratory tract, laryngeal angioedema can lead to imminent death by asphyxia. Read More

Clin Imaging 2017 May - Jun;43:122-126. Epub 2017 Mar 8.

Department of Radiology, Northwestern Memorial Hospital, Northwestern University Feinberg School of Medicine, 676 N. Saint Clair St., Suite 800, Chicago, IL 60611, United States. Electronic address:

Angioedema is a condition in which an increase in vascular permeability leads to the swelling of body tissues. There are both hereditary and acquired forms of the disease, with the latter often associated with the administration of angiotensin-converting enzyme inhibitor medication. Involvement of the intestinal tract is a rare manifestation of angioedema, and can present with abdominal pain, nausea, and vomiting. Read More

Crit Care Med 2017 Apr;45(4):725-735

1Division of Pulmonary, Critical Care, Allergy and Immunology, Department of Medicine, Wake Forest Baptist Medical Center, Winston-Salem, NC.2Division of Allergy and Clinical Immunology, Department of Medicine, W.G. (Bill) Hefner VA Medical Center, Salisbury, NC.

Objectives: Angioedema is a potentially life-threatening occurrence that is encountered by critical care providers. The mechanistic understanding of angioedema syndromes has improved in recent years, and novel medications are available that improve outcomes from these syndromes. This clinically focused review will describe the underlying genetics, pathophysiology, classification and treatment of angioedema syndromes, with an emphasis on the novel pharmacologic agents that have recently become available for acute treatment. Read More

J Allergy Clin Immunol Pract 2017 Sep - Oct;5(5):1307-1313. Epub 2017 Mar 9.

Department of Biomedical and Clinical Sciences, Luigi Sacco Hospital, University of Milan, Milan, Italy; ASST Fatebenefratelli Sacco, Milan, Italy.

Background: Acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE) is a rare disease with no prevalence data or approved therapies.

Objective: To report data on patients with C1-INH-AAE followed at Angioedema Center, Milan (from 1976 to 2015).

Methods: Diagnostic criteria included history of recurrent angioedema without wheals; decreased C1-INH antigen levels and/or functional activity of C1-INH and C4 antigen less than 50% of normal; late symptom onset (>40 years); no family history of angioedema and C1-INH deficiency. Read More

Int Arch Allergy Immunol 2017 22;172(1):55-63. Epub 2017 Feb 22.

Rheumatology, Allergology and Clinical Immunology, Department of "Medicina dei Sistemi", University of Rome Tor Vergata, Rome, Italy.

Background: Angioedema (AE) is a potentially life-threatening condition with hereditary (HAE), acquired (AAE), or iatrogenic causes. A careful workup allows for the identification of the etiology of attacks and the appropriate management. In this cohort study, based on a clinical practice setting, we aimed at investigating clinical and laboratory findings concerning different features of patients with recurrent AE who were referred to a single, tertiary-level center for HAE. Read More

G Ital Cardiol (Rome) 2016 Dec;17(12):966-972

Dipartimento di Scienze Biomediche e Cliniche "Luigi Sacco", Università degli Studi, Ospedale Luigi Sacco, Milano.

The definition of angioedema is an edema of subcutaneous and submucosal tissues due to increased vascular permeability and fluid extravasation. It can affect different areas, including extremities, genitals, upper airways and intestinal mucosa. The symptoms are disabling and this condition can be fatal if it involves the larynx. Read More

BMJ Case Rep 2017 Jan 10;2017. Epub 2017 Jan 10.

Department of Otorhinolaryngology, Head and Neck Surgery and Audiology, Rigshospitalet, Copenhagen, Denmark.

Angioedema (AE) of the upper airways is a severe and potentially life-threatening condition. The incidence has been increasing in the past two decades, primarily due to increased use of medications inhibiting the degradation of vasoactive peptides. Acquired angioedema related to angiotensin-converting enzyme inhibitors (ACEI-AAE) is well known, but other pharmaceutical agents also affect the degradation of bradykinin and substance P. Read More

Clin Exp Immunol 2017 Apr 9;188(1):148-153. Epub 2017 Feb 9.

