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.

• Swelling

  Swelling may affect the abdomen, face, throat, limbs, and intestines. [slideshare.net]

  Hereditary angioedema is a disorder characterized by recurrent episodes of severe swelling (angioedema). The most common areas of the body to develop swelling are the limbs, face, intestinal tract, and airway. [ghr.nlm.nih.gov]

  Minor trauma has triggered peripheral swellings. These swellings burn rather than itch and are deep to the skin. If affecting a hand or foot it can make physical activity difficult. [stemlynsblog.org]

  The main symptom is swelling, which can be severe. Diagnosis is by examination. Treatment is with airway management as needed, elimination or avoidance of the allergen, and drugs to minimize swelling (eg, H1 blockers). [msdmanuals.com]

  Usually, this swelling is not accompanied by itching, as it might be with an allergic reaction. Swelling of the gastrointestinal tract leads to cramping. Swelling of the airway may lead to obstruction, a potentially very serious complication. [rarediseases.org]

• Pain

  Swelling may be burning or painful, or there may be itching if there is associated urticaria. There may be abdominal pain if gastrointestinal mucosa is affected. [patient.info]

  A 55-year-old Asian female presented to the emergency department complaining of oral pain, swelling, and numbness. Her family reported that she began to experience sharp pain of the tongue and lips immediately after eating "elephant root." [ncbi.nlm.nih.gov]

  Depending upon the region affected, the swelling may be described as burning or having a deep itch and often described being painful. [stemlynsblog.org]

  Abdominal pain if the gut is affected. If affecting the eye, it causes swelling of conjunctiva which results in problems with vision. [medindia.net]

  In cases in which angioedema affects the oral pharynx or larynx, as well as internal organs (often presenting with severe abdominal pain), immediate treatment as described below needs to be initiated. [clinicaladvisor.com]

• Fatigue

  […] possibly associated with an increased incidence of autoimmune disease Prognosis variable after the attack occurs, they may recur throughout the patient's life attack frequency can be reduced with appropriate therapy Presentation Symptoms prodromal symptoms fatigue [step2.medbullets.com]

  It is estimated that 50% of HAE patients will experience laryngeal swelling during their lifetime.1,7 Approximately 40% to 87% of HAE episodes are preceded by a prodromal syndrome.9 The prodromal phase may consist of tingling skin, local discomfort, fatigue [uspharmacist.com]

  History A family history of HAE is present in 75% of cases A trigger factor (stress, infection, injury) may be present, but not always Prodromal symptoms such as fatigue, flu-like symptoms, indigestion, tingling, or a rash (erythema marginatum) may precede [pch.health.wa.gov.au]

  Many patients have prodromal symptoms before the onset of angioedema, and these symptoms include fatigue, myalgia, arthralgia, and nonpruritic erythematous rashes such as erythema marginatum (Figure 1) (Agostini et al 2004). Figure 1. [priory.com]

  These may include: Extreme fatigue Muscle ache 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]

• Collapse

  This will lead to the following signs: sudden or rapidly escalating breathing problems fainting or dizziness collapsing This is a medical emergency. Call 911 immediately if you notice these signs. [medicalnewstoday.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. Angioedema often occurs in the face, lips, tongue, and larynx. [fpnotebook.com]

  These symptoms are thought to be caused by local cerebral edema and consequent cerebral hypoperfusion, caused by the acute HAE episode. [jamanetwork.com]

• Stridor

  Edema of the upper airways may cause respiratory distress and stridor; the stridor may be mistaken for asthma. The airways may be completely obstructed. Edema of the intestine may cause nausea, vomiting, colicky abdominal pain, and/or diarrhea. [msdmanuals.com]

  Hydromorphone was administered with subsequent rapid development of stridor and edematous changes of the tongue, uvula, and surrounding mucosa. [ncbi.nlm.nih.gov]

