Hereditary angioedema results from an inherited autosomal dominant deficiency or a functional defect of the C1 esterase inhibitor. A study to examine the influence of the steroid sex hormones found a positive correlation between the frequency of angioneurotic edema episodes and the serum progesterone level, with an increase in the incidence during the luteal phase of the menstrual cycle.
The mechanism by which progesterone influences angioedema is largely unknown. It has been hypothesized that progesterone influences the equilibrium between the coagulation and the complement cascade and, thus, enables the cleavage of the C1 esterase inhibitor by proteases. An inhibition of the synthesis of the C1 esterase inhibitor in the liver has also been discussed (50).
Wilkinson, et al. described a patient with relapsing urticaria in the premenstrual phase. In spite of the possibility of provoking such cutaneous manifestations by systemic progesterone or estrogen administration, it was not possible to demonstrate an immunological reaction to progesterone or estrogen either in the epicutaneous test or in the intracutaneous test. Therefore, it appears more likely that metabolic rather than direct autoimmunological mechanisms are responsible for triggering urticaria in the premenstrual phase. In predisposed women, independent of an autoimmunological reaction, progesterone-induced urticaria can be provoked by hormonally triggered changes in the immune system. There are indications that, as a result of a metabolic effect, increased progesterone levels in the premenstrual phase of the menstrual cycle can lead to an intensification of Type I and IV hypersensitivity reactions (51).
Was this article helpful?