Question 1 of 10

    Angioedema (RAE)

    Angioedema2010[1]

    Angioedema is the swelling of skin, subcutaneous, or submucosal tissue due to acquired or genetic causes.

    The disorder is associated with this process: auto-immune.

    Presentation

    In hereditary angioedema, the patients present with swelling that develops 2 to 5 hours after any stimulus such as a minor trauma or infection. The swelling can involve any part of body and is often associated with rashes. The patients frequently experience abdominal symptoms such as nausea, vomiting, abdominal pain and diarrhea. The patients usually have one episode of attack per month. Some patient may have a higher frequency of attacks Attacks usually last from 12 to 48 hours.

    In acquired angioedema, the predominant feature of angioedema is swelling of the face, mucous membranes and hands. Swelling may be itchy and associated with skin rash. Sensations of the skin can be decreased due to extravasation of fluid that causes compression of the nerves. If the swelling of the mucus membranes of the upper respiratory tract is severe, respiratory distress along with cough and wheezing may occur [9][10]. The patient is unable to breathe which leads to rapid decrease in the oxygen concentration in the arterial blood. If this is not treated promptly, permanent brain damage may occur.

    Jaw & Teeth
    Lip Swelling
    • Six months later, the patient had an episode of bilateral facial swelling, lip swelling, and difficulty breathing.[hoajonline.com]
    • On post-transplant day 12, he developed upper and lower lip swelling ( Figure 1 ).[nature.com]
    • […] and face and arms); underactive thyroid gland (which can cause puffiness of the face and lips known as myxoedema); facial rosacea (causing non-specific puffiness of the face, redness, flushing and pimples); orofacial granulomatosis (fixed facial and lip[allergy.org.au]
    • swelling and eventually associated swelling of the tongue and larynx.[scielo.br]
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  • Entire body system
    Collapse
    • Dial 999 for an ambulance if you, or someone with you, has swelling and: sudden or worsening breathing problems feels faint or dizzy passes out or collapses These are signs of a serious allergic reaction (anaphylaxis) . [nhs.uk]
    • Emergency medical treatment will be necessary if the person: Suddenly develops symptoms of angioedema, like with an allergic reaction Has breathing problems that are sudden or are worsening Feels faint or dizzy, or if they faint or collapse If the person[medicalnewstoday.com]
    Fatigue
    • The swelling associated with certain autoimmune diseases, such as dermatomyositis, will be present for days, weeks or months at a time, is usually red, and often will be associated with other symptoms such as extreme fatigue, weakness, fevers, weight[allergies.about.com]
    • These may include: Extreme fatigue Muscle aches Tingling Headache Belly pain Hoarseness Mood changes Triggers Although you may not be able to tell what your triggers are, common ones include: Stress or anxiety Minor injury or surgery Illnesses such as[webmd.com]
    • , weight gain, family history of thyroid disease Hypothyroidism or Hashimoto's disease Weight loss and fatigue in patient with family historyof lymphoma Malignancy or lymphoma Travel out of country, exposure to disease Parasitic, fungal, bacterial, or[aafp.org]
    • ., injection-site reactions, headache, nausea, fatigue, diarrhea), this therapy has been associated with rare instances of allergic reactions and anaphylaxis.[aacijournal.biomedcentral.com]
    Localized Edema
    • A general term for a vascular reaction of the deep dermis, subcutaneous or submucosal tissues, which corresponds to localized edema 2º to vasodilation and capillary permeability. an·gi·o·e·de·ma ( an'jē-ō-ĕ-dē'mă ) 1.[medical-dictionary.thefreedictionary.com]
    • (NICHD) Definition (MSH) Swelling involving the deep DERMIS, subcutaneous, or submucosal tissues, representing localized EDEMA.[fpnotebook.com]
    Malaise
    • HAE attacks involving the larynx are particularly serious as the swelling can impair breathing and lead to suffocation. 1 HAE attacks are frequently associated with prodromal symptoms that include rash, influenza-like symptoms, malaise, paresthesia, headache[cslbehring.com]
    Pain
    Swelling
    • The swelling can be itchy or painful.[en.wikipedia.org]
    • If swelling is constant rather than coming and going, other possible causes of swelling should be considered.[allergy.org.au]
    • The swelling that results from severely low thyroid function is called myxedema , and often involves swelling around the eyes.[allergies.about.com]
    • The swelling is due to unregulated production of bradykinin which causes a deep tissue swelling.[aaia.ca]
    • Angioedema is swelling underneath the skin.[nhs.uk]
    Swelling of Limb
    • . , April 6, 2015 /PRNewswire/ -- Hereditary angioedema (HAE) – a rare genetic disorder that results in potentially life-threatening edema or swelling of limbs, abdomen, face, tongue or larynx – affects about 20 in 1 million Americans and still fewer[prnewswire.com]
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  • Face, Head & Neck
    Facial Swelling
    • During these two years she had intense abdominal pain twice, along with facial swelling.[scielo.br]
    • Six months later, the patient had an episode of bilateral facial swelling, lip swelling, and difficulty breathing.[hoajonline.com]
    • On day 10 post transplantation, she developed a pruritic skin rash on the upper and lower extremities, back and neck, followed by eyelid, lip and facial swelling.[nature.com]
    • Other causes of facial swelling or airway obstruction - eg, epiglottitis (can occur in adults), peritonsillar abscess or retropharyngeal abscess .[patient.info]
    • However, this condition is not always visible, as in cases involving the gastrointestinal (GI) tract. [10] Depending on the area of swelling, pain can be absent or mild, as in most peripheral or facial swelling, or it can be very severe, as in GI angioedema[emedicine.medscape.com]
    Neck Swelling
    • In the last few months before prescribing cannabis, the frequency and severity of the attacks worsened and included several presyncope events, associated with scrotal and neck swelling.[hindawi.com]
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  • neurologic
    Headache
    • If you find yourself feeling drowsy, you should avoid: driving drinking alcohol operating complex or heavy machinery Other side effects of the newer antihistamines include: headache dry mouth dry nose These side effects are usually short-lasting and should[hse.ie]