  If airway compromise is suspected (stridor, wheezing, diminished air movement) or the patient is in shock, treat immediately with IM epinephrine, start oxygen and IV fluids, and consider intubation to protect the airway. [amboss.com]

  If stridor occurs, re-intubation can be performed immediately over the exchange catheter. [emcrit.org]

   A physical exam might reveal abnormal sounds (stridor) when you breathe in if your throat is affected.  Blood tests or allergy testing may be ordered.  Testing for and Diagnosing Hereditary Angioedema  Blood tests can check the C1 inhibitor level [slideshare.net]

• Hoarseness

   Symptoms of intestinal swelling include:  Severe abdominal pain and cramping  dehydration  diarrhea  shock (in severe cases)  Symptoms of throat inflammation include:  hoarse voice  difficulty swallowing  difficulty breathing  Other symptoms [slideshare.net]

  Itching, burning, stinging, pain Dry, flaky, cracking, or scaly skin Swelling of the face, lips, tongue, or other parts of the body More severe symptoms may include: Trouble swallowing, or feeling like your throat is closing Trouble breathing or wheezing Hoarse [fairview.org]

  Your voice may sound hoarse. [msdmanuals.com]

  Examination General appearance and observations Look for airway swelling: tongue, fauces, soft palate, uvula Assess for laryngeal oedema: stridor, hoarseness, altered voice (ask caregivers) Assess hydration, and perform an abdominal examination Assess [pch.health.wa.gov.au]

• Dyspnea

  On admission, there was no pharyngeal or laryngeal edema and no dyspnea. Treatment with glucocorticoids and antihistamines had no response. [ncbi.nlm.nih.gov]

  She had no symptoms of upper airway obstruction or dyspnea, but her speech was dysarthric. The results of a physical examination were normal, and she had no urticaria. [cmaj.ca]

  […] 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 [litfl.com]

  […] acquired C1 inhibitor deficiency) ACE inhibitor-induced (rarely angiotensin-receptor blocker-induced): impaired bradykinin breakdown References:[1][2][3][4][5] Clinical features Common symptoms Facial edema (mouth, eyelids, tongue) Laryngeal involvement: dyspnea [amboss.com]

• Respiratory Distress

  If the swelling of the mucus membranes of the upper respiratory tract is severe, respiratory distress along with cough and wheezing may occur. [symptoma.com]

  Edema of the upper airways may cause respiratory distress and stridor; the stridor may be mistaken for asthma. The airways may be completely obstructed. Edema of the intestine may cause nausea, vomiting, colicky abdominal pain, and/or diarrhea. [msdmanuals.com]

  Mild/moderate angioedema episodes Present with: Swelling of the peripheries Mild/moderate abdominal pain Severe angioedema episodes Present with: Stridor or respiratory distress due to laryngeal oedema Other signs of potential airway compromise eg hoarse [rch.org.au]

  distress, stridor Face, hands, genitals Intestines--> nausea/vomiting, abdominal pain, diarrhea Differential Diagnosis Acute allergic reaction Allergic reaction/urticaria Anaphylaxis Angioedema Anxiety attack Asthma exacerbation Carcinoid syndrome Cold [wikem.org]

• Pleural Effusion

  Free fluid may be detected in serous cavities (Figure 3) and, if severe, detectable as ascites or pleural effusion. Figure 3. [priory.com]

  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]

• Abdominal Pain

  Abdominal associated with angioedema may manifest as severe acute-onset abdominal pain or as moderately severe chronic recurrent abdominal pain. Two medications are currently FDA-approved for the treatment of these patients. [ncbi.nlm.nih.gov]

  The majority of diagnosed HAE patients will only present to the ED when they are suffering from severe abdominal pain or airway threatening attacks. [rcemlearning.co.uk]

  A 19-year-old woman presents to the emergency department with light-headedness, severe abdominal pain, and intractable nausea and vomiting that began 12 hours earlier. [doi.org]

• Vomiting

  Involvement of the intestinal tract is a rare manifestation of angioedema, and can present with abdominal pain, nausea, and vomiting. [ncbi.nlm.nih.gov]

