What is Quincke’s edema and how is it treated?

Everyone has probably heard about Quincke’s edema or angioedema – you can see these synonymous names in the lists of side effects of the majority of medicinal drugs. But what is it?

Short information about Quincke’s edema or angioedema

Quincke’s edema or angioedema is an acute allergic reaction distinguished by immediate and massive swelling of the skin, subcutaneous adipose tissue, and mucous epithelium. In the majority of cases, the swelling occurs in areas with loose subcutaneous fat which is why when people think of Quincke's edema, they imagine a person with a swollen neck, eyelids, and lower face. The swelling of other parts of the body, for instance, feet, hands, internal organs can also occur but happens much less frequently.
Worth noting, angioedema is a quite common allergic reaction. It is estimated that every 10th person has had or will have it in their lifetime at least once. Statistically, Quincke's edema mostly occurs in young and middle-aged women.

Quincke's edema classification

Although it seems to be just an allergic reaction, Quincke's edema still has different types. There are not many of them. Angioedema can occur in an acute form (lasts less than a month and a half) and chronic (lasts from 1.5-3 months and longer). Also, Quincke's edema can be isolated (this is the only manifestation of allergy) or combined with urticaria, bronchial asthma, itchy skin, and rash.

Quincke's edema classification based on the mechanism of the reaction:

- Hereditary (with the help of laboratory tests, the relative or absolute deficiency of the C1-inhibitor in the blood is established. But with angioedema, its presence may fall into the reference values);
- Acquired;
- Develops with the use of certain medications, accompanies different manifestations of allergies, due to certain diseases, including infectious diseases;
- Idiopathic (identification of the allergen causing edema is not possible).

Causes of Quincke's edema

Since angioedema is about allergies, first of all, an allergen can provoke the onset of edema. With allergic etiology, Quincke's edema may be accompanied by additional body reactions developing after exposure to an allergen. It can be bronchospasm or urticaria, rhinoconjunctivitis is also often observed.

The most common causes of Quincke's edema are:

- Food products;
- Plant pollen;
- Medications;
- Cosmetics and perfumery;
- Household chemicals;
- A bite of an insect;
- Contact with an animal allergen;
- Parasitic infection;
- Viral infection;
- Pseudoallergic edema is triggered by exposure to cold, heat, sunlight, stress, radiation.

Quincke's edema symptoms

The swelling develops very quickly. Usually, it takes from two to five minutes from the ingress of the allergen into the human body to the manifested reaction. Sometimes (more often with a hereditary predisposition), angioedema develops over several hours.
If Quincke's edema occurs, the diagnosis is not difficult. Most often, angioedema is localized on the lips, eyelids, tongue, cheeks, and larynx.

Quincke's edema is characterized by the following external manifestations:

- The part of the body on which the edema is localized is enlarged, its contours are smoothed, and the skin has not changed;
- Swelled area is dense;
- There is burning sensation, itching and pain at the site of edema;
- There is a feeling of tension in tissues affected by edema;
- The skin at the site of edema is pale;
- A person develops anxiety.

If angioedema develops in internal organs, the symptoms include “acute stomach” – acute pain in the abdomen, nausea, and vomiting, intense diarrhea, and others. In edema that affects the meninges (upper brain layer), neurological disorders such as epileptic seizures occur.

In terms of complications, edema of the larynx, tonsils, soft palate, and tongue often leads to suffocation. This happens in every third episode of Quincke's edema. If the swelling has passed to the larynx, then there is difficulty breathing (it can be whistling and noisy), cough, hoarseness. With the swelling of the larynx, it is extremely difficult for the victim to breathe, the more breathing can stop. This, as you know, can be fatal, therefore it requires emergency medical intervention.

Edema of the mucous membranes of the gastrointestinal tract leads to the appearance of dyspeptic disorders (indigestion, nausea, burping, etc.), and symptoms of peritonitis (inflammation of the peritoneum) can also be observed.

Edema of the mucous membranes of the urogenital system can lead to difficulty urinating, up to acute urinary retention.

Quincke's edema diagnostics

The clinical picture that occurs during angioedema makes it easy to make the correct diagnosis. Such a development of events is possible with the localization of edema in open areas of the body. If we are talking about edema of internal organs, then here the diagnosis takes longer and is more difficult. But the most difficult case in diagnosing Quincke's edema is hereditary angioedema since it is extremely difficult to identify the specific causative factor that induced it.

During diagnostic measures, the first step is to determine the root cause of such a reaction of the body. Quincke's edema does not appear out of blue and it can threaten life, thus the diagnosis must be approached responsibly. The main task of the patient is to tell about their condition in detail and list all of the factors, foods, they had been exposed to lately. The doctor must be sure to carefully collect anamnestic information, i.e. the time of symptoms occurrence, what the patient ate, used (cosmetics, detergents, etc.), etc. That is why the allergist conducts a survey not only about the patient's well-being, their diseases, and episodes of allergic reactions in the past but also about the presence of such cases in the patient's relatives. It is important to identify the body's response to drugs, food, animals, household allergens, physical factors, etc. During the diagnostic process, blood sampling for analysis and/or skin testing for allergies may be prescribed.