    • HAE attacks involving the larynx are particularly serious as the swelling can impair breathing and lead to suffocation. 1 HAE attacks are frequently associated with prodromal symptoms that include rash, influenza-like symptoms, malaise, paresthesia, headache[cslbehring.com]
    • (severe one-sided headache associated with puffiness around the eye on the same side).[allergy.org.au]
    • These may include: Extreme fatigue Muscle aches Tingling Headache Belly pain Hoarseness Mood changes Triggers Although you may not be able to tell what your triggers are, common ones include: Stress or anxiety Minor injury or surgery Illnesses such as[webmd.com]
    • […] without any provocation or induced by precipitating factors, including local trauma, vigorous exercise, emotional stress, alcohol, and hormonal factors (menstruation, pregnancy, oestrogen) Some patients may get a transitory prodromal non-itchy rash , headache[dermnetnz.org]
    Irritability
    • The welts can also swell and turn pale if you irritate the area.[gazettereview.com]
    • You should also try not to irritate areas on the body that have been previously affected.[healthline.com]
    • They turn pale and swell if irritated.[nytimes.com]
    • Eczema, also known as ‘atopic eczema’ or ‘atopic dermatitis’, is a skin condition causing inflammation and intense irritation.[allergyuk.org]
    Seizure
    Tingling
    • The rash is characterized by red rings that appear on the trunk and appendages as part of a prodrome that may also include a tingling sensation in the area where swelling will occur. 7 Reproduced with permission from the US Hereditary Angioedema Association[jaoa.org]
    • Many people describe the symptoms of angioedema as tingling, numbness or a burning sensation, like you may feel after going to the dentist.[allergies.about.com]
    • These may include: Extreme fatigue Muscle aches Tingling Headache Belly pain Hoarseness Mood changes Triggers Although you may not be able to tell what your triggers are, common ones include: Stress or anxiety Minor injury or surgery Illnesses such as[webmd.com]
    • In many cases, patients experience swelling and tightness of the skin or a tingling sensation, and in some cases pain, but it is usually not described as itchy.[aaia.ca]
    • Whilst angioedema may be itchy, tingling, or burning, often there are no symptoms other than the discomfort of the swelling.[allergy.org.au]
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  • Skin
    Eruptions
    • Angioedema, NOS , Angioneurotic edema, NOS , angioneurotic edema , angioneurotic oedema , Edema;angioneurotic French URTICAIRE GEANTE , Angio-oedème , Urticaires géantes , Oedème de Qincke , Oedème angionévrotique , Angioedème , Oedème de Quincke , ERUPTION[fpnotebook.com]
    • Acute viral illnesses in children can be associated with urticarial eruptions which last a few weeks and then spontaneously subside.[worldallergy.org]
    • Other skin conditions - eg, bullous pemphigoid , cellulitis and erysipelas , contact dermatitis , erythema multiforme , lymphoedema, morbilliform drug eruptions , SLE or urticaria pigmentosa.[patient.info]
    • There are different types of solar urticaria, which are classified according to the wavelength of light that induces the eruption.[theasthmacenter.org]
    • , hymenoptera envenomations, idiopathic angioedema, Milton disease, Milton's disease, periodic edema, Quincke disease, Quincke edema, Quincke's disease, Quincke's edema, urticaria, urticaria gigans, urticaria gigantea, urticaria tuberosa, urticarial eruptions[thefreshcarrot.ca]
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  • respiratoric
    Dyspnea
    • […] isolated or co-exist with urticaria areas affected asymmetric swelling of the lips and face tongue, the floor of the mouth, neck, and eyelids may affect extremities, genitalia or viscera (e.g. intensitines) Features of significant airway involvement: Dyspnea[lifeinthefastlane.com]
    • […] this could be allergic in nature Consider Glucagon 1-5mg IV if patient is on beta-blockers and not responding to Epinephrine FFP for possible etiology related to bradykinin [2] 2 units Consider definitive airway if voice change, hoarseness, stridor, dyspnea[wikem.org]
    • […] swelling; laryngoscopy is needed to assess laryngeal or vocal cord involvement; document swelling by means of physician notes, photographs, or both Severe attacks of angioedema can herald the onset of systemic anaphylaxis, characterized initially by dyspnea[emedicine.medscape.com]
    Hoarseness
    • […] asymmetric swelling of the lips and face tongue, the floor of the mouth, neck, and eyelids may affect extremities, genitalia or viscera (e.g. intensitines) Features of significant airway involvement: Dyspnea, dysphagia, dysphonia, odynophagia, stridor, hoarseness[lifeinthefastlane.com]
    • In rare cases, people with angioedema may experience a swollen throat, hoarseness, and difficulty breathing.[healthline.com]
    • These may include: Extreme fatigue Muscle aches Tingling Headache Belly pain Hoarseness Mood changes Triggers Although you may not be able to tell what your triggers are, common ones include: Stress or anxiety Minor injury or surgery Illnesses such as[webmd.com]
    • […] there is any concern this could be allergic in nature Consider Glucagon 1-5mg IV if patient is on beta-blockers and not responding to Epinephrine FFP for possible etiology related to bradykinin [2] 2 units Consider definitive airway if voice change, hoarseness[wikem.org]
    • Symptoms that are felt just before an attack in the throat may include trouble swallowing, a hoarse voice, a swollen tongue, cough, or shortness of breath.[aaia.ca]
    Respiratory Distress
    • There was no tongue swelling, stridor or respiratory distress, and no airway obstruction on nasolaryngoscopy.[nature.com]
    • The patient suddenly developed increasing stridor and respiratory distress, necessitating tracheal intubation.[journals.lww.com]
    • Angioedema of the GI tract can cause severe abdominal pain, while angioedema of the larynx or tongue can lead to respiratory distress.[theasthmacenter.org]
    • However, complications can range from dysphonia (difficulty speaking) or dysphagia (difficulty swallowing) to respiratory distress, complete airway obstruction and death.[thefreshcarrot.ca]
    Stridor
    • A physical exam might reveal abnormal sounds ( stridor ) when you breathe in if your throat is affected.[nytimes.com]
    • […] affected asymmetric swelling of the lips and face tongue, the floor of the mouth, neck, and eyelids may affect extremities, genitalia or viscera (e.g. intensitines) Features of significant airway involvement: Dyspnea, dysphagia, dysphonia, odynophagia, stridor[lifeinthefastlane.com]
    • If your throat is affected, a physical exam might reveal abnormal sounds ( stridor ) when you breathe in.[nlm.nih.gov]