  In hereditary angioedema, caused by a defect in the immune system, swellings in the intestinal tract may produce pain, vomiting, or diarrhea, and edema of the larynx may cause death by asphyxiation. [britannica.com]

  […] cracking, or scaly skin Swelling of the face, lips, tongue, or other parts of the body More severe symptoms may include: Trouble swallowing, or feeling like your throat is closing Trouble breathing or wheezing Hoarse voice or trouble speaking Nausea, vomiting [fairview.org]

  Patients often present with non-specific findings of abdominal pain, nausea, and vomiting. [radiopaedia.org]

• Nausea

  […] urticaria or pruritus affecting the skin swelling of the extremities, face, and genitals can affect any area upper airway swelling of the lips, tongue, uvula, soft palate, and larynx gastrointestinal bowel wall edema resulting in gastrointestinal colic, nausea [step2.medbullets.com]

  Involvement of the intestinal tract is a rare manifestation of angioedema, and can present with abdominal pain, nausea, and vomiting. [ncbi.nlm.nih.gov]

  Patients often present with non-specific findings of abdominal pain, nausea, and vomiting. [radiopaedia.org]

• Diarrhea

  After an exhaustive laboratory work-up, her diarrhea resolved only after the discontinuation of lisinopril. [ncbi.nlm.nih.gov]

  Is severe diarrhea sign of angioedema? I have sudden onset of painful diarrhea with stomach pain. A. [medical-dictionary.thefreedictionary.com]

  In hereditary angioedema, caused by a defect in the immune system, swellings in the intestinal tract may produce pain, vomiting, or diarrhea, and edema of the larynx may cause death by asphyxiation. [britannica.com]

  The most common adverse drug reactions are injection site reactions, upper respiratory infections, headache, rash, muscle pain, dizziness, and diarrhea. "HAE attacks are painful, debilitating, and potentially life threatening. [medscape.com]

• Dysphagia

  A 65-year-old Saudi Arab man presented to an emergency department with lip and tongue swelling and dysphagia. There were no systemic symptoms and no past history of a similar event. [ncbi.nlm.nih.gov]

  […] 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 [litfl.com]

  Up to 20% of patients experience dyspnea, dysphagia, dysphonia, and stridor, which may progress rapidly to life-threatening airway obstruction unless treated. [americannursetoday.com]

• Lip Swelling

  Six months after the accident he developed acute onset bilateral facial swelling, lip swelling, and difficulty breathing. [hoajonline.com]

  On further questioning, she reported an episode of tongue and lip swelling occurring shortly after one of her abdominal attacks, which further supported the diagnosis. Subsequent testing described below confirmed the diagnosis. [web.archive.org]

  Further History: The patient has had swelling in the past, often around the face and peripherally and typically around menstruation. It has also been associated with URTI and often a lip swelling closely predates a cold sore eruption. [stemlynsblog.org]

  On post-transplant day 12, he developed upper and lower lip swelling ( Figure 1 ). There was no tongue swelling, stridor or respiratory distress, and no airway obstruction on nasolaryngoscopy. [nature.com]

  The most common areas of the body involved are the face, lips, tongue, throat and genital areas. Swelling in one area usually lasts between one to three days. [allergy.org.au]

• Tongue Edema

  edema pharyngeal edema (palate, uvula) laryngeal edema (true vocal cords, false vocal cords, arytenoids, aryepiglottic folds, epiglottis; the term upper airway oedema is probably more useful) lack of improvement during the ED course patients with isolated [litfl.com]

  (Ishoo stage I). b Tongue edema (Ishoo stage III). [doi.org]

• Macroglossia

  Infection (e.g. deep neck space infection) Foreign body Superior vena cava syndrome Macroglossia (e.g. due to acromegaly, amyloid, or hypothyroidism) Functional or factitious stridor Nasolaryngoscopy or bronchoscopy are preferred tests: (a) Identify location [emcrit.org]

