Vnitr Lek 2025, 71(6):343-352 | DOI: 10.36290/vnl.2025.066

Angioedema - differential diagnosis and new therapeutic options

Irena Krčmová

Ústav klinické imunologe a alergologie, FN Hradec Králové, LF UK Hradec Králové

Angioedema is defined as transient, recurrent swelling of the subcutaneous and/or submucosal tissues. Angioedema may affect the face, lips, neck and extremities, oral cavity, larynx and/or intestinal mucosa. Local swelling results from regionally increased permeability in subcutaneous or submucosal capillaries and postcapillary venules. Plasma extravasation occurs in response to vasoactive mediators released by activation of mast cells and/or basophils or the contact (kinin-kallikrein), complement and fibrinolytic enzyme systems of plasma, although biologically the situation is not clearly black and white. From a pathophysiological perspective, angioedema can be classified as histamine-mediated angioedema and bradykinin-mediated angioedema. Histamine-mediated angioedema is more common and is associated with activation and degranulation of mast cells and basophils, and is often accompanied by pruritic and erythematous urticaria. Bradykinin-mediated angioedema can include forms of hereditary angioedema, acquired C1 inhibitor deficiency, and angioedema associated with angiotensin-converting enzyme inhibitors or other drugs. It is characterized by excessive local production of bradykinin with the development of painful angioedema, is not associated with pruritic urticaria, has a longer duration, and often abdominal symptoms. It is resistant to standard therapies such as adrenaline, glucocorticoids, and antihistamines. As part of the differential diagnosis in the acute phase, it is appropriate to perform a laboratory test for tryptase to differentiate histamine angioedema in conjunction with anaphylaxis, and the C4 component of complement as a screening for bradykinin angioedema. Patient care is interdisciplinary, especially in the acute phases of angioedema. In recurrent histamine angioedema, which is mainly a concomitant phenomenon of chronic spontaneous urticaria, 2nd and 3rd line treatment is administered within the expertise of dermatology and immunology/allergology. In cases of hereditary angioedema, therapy is within the expertise of immunology/allergology, and 4 centers in the Czech Republic are designated for this rare disease. The advent of biological treatments for both types of angioedema has fundamentally changed the treatment approach.

Keywords: angioedema, diagnostics, differential diagnostics, therapy.

Accepted: September 21, 2025; Published: October 2, 2025  Show citation