    • In severe cases, stridor of the airway occurs, with gasping or wheezy inspiratory breath sounds and decreasing oxygen levels.[en.wikipedia.org]
    • […] concern this could be allergic in nature Consider Glucagon 1-5mg IV if patient is on beta-blockers and not responding to Epinephrine FFP for possible etiology related to bradykinin [2] 2 units Consider definitive airway if voice change, hoarseness, stridor[wikem.org]
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  • gastrointestinal
    Abdominal Cramps
    • Other symptoms may include: Abdominal cramping Breathing difficulty Swollen eyes and mouth Swollen lining of the eyes ( chemosis ) Back to Top Exams and Tests The health care provider will look at your skin and ask you if you have been exposed to any[nytimes.com]
    • Other symptoms may include: Abdominal cramping Breathing difficulty Swollen eyes and mouth Swollen lining of the eyes ( chemosis ) The health care provider will look at your skin and ask if you have been exposed to any irritating substances. [nlm.nih.gov]
    • Abdominal cramps, swollen eyes or mouth and swelling near the lining of the eyes are also common.[gazettereview.com]
    • […] pregnancy, oestrogen) Some patients may get a transitory prodromal non-itchy rash , headache, visual disturbance or anxiety Face, hands, arms, legs, genitals, digestive tract and airway may be affected; swellings spread slowly and may last for 3-4 days Abdominal[dermnetnz.org]
    Abdominal Pain
    • Abdominal pain occurs from intestinal involvement.[emedicine.medscape.com]
    • Abdominal pain from intestinal swelling is usually the most common type of swelling for patients.[aaia.ca]
    • Bowel wall angioedema presents as colicky abdominal pain.[uptodate.com]
    • Edema of the gastrointestinal mucosa typically leads to severe abdominal pain; in the upper respiratory tract, it can be life-threatening.[en.wikipedia.org]
    • Symptoms in the larynx and throat, as well as non-specific symptoms, such as dizziness and abdominal pain, were more frequent than previously reported.[medicaljournals.se]
    Colic
    • The hereditary form (HAE) often goes undetected for a long time, as its symptoms resemble those of more common disorders, such as allergy or intestinal colic.[en.wikipedia.org]
    • Occasionally HANE will produce peripheral edema or GI tract edema (colic) but an absence of urticaria or pruritus.[journals.lww.com]
    Diarrhea
    • Attacks involving the abdomen are often associated with severe pain, vomiting and diarrhea.[shire.com]
    • Reduce dose if diarrhea develops.[umm.edu]
    • Symptoms of abdominal swelling include mild discomfort to severe pain accompanied by vomiting and/or diarrhea.[cslbehring.com]
    • Patients with HAE can also have recurrent episodes (often called "attacks") of abdominal pain, usually accompanied by intense vomiting, weakness, and in some cases, watery diarrhea, and an unraised, nonitchy splotchy/swirly rash.[en.wikipedia.org]
    Dysphagia
    • […] or co-exist with urticaria areas affected asymmetric swelling of the lips and face tongue, the floor of the mouth, neck, and eyelids may affect extremities, genitalia or viscera (e.g. intensitines) Features of significant airway involvement: Dyspnea, dysphagia[lifeinthefastlane.com]
    • However, complications can range from dysphonia (difficulty speaking) or dysphagia (difficulty swallowing) to respiratory distress, complete airway obstruction and death.[thefreshcarrot.ca]
    • Regardless of the cause of uvular edema, the clinical condition is manifested as a foreign body sensation in the throat, dysphagia, muffled voice, gagging, and occasionally signs of upper airway obstruction.[journals.lww.com]
    Nausea
    • It is sometimes accompanied by nausea, vomiting, and ascites; and can sometimes be the only presenting symptom of angioedema.[emedicine.medscape.com]
    • Patients often present with nonspecific findings of abdominal pain, nausea, and vomiting.[radiopaedia.org]
    • Symptoms and signs include nausea, vomiting, Anaphylaxis Anaphylaxis is a serious allergic reaction that affects a number of different areas of the body at one time, and can be fatal.[medicinenet.com]
    • Side effects of prednisolone include: indigestion nausea (feeling sick) abdominal (tummy) pain increased appetite tiredness oral thrush - fungal infection inside your mouth dizziness Again, if you feel dizzy after taking prednisolone, avoid driving and[hse.ie]
    • Swelling in your belly can cause: Extreme pain Nausea Vomiting Diarrhea You may notice early warning signs before swelling begins.[webmd.com]
    Vomiting
    • It is sometimes accompanied by nausea, vomiting, and ascites; and can sometimes be the only presenting symptom of angioedema.[emedicine.medscape.com]
    • In the C1 INH test normal group angioedema was still active at the time of the study in 53% of the patients, and 36% reported symptoms in the throat, 23% in the abdominal area, 17% had diarrhoea, 11% had vomiting and 6% fainted during attacks.[medicaljournals.se]
    • Attacks involving the abdomen are often associated with severe pain, vomiting and diarrhea.[shire.com]
    • Patients often present with nonspecific findings of abdominal pain, nausea, and vomiting.[radiopaedia.org]
    • Symptoms and signs include nausea, vomiting, Anaphylaxis Anaphylaxis is a serious allergic reaction that affects a number of different areas of the body at one time, and can be fatal.[medicinenet.com]
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  • musculoskeletal
    Myalgia
    • Cold exposure causes a burning rash, fever, chills, arthralgia, myalgia, headache, and leukocytosis.[theasthmacenter.org]
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  • cardiovascular
    Chest Pain
    • Internal swelling is relatively common, resulting in presentation to hospital with severe stomach or chest pains without external swelling at the same time.[allergy.org.au]
    Tachycardia
    • Side effects of terbutaline include tachycardia and insomnia. 35 Other interventions have been used to treat refractory urticaria.[clevelandclinicmeded.com]
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  • urogenital
    Urinary Retention
    • The sedation can impair driving, increase the risk for occupational accidents, and adversely affect academic performance and workplace productivity. 29 Anticholinergic effects include dry mouth, constipation, urinary retention, and blurred vision.[clevelandclinicmeded.com]
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  • psychiatrical
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  • Eyes
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  • Ears
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  • Workup