  Gilbert Triplett, Massive Macroglossia Secondary to Angioedema: A Review and Presentation of a Case, Journal of Oral and Maxillofacial Surgery, 73, 5, (905), (2015). [doi.org]

• Oral Edema

  Life-threatening attacks, such as laryngeal edema or severe oral edema, require 20 U/kg IV. [reliasmedia.com]

• Hypotension

  Alternatively, the shifting of intravascular fluid into the tissues may result in hypotension and tachycardia regardless of the AE mechanism. [airwayelearning.com]

  Physical examination revealed a swollen tongue and no hypotension, rash, bronchospasm, urticaria, or flushing. [nejm.org]

  After he received intravenous alteplase infusion, he developed orolingual angioedema followed by transient bradycardia with subsequent hypotension, resulting in the deterioration of his neurological signs and expansion of the ischemic lesion. [ncbi.nlm.nih.gov]

  […] agonist terbutaline (Brethaire, Brethine) has been shown to be more effective at controlling urticaria than placebo. [6] However, efficacy is low for urticaria and angioedema; therefore, it is seldom used for treatment of angioedema. [6] Patients with hypotension [emedicine.com]

• Chest Pain

  It also affects mouth, throat and stomach and cause internal swelling resulting in severe stomach ache, difficulty breathing or chest pain. Common allergens like pollen, animal dander, latex and insect stings. [medindia.net]

  Call 911 Call 911 right away if any of these occur: Trouble breathing or swallowing, or wheezing Hoarse voice or trouble speaking, or drooling Chest pain or tightness Confusion, lightheadedness, or dizziness Extreme drowsiness or trouble awakening Fainting [fairview.org]

  Recurrent pulmonary and esophageal symptoms have been documented, including chest pain, shortness of breath, and severe pain while swallowing food. Urinary symptoms of HAE include urinary retention, bladder spasm, anuria, or pain at micturition. [ajmc.com]

  The swellings are not itchy or painful and skin colour is often normal. 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]

  Transient pleural effusions, sometimes with cough and mild pleuritic chest pain, can also occur. 9 Gastrointestinal tract symptoms of HAE, caused by visceral edema, result in varying degrees of intestinal obstruction. [jamanetwork.com]

• Tachycardia

  Alternatively, the shifting of intravascular fluid into the tissues may result in hypotension and tachycardia regardless of the AE mechanism. [airwayelearning.com]

  […] have airway compromise IMPENDING AIRWAY DISASTER TRIAD Rapid onset aphagia or severe dysphagia, frequently associated with a severe sore throat Rapid onset laryngeal voice change: hoarse, croaky, husky or no voice Systemically very unwell: pyrexia, tachycardia [entsho.com]

  English Leukocytopenia, thrombocytopenia, allergic reactions, hyperglycaemia, hypertonia, peripheral neuropathy, myocardial infarction, arrhythmia (including bradycardia, ventricular tachycardia and atrial fibrillation), vasculitis, pancreatitis, gastritis [mymemory.translated.net]

  For this group of patients adrenaline is not effective, or only marginally/briefly effective and may trigger unnecessary and sometimes severe side effects involving tachycardia, hypertension, arrhythmia and a risk of cerebral and cardiac insult (felleskatalogen.no [tidsskriftet.no]

• Periorbital Edema

  Periorbital edema of dermatomyositis may mimic angioedema [41]. [intechopen.com]

  Hypothyroidism Puffiness of face and lips; cold, dry skin; dry, coarse, brittle hair; pretibial myxedema Trichinosis Periorbital edema, history of eating raw/undercooked meat, myalgias, abdominal pain, diarrhea, vomiting Filarial parasites Lymphangitis [reliasmedia.com]

• Arthralgia

  Main symptoms are fever, arthralgias and skin involvement with urticaria-like nonpruritic lesions. Histopathologically, the perivascular infiltration is composed of polymorphonuclear cells in contrast with classical urticaria [29]. 11. [intechopen.com]

