Angioedema histamine-mediated T78.3

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 24.08.2022

Definition
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Acute, mostly histamine-mediated (usually occurring in acute or chronic urticaria), single or recurrent at irregular intervals, lasting 1-7 days, circumscribed swelling of the skin and/or mucosa. Frequent occurrence on the face (periorbital and lips), induced by massive edema formation in the subcutis. But also other lower and/or upper extremities.

Most common form of angioedema with close causal association with urticaria. Often occurring in the setting of chronic idiopathic urticaria, intolerance urticaria, or in other forms of urticaria.

Note: Occurrence of chronic recurrent ("idiopathic") angioedema is also possible without concomitant presence of urticaria, hereditary angioedema, or renin-angiotensin-aldosterone system blocker-induced angioedema (e.g., due to ACE inhibitors).

Etiopathogenesis
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Often IgE-mediated(similar to an anaphylactic or anaphylactoid reaction) or non-immunological mechanism. Infectious causes (dental root granulomas, sinusitis, urinary tract infections) are found in about 5% of cases. > 40% of cases remain etiologically unexplained.

In children, infections are considered the most common causes of histamine-mediated angioedema.

Viral infections (herpes simplex viruses, Coxsackie A viruses, hepatitis B viruses, Epstein-Barr viruses) and bacterial infections (otitis media, sinusitis, tonsillitis, and urinary tract infections) have been described as precipitating factors.

Parasitic infections are less common causative factors(Strongyloides, Toxocara, filariae). Helicobacter pylori has been shown to be an exacerbating factor in hereditary angioedema.

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Manifestation
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No familial clustering. Beginning in adulthood.

Localization
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Predominantly located in the area of the face, especially on the eyelids and around the lips.

Clinical features
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Frequent occurrence of urticaria in the medical history. Frequent occurrence of unilateral and periorbital oedema. Occurrences of edema of the tongue and glottis are rather rare. The duration of angioedema is 1-5 days, followed by restitutio ad integrum. In contrast to kinin-mediated angioedema, gastrointestinal symptoms are rare to absent. Histamine-mediated angioedema usually occurs in combination with urticaria.

Laboratory
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For IgE-mediated reactions, detection of specific allergens in the RAST test. A significantly increased IgE may also indicate parasitosis (e.g. echinococcosis). Otherwise often no pathological laboratory findings.

Differential diagnosis
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Hereditary angioedema (important DD, because the therapeutic procedure differs, especially in laryngeal edema): can be excluded by the anamnestic data.

Erysipelas: High red, painful and febrile. Painful regional lymphadenitis. Leukocytosis and CRP elevation almost obligatory in acute erysipelas.

Contact dermatits: Acute contact dermatitis (especially of the capillitium) may be accompanied by significant reactive swelling of the facial skin. History of weeping pruritic scalp is diagnostic.

Photodermatitis: History, sharply demarcated, high-red dermatitis.

Therapy
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Clarification of the cause and consecutive prevention. This form of angioedema can also be described as "cortisone-sensitive". Antihistamines and glucocorticoids can be used if necessary. S.a.u. chronic urticaria.

Note(s)
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Due to the different tissue texture compared to the skin, generalized urticaria does not lead to the development of wheals but always to angioedema.

Literature
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Anliker MD et al (2003) Acute urticaria and angioedema due to ehrlichiosis. Dermatology 207: 417-418
Wedi B et al (2010) Infection focus and chronic spontaneous urticaria. dermatologist 61: 758-764