    The diagnosis of angioedema can often be made on clinical grounds. Certain investigations are helpful in confirming the diagnosis and etiology of this disease. Mast cell tryptase levels are raised in the patients suffering from angioedema. However, unlike other allergic conditions, there is no response to therapy with antihistaminic and steroidal drugs.

    In hereditary angioedema, there is often a typical family history. In addition, the deficiency of C2 and C4 component of the complement system is present. In patients with F12 gene mutation, the diagnosis is done by excluding other types of angioedema.

    Test Results

    Laryngoscopy
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  • Laboratory

    Serum
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  • Pleura
    Pleural Effusion
    • Abdominal computed tomography – This may show severe edema of the bowel wall [1] Chest radiography – This may show pleural effusion Soft-tissue neck radiography – This may show soft-tissue swelling [ 5 ] See Workup for more detail.[emedicine.medscape.com]
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  • Imaging

    X-ray
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  • CT
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  • Treatment

    The treatment of angioedema depends upon the etiology.

    Hereditary angioedema:

    • The patient should avoid specific stimuli.
    • C1-INH concentrated blood should be transfused.
    • Fresh frozen plasma containing C1-INH may be used.
    • Androgens such as danazol which can increase aminopeptidase-P are given. Aminopeptidases-P inhibits bradykinin.

    ACE-inhibitors angioedema:

    • The use of the ACE inhibitor drug should be discontinued.
    • Alternative drugs such as Angiotensin Receptor Blockers (ARBs) are given.

    Allergic angioedema:

    • Exposure to the allergen should be avoided. If possible, desensitization should be done.
    • High dose cetirizine in given in acute attacks.
    • Steroids may be used.
    • Adrenaline may be used if respiratory distress occurs.

    Acquired angioedema:

    • Antifibrinolytics such as tranexamic acid or ε-aminocaproic acid are used in this case.
    • Cinnarizine can also be used.

    Burn angioedema:

    • Endotracheal tube should be passed prophylactically as soon as the patient is received as inhalation burns can lead to swelling obstruction of the airway. If obstruction has developed, endotracheal tube can not be passed.
    • If angioedema develops and endotracheal tube has not yet been passed, emergency cricothyriodectomy or mini-tracheostomy is necessary.

    Prognosis

    Early recognition of angioedema and aggressive treatment results in a very good prognosis. Early intubation with endotracheal tube lead also improves the prognosis by limiting the respiratory distress. If the treatment is delayed, it may even prove fatal for the patient.