  Systemic vasculitis with hives often has elevated sedimentation rates, arthralgia, fever, etc. [theasthmacenter.org]

  Intestinal permeability in patients with chronic urticaria-angioedema with and without arthralgia. Ann Allergy. 1991;66(2):181-184. PMID: 1994789 www.ncbi.nlm.nih.gov/pubmed/1994789. Rye Rasmussen EH, Binslev-Jensen C, Bygum A. [stlukes-stl.com]

  […] often show cobblestone thickening of buccal mucous membranes Hypocomplementemic urticarial vasculitis syndrome (HUVS) Leukocytoclastic vasculitis, severe angioedema, laryngeal edema, associated with chronic obstructive pulmonary disease, arthritis, arthralgia [reliasmedia.com]

• Myalgia

  Cold exposure causes a burning rash, fever, chills, arthralgia, myalgia, headache, and leukocytosis. Hereditary vibratory angioedema This reaction consists of pruritus and swelling minutes after a vibratory stimulus. [theasthmacenter.org]

  Björn Gerdle, Ulrich Hilgenfeldt, Britt Larsson, Jesper Kristiansen, Karen Søgaard and Lars Rosendal, Bradykinin and kallidin levels in the trapezius muscle in patients with work-related trapezius myalgia, in patients with whiplash associated pain, and [doi.org]

  Many patients have prodromal symptoms before the onset of angioedema, and these symptoms include fatigue, myalgia, arthralgia, and nonpruritic erythematous rashes such as erythema marginatum (Figure 1) (Agostini et al 2004). Figure 1. [priory.com]

  Idiopathic episodes of recurrent edema that lead to permanent enlargement of lips Hypothyroidism Puffiness of face and lips; cold, dry skin; dry, coarse, brittle hair; pretibial myxedema Trichinosis Periorbital edema, history of eating raw/undercooked meat, myalgias [reliasmedia.com]

  […] who did not respond or responded suboptimally to androgen therapy may respond to adminstration of antifibrinolytic agents. 23 After an initial oral loading dose of 5 g, typical dosages effective for management of HAE have ranged from 7 to 10 g/d. 9 Myalgia [jamanetwork.com]

• Swelling of Hand

  Angioedema symptoms include: Swelling around the eyes, lips and ears. Rarer cases will also get swelling on hands, feet and throat. The swelling may also itch and is sometimes accompanied by a burning sensation and pain. [hives.org]

  Examples are hand swelling upon persistent contact with a cold object, or lip swelling when eating ice cream, or angioedema associated with swimming. [link.springer.com]

• Angioedema

  Angioedema Asem shadid 2. [slideshare.net]

  angioedema is severe or refractory. [msdmanuals.com]

  […] of other forms of angioedema. [karger.com]

  Histamine-related angioedema and angioedema in chronic spontaneous urticaria Histamine-related angioedema and angioedema in chronic spontaneous urticaria can occur as an isolated symptom or in combination with recurrent wheals. [clinicaladvisor.com]

  Hereditary angioedema is a disorder characterized by recurrent episodes of severe swelling (angioedema). The most common areas of the body to develop swelling are the limbs, face, intestinal tract, and airway. [ghr.nlm.nih.gov]

• Urticaria

  The procedure for angioedema is closely related to that for urticaria (see chapter on Chronic Spontaneous Urticaria.) [clinicaladvisor.com]

  There, these mediators cause urticaria and pruritus, which thus usually accompany mast cell–mediated angioedema. In bradykinin-mediated angioedema, the dermis is usually spared, so urticaria and pruritus are absent. [msdmanuals.com]

  Urticaria may occur alone in about 50% of cases. Urticaria occurs with angio-oedema in around 40-85% of cases; angio-oedema without urticaria occurs in only 10% of cases. [patient.info]

