Exacerbations occur whenever endogenous or exogenous levels of progestogens rise as detailed in Table 13.2. The most classic manifestation is cyclic—appearing at the end of luteal phase of the ovulatory cycle when progesterone levels are high, resolving a few days after menses [37, 38].

The condition is clinically suspected from the cyclic response and/or exacerbation due to external progestins. Confirmations of the diagnosis can be achieved using progesterone skin tests [40, 41].

The classic first line treatment is inhibition of endogenous progesterone secretion by suppression of ovulation. This can be achieved pharmacologically using estrogens, or continuous GNRH agonists. Administration of unopposed estrogens may increase the risk of endometrial carcinoma—thus limiting the use of estrogens [40]. An alternative first line treatment is desensitization with small doses of progesterone [36, 42]. This approach is appropriate for non dermal manifestations such as dysmenorrhea and premenstrual syndrome [36, 43].

The use of high dose systemic steroids is controversial both because inconsistent data regarding benefit [40] and possible side effects.

In a few patients with refractory symptoms, bilateral oophorectomy has been used. This option may succeed in controlling hypersensitivity symptoms but should be considered to be treatment of last resort [40, 44].