Shire, Zug, Switzerland.

Icatibant is used to treat acute hereditary angioedema with C1 inhibitor deficiency types I/II (C1-INH-HAE types I/II) and has shown promise in angioedema due to acquired C1 inhibitor deficiency (C1-INH-AAE). Data from the Icatibant Outcome Survey (IOS) were analysed to evaluate the effectiveness of icatibant in the treatment of patients with C1-INH-AAE and compare disease characteristics with those with C1-INH-HAE types I/II. Key medical history (including prior occurrence of attacks) was recorded upon IOS enrolment. Read More

Immunol Allergy Clin North Am 2017 02 28;37(1):201-215. Epub 2016 Oct 28.

Yale School of Medicine, Gesher LLC, Allergy, Asthma and Clinical Immunology, 4 Clifton Avenue, Waterbury CT 06710, USA. Electronic address:

Differential diagnosis of urticaria and angioedema has been based on the phenotype as either acute or chronic depending on the duration of more than 6 to 8 weeks, respectively. Additional subdivisions include poorly defined terms such as idiopathic, spontaneous, or autoimmune. In this article, the author suggests that an increased understanding of the acquired and innate immune system and data from novel proteomic technology have blurred the lines between these categories of diagnosis. Read More

J Clin Immunol 2017 Jan 8;37(1):80-84. Epub 2016 Nov 8.

Internal Medicine Department, Grenoble University Hospital, Grenoble, France.

Idiopathic non-histaminergic acquired angioedema (InH-AAE) is a rare disease characterized by AE resistant to antihistamines and a chronic course. We report five new cases of InH-AAE (two women and three men) with a rapid and dramatic response to the anti-immunoglobulin-E antibody omalizumab. In our literature review, we found 13 other relevant cases with a good response to this treatment. Read More

Rev Alerg Mex 2016 Oct-Dec;63(4):373-384

Universidad de Antioquia. Medellín, Antioquia, Colombia.

Angioedema is defined as edema of the skin or mucosa, including the respiratory and the gastrointestinal mucosa, which is self-limiting, and in most cases is completely resolved in less than 72 hours. It occurs due to increased permeability of the mucosal and submucosal capillaries and postcapillary venules, with resulting plasma extravasation. There are different types of angioedema: histaminergic (which may be mediated by immunoglobulin E), hereditary, from acquired C1 inhibitor deficiency, from angiotensin converting enzyme inhibitor, bradykinin-mediated, and non-histaminergic idiopathic angioedema. Read More

Adv Clin Chem 2016;77:1-75. Epub 2016 Jul 5.

Mayo Clinic, Rochester, MN, United States. Electronic address:

Historically, complement disorders have been attributed to immunodeficiency associated with severe or frequent infection. More recently, however, complement has been recognized for its role in inflammation, autoimmune disorders, and vision loss. This paradigm shift requires a fundamental change in how complement testing is performed and interpreted. Read More

J Clin Anesth 2016 Nov 5;34:385-91. Epub 2016 Jun 5.

Department of Anesthesiology, Mayo Clinic, Rochester, MN. Electronic address:

Purpose: Two types of bradykinin-mediated angioedema, hereditary angioedema (HAE) and acquired angioedema (AAE), result from deficiency or dysfunction of C1 esterase inhibitor, leading to an overproduction of bradykinin, which can lead to vascular permeability and life-threatening angioedema of the airway. The objective of this study was to review perioperative outcomes in a series of patients with HAE and AAE and to review current knowledge about anesthetic complications in patients with HAE or AAE.