    Complications

    Airway Obstruction
    • LMA) are more likely to fail due to able supraglottic soft-tissue swelling and airway obstruction emergency surgical airway may be difficult due to distortion of anatomy and subcutaneous edema (extended vertical incision may be required initially) the[lifeinthefastlane.com]
    • Attacks of the larynx can lead to airway obstruction and can be life threatening.[shire.com]
    • Although most patients present with urticaria [ 1 ], severe attacks may lead to airway obstruction and even death [ 2 ].[hindawi.com]
    • The patient had been in good health, but had had a similar episode that resulted in complete airway obstruction six years earlier.[journals.lww.com]
    • There was no tongue swelling or airway obstruction.[nature.com]
    Anaphylaxis
    • This is called exercise-induced anaphylaxis.[drugs.com]
    • Banerji, Aleena, MD Clinical Interests Drug hypersensitivity Angioedema Asthma Immunodeficiency Rhinosinusitis Cough Anaphylaxis Eosinophilic disorders Food sensitivity Dermatitis Urticaria Board Certifications Allergy & Immunology, American Board of[massgeneral.org]
    • Symptoms and signs include nausea, vomiting, Anaphylaxis Anaphylaxis is a serious allergic reaction that affects a number of different areas of the body at one time, and can be fatal.[medicinenet.com]
    • Differential diagnosis Anaphylaxis .[patient.info]
    • […] swelling that involves the reticular dermis, subcutaneous, and submucosal layers Angioedema may be life-threatening, depending on the underlying cause and the body location affected Airway involvement is usually the immediate life-threat The possibility of anaphylaxis[lifeinthefastlane.com]
    Angioedema
    • Drug-induced angioedema Gleich's syndrome (unexplained angioedema with high eosinophil counts) Angioedema at DMOZ[en.wikipedia.org]
    • In HAE, idiopathic angioedema (IAE), and drug-induced angioedema, episodes consist of relapsing, self-limiting edema.[emedicine.medscape.com]
    • Differential diagnosis The differential diagnosis should include intestinal occlusion syndrome, hereditary angioedema and histamine-induced angioedema (of allergenic or nonallergenic origin) generally associated with urticaria.[orpha.net]
    • […] study of rhC1INH to evaluate Hereditary Angioedema Rostock 18147 A clinical trial seeking patients for a research study for the treatment of Hereditary AngioEdema, Angioedema, Hereditary, Angio-Oedema of Lips or Angio-Oedema of Tongue Ulm 89075 Patients[centerwatch.com]
    • Excellence in Angioedema Care UC San Diego Health operates a comprehensive angioedema program and is at the forefront of angioedema research.[health.ucsd.edu]
    Anxiety
    • Precipitating factors of HAE 1 thought to trigger attacks include: trauma, anxiety, puberty, infection, alcohol consumption, exercise and stress.[orpha.net]
    • Precipitating factors of HAE 2 thought to trigger attacks include: trauma, anxiety, puberty, infection, alcohol consumption, exercise and stress.[orpha.net]
    • These may include: Extreme fatigue Muscle aches Tingling Headache Belly pain Hoarseness Mood changes Triggers Although you may not be able to tell what your triggers are, common ones include: Stress or anxiety Minor injury or surgery Illnesses such as[webmd.com]
    • Physiology more closely related to bradykinin-mediated pathway than IgE-mediated pathway, therefore current treatments may be insufficient Differential Diagnosis Acute allergic reaction Allergic reaction /urticaria Anaphylaxis Angioedema Asthma exacerbation Anxiety[wikem.org]
    Ascites
    • The edemas may involve the digestive tract resulting in a clinical picture similar to that seen in intestinal occlusion syndrome, sometimes associated with ascites and hypovolemic shock.[orpha.net]
    • It is sometimes accompanied by nausea, vomiting, and ascites; and can sometimes be the only presenting symptom of angioedema.[emedicine.medscape.com]
    • Edemas may involve the digestive tract resulting in a clinical picture similar to that seen in intestinal occlusion syndrome, sometimes associated with ascites and hypovolemic shock.[orpha.net]
    • […] internal organ involvement is suspected, during acute attacks, the following studies can be performed: Plain abdominal films – These may show a “stacked coin” or “thumbprint” appearance of the intestines [ 4 ] Abdominal ultrasonography – This may show ascites[emedicine.medscape.com]
    Asthma
    • Banerji, Aleena, MD Clinical Interests Drug hypersensitivity Angioedema Asthma Immunodeficiency Rhinosinusitis Cough Anaphylaxis Eosinophilic disorders Food sensitivity Dermatitis Urticaria Board Certifications Allergy & Immunology, American Board of[massgeneral.org]
    • […] inbox the latest updates and information on allergies and asthma.[medicinenet.com]
    • Allergy, Asthma & Clinical Immunology 2010;6:20.[aaia.ca]
    • An atopic individual is likely to have more than one allergic condition during their lifetime, such as eczema, asthma, hay fever or food allergy.[allergyuk.org]
    • American College of Allergy, Asthma, and Immunology. .[mayoclinic.org]
    Constipation
