Mastocytosis is a rare disease characterized by an excessive number of mast cells in the body. The condition is classified as cutaneous when symptoms are limited to the skin, and systemic when at least one organ beyond the skin is affected. It often appears in children, mainly between the ages of 1–4 years, but its incidence also increases between the ages of 30–50. Cutaneous mastocytosis includes various forms such as: Urticaria pigmentosa (the most common form, where the skin appears “leopard-like”). Diffuse cutaneous mastocytosis (DCM). Solitary mastocytoma (usually appears in infancy as a single nodule). Telangiectasia macularis eruptiva perstans.

There are two types of angioedema: hereditary and acquired. In the hereditary form, swelling may also occur in other parts of the body or even in internal organs such as the intestine. A key characteristic is that it does not respond to antihistamines or corticosteroids and lasts much longer than other forms of angioedema. It is caused by a deficiency of the C1 inhibitor (C1inh) protein, which regulates the activation of the kinin system, complement system, and blood coagulation. In the acquired form, the deficiency of C1inh is secondary and usually associated with hematological malignancies. Similar to hereditary angioedema, acquired angioedema may cause dangerous swelling in the larynx; however, its management differs from that of allergic angioedema.

Due to the severity of angioedema, it is extremely important to establish an accurate diagnosis so that the patient understands the exact cause and receives the appropriate treatment.