In Quincke’s edema – colloquially called angioedema (AE) – (synonyms: Acute Essential Edema; Acute Quincke’s Edema of the Skin; Acute Circumscribed Edema; Acute Circumscript Edema; Allergic Angioneurotic Edema; Allergic Angioedema; Allergic Glottic Edema; Angioneurotic edema; Angioneurotic edema with urticaria; Angioedema with urticaria; Bannister disease; Laryngeal urticaria; Periodic edema; Quincke syndrome; Urticaria gigantea; angioedema (AE); ICD-10 T78. 3) is an often massive swelling of the subcutis (submucosa) or submucosa (submucosal connective tissue) that usually affects the lips and eyelids, but may also involve the tongue or other organs.
Different causes can be distinguished. In most cases, it is an allergic reaction or the side effect of medication.
According to the cause, the following forms are distinguished:
- Histamine-mediated angioedema.
- Allergic angioedema; occurs in half of cases in association with urticaria (hives); most common form
- Allergy-like angioedema – in the context of infections, intolerances (intolerance reactions; usually triggered by drugs such as acetylsalicylic acid, ASS) or autoimmune reactions (pathological (pathological) reaction of the immune system against the body’s own tissue).
- Physical-conditional: e.g. pressure, cold, light, etc.
- Idiopathic angioedema – without apparent cause (rare).
- Bradykinin-mediated angioedema (bradykinin is a peptide and tissue hormone that is, among other things, vasoactive (blood vessel altering) and involved in pain production).
- Hereditary angioedema (HAE) – due to C1 esterase inhibitor (C1-INH) deficiency (blood protein deficiency); approximately 6% of cases:
- Type 1 (85% of cases) – decreased activity and concentration of C1 inhibitor.
- Type II (15% of cases) – decreased activity with normal or increased concentration of C1 inhibitor.
- RAAS inhibitor-induced angioedema (RAE) – due to cardiovascular drugs: ACE inhibitors (common: >50% of cases with severe angioedema) or AT1 antagonists (angiotensin II receptor subtype 1 antagonists, AT1 receptor antagonists, AT1 blockers, angiotensin receptor blockers, “sartans“) (rare).
- Acquired angioedema:
- Especially in malignant lymphoma (colloquially lymph node cancer) (type 1) or
- C1 inhibitor antibodies (type 2).
- Hereditary angioedema (HAE) – due to C1 esterase inhibitor (C1-INH) deficiency (blood protein deficiency); approximately 6% of cases:
- Other angioedema – the development cannot be explained by histamine alone, nor by bradykinin alone; other endogenous substances play a role here.
Frequency peak: allergic angioedema occurs predominantly in adulthood, while the hereditary form usually first appears in childhood and adolescence. The acquired form usually occurs after the age of 30.
The prevalence (disease frequency) for hereditary angioedema is < 1% (in Germany). In Germany, it is estimated that several thousand people are affected by recurrent (recurring) Quincke’s edema each year. Approximately 0.1-0.6% of patients taking ACE inhibitors develop angioedema. The proportion is even lower when taking AT1 blockers.
The incidence (frequency of new cases) for hereditary angioedema is 2-4 cases per 100,000 population per year (in Germany).
Course and prognosis: Quincke’s edema occurs acutely. It is painless and rarely itchy. The swelling may last for hours to days. If Quincke’s edema develops rapidly and involves the pharyngeal mucosa, it is a medical emergency because obstruction (narrowing) of the respiratory tract and consequent suffocation may occur.