    • The sedation can impair driving, increase the risk for occupational accidents, and adversely affect academic performance and workplace productivity. 29 Anticholinergic effects include dry mouth, constipation, urinary retention, and blurred vision.[clevelandclinicmeded.com]
    Edema
    • Dental procedures are a triggering factor for laryngeal edema.[orpha.net]
    • During episodes, edema may occur in the subcutaneous tissue, the wall of the intestine, and the larynx.[emedicine.medscape.com]
    • , giant urticaria , circumscribed; edema , Quincke; edema , Quincke , edema; Quincke , edema; angioneurotic , edema; circumscribed , giant; urticaria , angioneurotic; edema , urticaria; giant , Angioedema, NOS , Angioneurotic edema, NOS , angioneurotic[fpnotebook.com]
    • […] of allergic edema (Table 1).[oralhealthgroup.com]
    Erythema Marginatum
    • The serpiginous, nonpruritic rash of erythema marginatum, which precedes an attack of hereditary angiodema in one-third of patients.[jaoa.org]
    • About one-third of people with this condition develop a non-itchy rash called erythema marginatum during an attack.[ghr.nlm.nih.gov]
    • marginatum, an erythematous serpentine but nonpruritic and non-raised rash (do not confuse with urticaria) family history See Table 4 of Moellman et al (2014) for a comparison of historical features of different causes of angioedema INVESTIGATIONS There[lifeinthefastlane.com]
    • marginatum, which is characteristic of HAE due to INHC1 deficiency Bork suggested that the face would be the site most affected in these patients, especially the lips. 12 It is observed that the clinical manifestations of HAE type III are highly variable[scielo.br]
    Hypotension
    • There Low Blood Pressure (Hypotension) Low blood pressure, also referred to as hypotension, is blood pressure that is so low that it causes symptoms or signs due to the low flow of blood through Rash The word "rash" means an outbreak of red bumps on the[medicinenet.com]
    • Patients may also experience hypotension, with feelings of light-headedness and dizziness.[cslbehring.com]
    • According to guidelines published by The World Allergy Organization (WAO) [ 3 ], the workup of patients presenting with signs and symptoms of angioedema (urticaria, flushing, generalized pruritus, bronchospasm, throat tightness, and/or hypotension) includes[hindawi.com]
    • Exercise-induced anaphylaxis was first described in a series of patients in whom combinations of pruritus, urticaria, angioedema, wheezing, and hypotension occurred as a result of exercise.[worldallergy.org]
    • […] angioedema or isolated angioedema episodes should be prescribed epinephrine in an auto-injectable instrument (EpiPen) in a dosage of 0.3 mg given intra-muscularly in the thigh and be instructed in its proper use for laryngeal edema, bronchospasm, and hypotension[aafp.org]
    Laryngeal Edema
    • Dental procedures are a triggering factor for laryngeal edema.[orpha.net]
    • Laryngeal edema and death from asphyxiation after tooth extraction in four patients with hereditary angioedema.[oralhealthgroup.com]
    • Isolated angioedema, especially laryngeal edema, requires consideration for hereditary or acquired C1-inhibitor deficiency.[clevelandclinicmeded.com]
    • […] resolution has occurred this often requires admission to an HDU/ICU admission to hospital, rather than in ED, is preferred in the following situations (Winters et al, 2013): previous history of angioedema tongue edema pharyngeal edema (palate, uvula) laryngeal[lifeinthefastlane.com]
    Leukocytosis
    • Also in the differential diagnosis is upper airway infection in the presence of pain, fever, or leukocytosis and the rare case of epiglottitis.[journals.lww.com]
    • Cold exposure causes a burning rash, fever, chills, arthralgia, myalgia, headache, and leukocytosis.[theasthmacenter.org]
    Serum Sickness
    • An example is a transfusion reaction in which IgG and IgM anti–red-cell antibodies activate complement and cause cell lysis. 9 Serum sickness caused by drugs or proteins is an example of a type III hypersensitivity (antigen-antibody complex-mediated)[aafp.org]
    • See also anaphylaxis , serum sickness , urticaria.[medical-dictionary.thefreedictionary.com]
    Upper Airway Obstruction
    • Clinical studies of sudden upper airway obstruction in patients with hereditary angioedema due to C1 esterase inhibitor deficiency.[oralhealthgroup.com]
    • Regardless of the cause of uvular edema, the clinical condition is manifested as a foreign body sensation in the throat, dysphagia, muffled voice, gagging, and occasionally signs of upper airway obstruction.[journals.lww.com]
    Urticaria
    • When angioedema is associated with urticaria, the diagnosis and treatment approach will follow the intervention for urticaria.[emedicine.medscape.com]
    • Edema , Urticaria, Giant , Urticarias, Giant , Edema, Quincke's , ANGIOEDEMA , ANGIONEUROTIC EDEMA , ANGIONEUROTIC OEDEMA , Edema, Angioneurotic , GIANT HIVES , GIANT URTICARIA , Giant Urticaria , QUINCKE'S EDEMA , QUINCKE'S OEDEMA , Urticaria giant[fpnotebook.com]
    • Solar urticaria Solar urticaria is a rare disorder in which brief exposure to light causes the development of urticaria within 1-3 minutes.[worldallergy.org]
  • more...
  • Etiology