  The presence or absence of urticaria should always be considered and determined. When angioedema is associated with urticaria, the diagnosis and treatment approach will follow the intervention for urticaria. [emedicine.medscape.com]

• Eruptions

  Itay Tokatly Latzer, Nancy Agmon‐Levin, Raz Somech and Daniel Stein, Eruption of urticaria and angioedema induced by binging and purging in an anorexia nervosa patient, International Journal of Eating Disorders, 49, 8, (822-825), (2016). [doi.org]

  […] urticaria; giant, 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]

  Other skin conditions - eg, bullous pemphigoid, cellulitis and erysipelas, contact dermatitis, erythema multiforme, lymphoedema, morbilliform drug eruptions, SLE or urticaria pigmentosa. [patient.info]

• Hunger

  […] diagnostic indicator.13 Recurrent abdominal pain has been reported to occur in 70% to 90% of patients diagnosed with HAE.6,17 Attacks are often preceded by a prephase composed of nonspecific symptoms, such as irritability, aggressiveness, fatigue, or hunger [ajmc.com]

• Facial Swelling

  Female patient, 35 years old, married, doctor, reported crisis of intense facial swelling for nine years, always attacking the lips. [scielo.br]

  In 2009, FDA approved Berinert, to treat acute abdominal attacks and facial swelling associated with HAE in adults and adolescents. It is a protein product derived from human plasma, and is manufactured by CSL Behring, Inc. [rarediseases.org]

  Six months after the accident he developed acute onset bilateral facial swelling, lip swelling, and difficulty breathing. [hoajonline.com]

  Other causes of facial swelling or airway obstruction - eg, epiglottitis (can occur in adults), peritonsillar abscess or retropharyngeal abscess. Other causes of an acute abdomen. [patient.info]

• Facial Edema

  Here we report a case who developed oral-facial edema four years after using lisinopril/hydrochlorothiazide. Laryngeal oedema is a main cause of death in angioedema. [ncbi.nlm.nih.gov]

  However, life-threatening laryngeal edema may occur, which requires immediate airway protection. Treatment generally consists of supportive care and avoidance of triggers, if applicable. [amboss.com]

  The fatal attack, occurring at age 11, started with facial edema, followed by progressive dysphagia, dysphonia, and dyspnea. [hindawi.com]

  Skin edema is nonpitting, with ill-defined margins, and most commonly affects areas of the face, extremities, and genitals. Facial areas typically involved are the lips, eyelids, and tongue. [jamanetwork.com]

• Lip Edema

  (Fig. 5a ) Fig. 5 Presentation of angioedema in the emergency department. a Facial/lip edema (Ishoo stage I). b Tongue edema (Ishoo stage III). [doi.org]

  (Fig. 5a) Fig. 5 Presentation of angioedema in the emergency department. a Facial/lip edema (Ishoo stage I). b Tongue edema (Ishoo stage III). [intjem.biomedcentral.com]

• Neck Swelling

  A score for the differential diagnosis of bradykinin- and histamine-induced head and neck swellings. Eur Arch Otorhinolaryngol. 2018;275(7):1767-73. 98. Calbo L, Quattrocchi P, Ferlazzo B. [westjem.com]

• Head and Neck Swelling

  A score for the differential diagnosis of bradykinin- and histamine-induced head and neck swellings. Eur Arch Otorhinolaryngol. 2018;275(7):1767-73. 98. Calbo L, Quattrocchi P, Ferlazzo B. [westjem.com]

• Urinary Retention

  Urinary symptoms of HAE include urinary retention, bladder spasm, anuria, or pain at micturition. [ajmc.com]

  However, rarer clinical manifestations, including intense headaches caused by brain edema, urinary retention, and acute pancreatitis, can also occur. 12,35 The incidence and severity of the clinical manifestations of HAE vary among individuals. [scielo.br]

• Anuria

  Urinary symptoms of HAE include urinary retention, bladder spasm, anuria, or pain at micturition. [ajmc.com]

