Chronic spontaneous urticaria (CSU) is defined as persistent wheals, angioedema, or both lasting for >6 weeks due to known or unknown causes. Some epidemiological studies and case reports suggest that internal parasite infections (PI) can cause CSU. Here, we provide a systematic overview of published findings on the prevalence and relevance of PI in CSU and we discuss possible pathomechanisms. The prevalence of PI in CSU was investigated by 39 independent studies and comorbidity reportedly ranged from 0 to 75.4% (two-thirds of these studies reported infection rates of 10% or less). The prevalence of PI in adult and pediatric CSU patients ranged from 0% to 75.4% and from 0% to 37.8%, respectively. CSU patients were more often diagnosed with protozoa and had a significantly higher risk of toxocariasis seropositivity and Anisakis simplex sensitization when compared to healthy controls. Patients with chronic urticaria more frequently had seropositivity of fasciolosis, Anisakis simplex sensitization, and the presence of Blastocystis hominis allele 34 (ST3) as compared with control subjects. In 21 studies, efficacy of treatment with antiparasitic drugs ranged from 0 to 100% (35.7% of 269 CSU patients benefitted). In 9 (42.8%) of 21 studies, more than 50% of efficacy was observed. The reported rate of urticaria comorbidity in PI patients in 18 independent studies is 1-66.7%. Urticaria including CSU might be a quite common symptom of strongyloidiasis and blastocystosis. Pathogenic mechanisms in CSU due to PI may include specific IgE, Th2 cytokine skewing, eosinophils, activation of the complement, and the coagulation systems.Chronic spontaneous urticaria (CSU) is defined as the recurrent development of transient wheals (hives), angioedema (AE), or both for >6 weeks due to known or unknown causes (1). The prevalence of CSU in the general population has been estimated to range from 0.5% to 5% (1, 2). In up to 90% of CSU cases, the search for underlying causes is not successful, in routine clinical practice (1-3). Autoimmunity, food intolerance, and infections, including internal parasitic infections (PI), have been described as underlying causes of CSU (1, 4). A possible role for PI in urticaria was suggested as early as in 1949 (5): in a 16-year-old boy with Giardia lamblia and CSU. His CSU resolved completely after specific and effective treatment of the infection. Since then, infections with endoparasites, such as helminths (worms, which consist of many cells and have internal organs) and protozoa (consist of only one cell), have been discussed to cause chronic urticaria, and several clinical studies and reviews on the prevalence and relevance of PI in patients with CSU were performed and published (4,(6)(7)(8)(9)(10).Parasites are more prevalent in equatorial regions and countries with a tropical climate, high humidity, poor sanitation, dirty water, substandard and crowded housing, and in populations with low socioeconomic status (sub-Saharan Africa, Asia, Latin America, and Caribbean) (11,12). According to a ...