000531 - Autoimmune progesterone dermatitis in two sisters

Autoimmune progesterone dermatitis is a rare hypersensitivity disorder characterized by recurring dermatologic manifestations during the menstrual cycle in women. It presents with a variety of dermatologic and allergic signs and symptoms including recurrent and cyclical urticaria, anaphylaxis, dermatitis, and other skin manifestations. There are no definitive diagnostic laboratory tests or specific histopathologic findings for the disease. Intradermal progesterone tests show variable results and the diagnostic accuracy. The pathogenesis of this disorder is unclear, however, it is usually considered as an immune reaction to increased levels of endogenous progesterone during the luteal phase of the menstrual cycle. We report familial autoimmune progesterone dermatitis in two sisters with positive intradermal tests.

A 28-year-old woman (patient 1) developed recurrent perioral and hand dermatitis one year ago. Her cutaneous symptoms occurred during her menstruation. A 27-year-old woman (patient 2), the younger sister of patient 1, experienced erythematous rashes with severe pruritus on the breast and the perioral area during her menstruation. The symptoms got worse every time she menstruates for 2 years. The lesions occurred on the first day of the menstrual cycle, and gradually improved with hyperpigmentation on days 3~4 of the menstrual cycle.in both patients. Skin prick testing using 50mg/mL progesterone was negative, however, intradermal testing (5mg/ml and 50mg/ml) was positive in two patients (Fig. A and B). On challenge testing with intramuscular progesterone, patient 1 showed no response, but patient 2 developed skin rash on extremities and trunk (Fig. C and D) after 3 days and erythematous swelling on the site of intradermal testing (Fig. E) after 10 days. Patient 2 started a combined oral contraceptives therapy with dirospirenone (3mog) and ethinylestradiol (0.02mg) 2 months ago.   

Here, we report two cases of autoimmune progesterone dermatitis occurred in two sisters confirmed by intradermal testing.