• Irritability

  Cases linked to physical irritation are extremely rare. [medicaljournals.se]

  They turn pale and swell if irritated. The deeper swelling of angioedema may also be painful. [ufhealth.org]

• Headache

  The adverse reactions reported include headache, rash, and, rarely, hypersensitivity reactions. [priory.com]

  The most common adverse drug reactions are injection site reactions, upper respiratory infections, headache, rash, muscle pain, dizziness, and diarrhea. "HAE attacks are painful, debilitating, and potentially life threatening. [medscape.com]

  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]

  Other side effects of antihistamines can include: headaches a dry mouth a dry nose If the swelling is severe, your GP may prescribe a short course of steroid medicine. [nhs.uk]

• Tingling

  […] øjenlågene og læberne (Quinckes ødem) og shock- lignende allergiske reaktioner med symptomer som prikken, rødme, kløe, hedeslag og forhøjet puls English region of the eyelids and the lips (Quincke's oedema) and shock-like allergic reactions with symptoms of tingling [mymemory.translated.net]

  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]

  There may be pain, numbness, tingling, or warmth in the affected area. Sometimes angioedema arises in conjunction with hives, which are red and itchy. [lifespan.org]

  You may also experience skin tingling or tightness. [health.ucsd.edu]

  History A family history of HAE is present in 75% of cases A trigger factor (stress, infection, injury) may be present, but not always Prodromal symptoms such as fatigue, flu-like symptoms, indigestion, tingling, or a rash (erythema marginatum) may precede [pch.health.wa.gov.au]

• Seizure

  To help address this question, the Observational Health Data Sciences and Informatics research network conducted a retrospective observational new-user cohort study of seizure patients exposed to levetiracetam (n = 276,665) across 10 databases. [ncbi.nlm.nih.gov]

  Hoarse voice or trouble speaking, or drooling Chest pain or tightness Confusion, lightheadedness, or dizziness Extreme drowsiness or trouble awakening Fainting or loss of consciousness Rapid heart rate Vomiting blood, or large amounts of blood in stool Seizure [fairview.org]

  […] characteristics in women Stanozolol Unknown 2-4 mg/day PO Hepatotoxicity, liver tumors, male secondary sex characteristics in women Tranexamic acid Cyklokapron, Lysteda Binds plasminogen binding sites Adults: 1 g twice daily Peds: 20 mg/kg/day PO Anaphylaxis, seizures [reliasmedia.com]

  Others 9, 16, 17 have reported seizures and hemiparesis. These symptoms are thought to be caused by local cerebral edema and consequent cerebral hypoperfusion, caused by the acute HAE episode. [jamanetwork.com]

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.

• Pleural Effusion

  Free fluid may be detected in serous cavities (Figure 3) and, if severe, detectable as ascites or pleural effusion. Figure 3. [priory.com]

  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]

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.

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.

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

• 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.

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].

Lanadelumab provides targeted inhibition of plasma kallikrein, an enzyme that is chronically uncontrolled in people with HAE, to help prevent attacks. [medscape.com]

This causes fewer side effects, but may not be as effective in preventing swelling. [nhs.uk]

HAE Attacks Triggered by Medical or Dental Work, Study Reports News NORD 2019 Rare Disease Summit Set for Oct. 21-22 in Washington, DC News NICE Recommends Takhzyro as Preventive Treatment for HAE Attacks News Preventative Haegarda Therapy Quickly Reduces [angioedemanews.com]

Treatment options include antihistamines and male steroids (androgens) that can also prevent the recurrent attacks. Also called hereditary angioedema. [medicinenet.com]

Completed NCT03888755 Phase 3 Icatibant 25 A Phase 3, Multicenter, Randomized, Single-Blind, Dose-Ranging, Crossover Study to Evaluate the Safety and Efficacy of Intravenous Administration of CINRYZE® (C1 Esterase Inhibitor [Human]) for the Prevention [malacards.org]

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.

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.