Methods: Medical records were retrospectively reviewed for perioperative complications in patients with HAE or AAE who underwent general anesthesia from January 1, 2000, to December 31, 2014, at our institution. Read More

Medicine (Baltimore) 2016 Aug;95(33):e4363

aInternal Medicine Department, Saint Antoine Hospital, Assistance Publique-Hôpitaux de Paris, DHU i2B, Paris 6 University, Paris bHematology Department, Pitié Salpétrière Hospital, Assistance Publique-Hôpitaux de Paris, Paris 6 University, Paris cImmunology Laboratory, University Hospital, Grenoble dCentre de Référence et d'Etude des Angioedèmes à Kinine (CREAK) , Grenoble ePublic Health Department, Tenon Hospital, Assistance Publique-Hôpitaux de Paris, Paris 6 University fImmunology Laboratory, Georges Pompidou European Hospital, Assistance Publique-Hôpitaux de Paris, Paris 5 University, Paris gJoint Unit 1036 CNRS-CEA-INSERM, University Grenoble Alpes hInternal Medicine Department, University Hospital, Grenoble iUniversité Joseph Fourier Grenoble, GREPI/AGIM CNRS FRE 3405, Grenoble jInternal Medicine Department, La Conception Hospital, AP-HM, Marseille kInternal Medicine and Clinical Immunology Department, Lille University Hospital lLIRIC, INSERM UMR 995, EA2686, Lille mDermatology Department, L'UNAM Université, University Hospital, Angers, France.

Acquired angioedema (AAE) due to C1-inhibitor (C1INH) deficiency is rare. Treatment options for acute attacks are variable and used off-label. Successful treatment of the associated lymphoma with rituximab seems to prevent acute attacks in subjects with AAE. Read More

J Eur Acad Dermatol Venereol 2017 Jan 8;31(1):45-52. Epub 2016 Aug 8.

Faculté de Médecine, Université de Strasbourg et Clinique Dermatologique, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.

Some dermatologic entities are strongly associated with the presence of a monoclonal gammopathy. They should be referred to as monoclonal gammopathy of cutaneous significance (MGCS). A short review of the main entities that fit into the spectrum of MGCS is provided. Read More

J Am Acad Dermatol 2016 Nov 30;75(5):918-924.e2. Epub 2016 Jul 30.

Department of Dermatology, Hospital del Mar- Institut Mar d'Investigacions Mèdiques, Universitat Autònoma de Barcelona (UAB), Barcelona, Spain.

Background: Data about special phenotypes, natural course, and prognostic variables of patients with acquired cold urticaria (ACU) are scarce.

Objectives: We sought to describe the clinical features and disease course of patients with ACU, with special attention paid to particular phenotypes, and to examine possible parameters that could predict the evolution of the disease.

Methods: This study was a retrospective chart review of 74 patients with ACU who visited a tertiary referral center of urticaria between 2005 and 2015. Read More

J Investig Allergol Clin Immunol 2016 ;26(4):212-21; quiz two pages after page 221

Department of Biomedical and Clinical Sciences Luigi Sacco, University of Milan, ASST Fatebenefratelli Sacco, Milano, Italy.

Angioedema is defined as local, noninflammatory, self-limiting edema that is circumscribed owing to increased leakage of plasma from the capillaries located in the deep layers of the skin and the mucosae. Two mediators, histamine and bradykinin, account for most cases of angioedema. Angioedema can occur with wheals as a manifestation of urticaria, and this form is frequently allergic. Read More

Int Arch Allergy Immunol 2016 28;170(2):101-7. Epub 2016 Jul 28.

Department of Dermatology, Johannes Gutenberg University, Mainz, Germany.

Objective: To evaluate whether activated partial thromboplastin time (APTT) could be used in the laboratory diagnosis of hereditary or acquired angioedema (HAE or AAE) with and without C1 inhibitor (C1-INH) deficiency.

Methods: In a prospective investigation, APTT and other coagulation parameters were determined in 149 adult patients with various types of angioedema and in 26 healthy participants (HP).

Results: Mean APTT was significantly shortened in HAE-C1-INH type I (p < 0. Read More

Dimens Crit Care Nurs 2016 Jul-Aug;35(4):181-9

Angela Smith Collins-Yoder PhD, RN, CCNS, ACNS BC, is a clinical professor at the University of Alabama Capstone College of Nursing and is a critical care clinical nurse specialist.