As for the diagnosis of hereditary angioedema (HAE), the initial collection of information from the patient and their thorough examination allow outlining the distinction between hereditary or acquired angioedema. Additionally, laboratory tests should be carried out. If Quincke's edema is hereditary, then in the overwhelming majority of cases the allergic reaction will slowly build up (these are the very few hours until edema occurs) and persist for a long time. Moreover, antihistamines will not work, which is understandable, since the edema is not caused by an allergen. Hereditary Quincke's edema often affects the respiratory tract and the digestive tract. There are no associated allergic reactions with HAE. That is, no urticaria, no bronchospasm, no hay fever, etc. The presence of such additional reactions is characteristic of edema of allergic genesis.

If you have noisy, wheezing breathing, a visual examination of the larynx (laryngoscopy) may be required. If edema is observed in the area of the gastrointestinal mucosa, then a consultation with a surgeon and endoscopic examination is necessary.

Treatment of angioedema

Usually, if a person has Quincke's edema, they should be hospitalized. There have been cases when angioedema was overcome with antihistamines over the counter, such as, for instance, Periactin (Cyproheptadine) or the attack went away on its own. If Quincke's edema threatens the patient's life (for example, the larynx is swollen and asphyxiation occurs), then specialists should take resuscitation measures.

If we consider therapeutic therapy for angioedema, then it can be divided into two stages:

- Relief of an attack;
- Treatment of the cause of edema - allergies.

When stopping an attack, antihistamines are administered. Most often, injections are used, since the hypothetical swelling of internal organs simply does not allow the necessary substances to penetrate through the digestive tract. Also, medications are used to narrow the peripheral vessels, if the patient has low blood pressure, or the edema has passed to the mucous membranes of the respiratory tract, then adrenaline is used. In emergency cases, resuscitation, intubation, or tracheostomy are performed.

Treating the underlying cause includes identifying risk factors, treating symptomatic relief, and preventing flare-ups. After the localization of the attack and the initiation of therapy, a special diet, which excludes the use of the most allergenic foods, is indicated. Outside of such a diet are foods that contain large amounts of histamine or provoke its production. These are cocoa and products containing cocoa, strawberries, bananas, peanuts, fermented cheeses, sauerkraut, spinach, tomatoes, citrus fruits, eggs, milk, fish, etc.

The duration of such a treatment and nutrition regimen depends on the nature of the course of the disease, the attack itself and is calculated by the attending physician. Symptomatic therapy with antiallergic drugs is prescribed by a doctor in the chronic course of the disease when Quincke's edema appears more than once, and there is a risk of relapse.

As for HAE, its treatment has nothing to do with the treatment of conventional angioedema. If you do not recognize HAE and start treating it incorrectly, then it can end with the death of the patient. During an exacerbation of HAE, replacement therapy for C1-inhibitor deficiency is performed.

Prevention of Quincke's edema

The first thing to do to prevent the recurrence of Quincke's edema is to identify the cause of angioedema and, if possible, avoid contact with the allergen. If the edema occurs due to some kind of physical impact, stress, or is of a pseudo-allergic nature, then the influence of such factors must be limited. Without this condition, the treatment will be useless. In addition, it is recommended to come to grips with your health and cure foci of chronic infection (if any), as they weaken the immune system and allow allergens to better penetrate the body. For maintenance therapy, periodically throughout the year, it is necessary to drink courses of antihistamines (for instance, Cyproheptadine) prescribed by a doctor. If the edema is not related to allergic genesis, then the therapy is preceded by an examination, tests, and study of the family history of the disease. And prevention consists of a nonspecific hypoallergenic diet that restricts the patient's consumption of certain foods, especially those with a proven allergy to them.

First aid for angioedema

Quincke's edema is an insidious condition that requires quick and competent assistance to the patient, since most often during an episode of Quincke's edema, the mucous membranes of the respiratory tract begin to swell, which is fraught with their obstruction, asphyxia, and death. Therefore, it is better to know how to provide first aid, so as not to get confused.

So, the sequence of actions should be as follows:

- Call the ambulance;
- If an allergen is identified, then immediately stop contact with it;
- If the swelling is a reaction to an insect bite or injection with medicine, then a pressure bandage should be applied to this place. If applying a bandage is impossible or difficult, then using a cold compress or ice, narrow the vessels at the injection site (bite), so you slow down the movement of the allergen in the systemic circulation;
- Give the patient the opportunity to breathe freely (loosen the collar of their shirt, unfasten the buttons, belt on their trousers);
- Provide a supply of fresh air;
- Reassure the patient, stay with them until the arrival of the team of specialists.

Remember that panic is your biggest enemy. Help calmly and confidently. Carefully monitor your health and the health of your loved ones. If you, or someone from your family, have allergic reactions, including episodes of Quincke's edema, then it is advisable to have a medical bracelet with information about the disease.

Post by: Kevin Phillips, dermatologist, Family Medicine Centre, Bradford, United Kingdom