    Hereditary angioedema: Occurs due to an autosomal dominant gene mutation. The genes include the SERPING1 gene and F12 gene. Both of these genes lead to pathological activation of complement pathway.

    Acquired angioedema: Acquired angioedema can result from the following causes:

    • Immunological
    • Allergy to certain allergens such as peanuts and food colors [1]
    • Reaction to medications such as ACE inhibitors
    • Burn
    • Idiopathic

    Epidemiology

    • Allergic angioedema is most common in the United States.
    • Drug-induced angioedema is more common in African-American.
    • Annually 80000 to 112000 patients suffering from angioedema visit emergency department.

    Sex distribution
    Age distribution

    Pathophysiology

    Angioedema is the swelling of subcutaneous tissues and mucosa due to accumulation of fluid [2] [3]. It results from various stimuli that cause the release of bradykinin [4] [5]. In addition to being a potent vasodilator, bradykinin also facilitates the extravasation of fluid from the blood vessels. Intravascular fluid therefore starts accumulating extravascularly leading to the swelling. The skin around the eyes is the loosest skin of the body; therefore, the fluid accumulate there first and later other area of the face also succumb to swelling.

    Angiotensin converting enzyme (ACE) normally causes the breakdown of bradykinin in the body. However, when ACE inhibitors are used, bradykinin breakdown is suppressed and angioedema can develop.

    In hereditary angioedema, there is a decreased production of C1-INH which leads to the activation of the complement system [6] [7]. This unopposed activation of complement system results in the continuous production of bradykinin. In autoimmune angioedema, antibodies are formed against C1-INH and bradykinin is similarly increased.

    In addition, some agents such as alcohol and some drugs (such as ibuprofen and aspirin) can increase the incidence of angioedema [8].

    Prevention

    • Susceptible individuals should avoid drugs such as alcohol, aspirin and ACE Inhibitors.
    • Exposure to known allergens should be avoided.
    • Burns should be prevented.