Angioedema (AE) is a unique clinical presentation of an unchecked release of bradykinin. The origin of this clinical presentation can be either genetic or acquired. The outcome within the patient is subcutaneous swelling of the lower layers of the epidermis. Read More

R I Med J (2013) 2016 Jun 1;99(6):41-4. Epub 2016 Jun 1.

Clinical Associate Professor of Medicine, Alpert Medical School of Brown University.

Hereditary and acquired angioedema are potentially life-threatening diseases characterized by spontaneous episodes of subcutaneous and submucosal swelling of face, lips, oral cavity, larynx, and GI tract. Hereditary angioedema (HAE) usually presents within the first and second decades of life, whereas acquired angioedema presents in adults after 40 years of age. These clinical symptoms together with reduced C1 inhibitor levels and/or activity can usually confirm the diagnosis. Read More

J Investig Allergol Clin Immunol 2016 ;26(2):111-2

Department of Medicine, University of Salerno, Salerno, Italy.

Clin Rev Allergy Immunol 2016 Oct;51(2):140-51

Research Laboratory, 3rd Department of Internal Medicine, Semmelweis University, Kútvölgyi út 4, H-1125, Budapest, Hungary.

Angioedema, as a distinct disease entity, often becomes a clinical challenge for physicians, because it may cause a life-threatening condition, whereas prompt and accurate laboratory diagnostics may not be available. Although the bedside diagnosis needs to be established based on clinical symptoms and signs, family history, and the therapeutic response, later, laboratory tests are available. Currently, only for five out of the nine different types of angioedema can be diagnosed by laboratory testing, and these occur only in a minority of the patient population. Read More

Case Rep Emerg Med 2016 31;2016:3930923. Epub 2016 Mar 31.

Department of Otorhinolaryngology, Head and Neck Surgery and Audiology, Rigshospitalet, Blegdamsvej 9, 2100 Copenhagen East, Denmark.

Angioedema of the upper airways is a severe and potentially life-threatening condition. The incidence has been increasing in the past two decades, primarily due to pharmaceuticals influencing the generation or degradation of the vasoactive molecule bradykinin. Plasma-derived C1-esterase inhibitor concentrate is a well-established treatment option of hereditary and acquired complement C1-esterase inhibitor deficiency, which are also mediated by an increased level of bradykinin resulting in recurrent angioedema. Read More

Clin Rev Allergy Immunol 2016 Oct;51(2):162-9

Department of Biomedical and Clinical Sciences "Luigi Sacco", University of Milan, Luigi Sacco Hospital, Milan, Italy.

Due to marked heterogeneity of clinical presentations, comprehensive knowledge of angioedema phenotypes is crucial for correct diagnosis and choosing the appropriate therapeutic approach. One of the ways to a meaningful clinical distinction can be made between forms of angioedema occurring "with or without wheals." Angioedema with wheals (rash) is a hallmark of urticaria, either acute or chronic, spontaneous or inducible. Read More

Ann Allergy Asthma Immunol 2016 May 26;116(5):392-3. Epub 2016 Mar 26.

Division of Allergy-Immunology, Northwestern University, Chicago, Illinois.

Ann Med 2016 26;48(4):256-67. Epub 2016 Mar 26.

c Department of Internal Medicine , Academic Medical Center, University of Amsterdam , The Netherlands.

Uncontrolled generation of bradykinin (BK) due to insufficient levels of protease inhibitors controlling contact phase (CP) activation, increased activity of CP proteins, and/or inadequate degradation of BK into inactive peptides increases vascular permeability via BK-receptor 2 (BKR2) and results in subcutaneous and submucosal edema formation. Hereditary and acquired angioedema due to C1-inhibitor deficiency (C1-INH-HAE and -AAE) are diseases characterized by serious and potentially fatal attacks of subcutaneous and submucosal edemas of upper airways, facial structures, abdomen, and extremities, due to inadequate control of BK generation. A decreased activity of C1-inhibitor is the hallmark of C1-INH-HAE (types 1 and 2) due to a mutation in the C1-inhibitor gene, whereas the deficiency in C1-inhibitor in C1-INH-AAE is the result of autoimmune phenomena. Read More