    Summary

    Angioedema is swelling of skin, subcutaneous and submucosal tissues and can be acquired or genetic. In genetic angioedema, SERPING1 and F12 genes are involved. Acquired angioedema can be due to drugs, allergy or burns. The main mechanism of swelling in angioedema is the activation of bradykinin which is a potent vasodilator. The patients usually present with swelling of face and hands. In severe cases, respiratory distress can occur.

    Patient Information

    Angioedema means the swelling of the skin and underlying tissue because of the collection of fluid. It can result from genetic causes or from acquired causes. The patients usually develop swelling of the face, arms and hands. Sometimes, swelling may involve the upper respiratory tract which leads to breathing difficulties. Emergency management may be indicated in such cases. The treatment depends upon the cause but the overall prognosis is good.

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    References

    1. Frigas E, Nzeako UC. Angioedema. Pathogenesis, differential diagnosis, and treatment. Clinical reviews in allergy & immunology. Oct 2002;23(2):217-231.
    2. Scheirey CD, Scholz FJ, Shortsleeve MJ, Katz DS. Angiotensin-converting enzyme inhibitor-induced small-bowel angioedema: clinical and imaging findings in 20 patients. AJR. American journal of roentgenology. Aug 2011;197(2):393-398.
    3. Wakisaka M, Shuto M, Abe H, et al. Computed tomography of the gastrointestinal manifestation of hereditary angioedema. Radiation medicine. Dec 2008;26(10):618-621.
    4. Kaplan AP. Mediators of urticaria and angioedema. The Journal of allergy and clinical immunology. Nov 1977;60(5):324-332.
    5. Oschatz C, Maas C, Lecher B, et al. Mast cells increase vascular permeability by heparin-initiated bradykinin formation in vivo. Immunity. Feb 25 2011;34(2):258-268.
    6. Kalmar L, Hegedus T, Farkas H, Nagy M, Tordai A. HAEdb: a novel interactive, locus-specific mutation database for the C1 inhibitor gene. Human mutation. Jan 2005;25(1):1-5.
    7. Cugno M, Zanichelli A, Foieni F, Caccia S, Cicardi M. C1-inhibitor deficiency and angioedema: molecular mechanisms and clinical progress. Trends in molecular medicine. Feb 2009;15(2):69-78.
    8. Asero R, Bavbek S, Blanca M, et al. Clinical management of patients with a history of urticaria/angioedema induced by multiple NSAIDs: an expert panel review. International archives of allergy and immunology. 2013;160(2):126-133.
    9. Rye Rasmussen EH, Bindslev-Jensen C, Bygum A. Angioedema--assessment and treatment. Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke. Nov 12 2012;132(21):2391-2395.
    10. Kaplan AP. Angioedema. The World Allergy Organization journal. Jun 2008;1(6):103-113.

    • Bibliography Current World Literature - RL Carrau, A Khidr, KF Gold - Head and Neck Surgery, 2006 - journals.lww.com
    • Angioneurotic edema attributed to the use of losartan - EW van Rijnsoever, WJM Kwee-Zuiderwijk - Archives of internal , 1998 - Am Med Assoc
    • Angiotensin-converting enzyme inhibitor-induced angioedema: a multicenter review and an algorithm for airway management. - AG Chiu, KA Newkirk, BJ Davidson - The Annals of otology, , 2001 - ukpmc.ac.uk
    • A survey on the management of pollen-food allergy syndrome in allergy practices - S Ma, SH Sicherer, A Nowak-Wegrzyn - Journal of allergy and clinical , 2003 - Elsevier
    • Angioedema acute hypersensitivity reaction to injectable hyaluronic acid - JM Leonhardt, N Lawrence, RS Narins - Dermatologic surgery, 2005 - Wiley Online Library
    • THE CHARACTERISTICS OF ACUTE KIDNEY INJURY COMPLICATED IN ACUTE HEPATITIS AETIOLOGY AND OUTCOME OF ACUTE RENAL FAILURE RELATED - V Luyckx, M Obeidat, A Zadovni, S Jackson - NDT , 2009 - ckj.oxfordjournals.org
    • A vote for inhaled adrenaline in the treatment of severe upper airway obstruction caused by piercing of the tongue in hereditary angioedema - D Trachsel, J Hammer - Intensive care medicine, 1999 - Springer
    • Adverse reactions to benzoates and parabens. - DW Jacobsen, DD Metcalfe, HA Sampson - Food allergy: adverse , 1996 - cabdirect.org
    • Acute Angioedema and Urticaria Associated with Herpes Simplex Infection - P Youngnak-Piboonratanakit, Y Takahashi - Acta stomatologica , 2008 - hrcak.srce.hr
    • Allergic reactions associated with airborne fish particles in IgE‐mediated fish hypersensitive patients - JF Crespo, C Pascual, C Dominguez, I Ojeda - Allergy, 1995 - Wiley Online Library

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