Autoimmune progesterone dermatitis that changed its clinical manifestation from anaphylaxis to fixed drug eruption-like erythema

摘要

Autoimmune progesterone dermatitis (APD) is a rare periodic premenstrual reaction to progesterone with variable clinical manifestations, such as erythema multiforme, eczema, fixed drug eruption, urticaria, angioedema and anaphylaxis.1 Although detailed pathological mechanisms remain unknown, allergic reactions to endogenous progesterone has been thought to be involved. Here, we present a case of APD that developed initially as anaphylaxis, and then turned to a fixed drug eruption-like erythematous skin lesion after an i.d. challenge test with progesterone.A 21-year-old woman had experienced a recurrent history of monthly systemic urticarial eruptions and dyspnea for 7 months. The symptoms occurred as urticarial rash with pruritus all over the body at the beginning of the menstrual period, and were occasionally associated with severe respiratory distress. She had been treated with systemic administration of steroid in an emergency department. We suspected a diagnosis of APD, and performed a challenge test with progesterone (Progehormon; Mochida, Tokyo, Japan) after the informed consent was obtained from the patient. A total 20 (iL of progesterone (10 ng/j.iL) was injected i.d. on the left forearm for the challenge test. Within 1 min of the injection, pruritus was induced at the challenged skin area, followed by erythema and swelling (Fig. 1a), which disappeared the next day. We diagnosed her as having APD. After the challenge test, she did not develop any urticarial eruption or respiratory distress at the beginning of the menstrual period. Instead, she began to develop severe edematous erythema with pruritus at the left forearm where the progesterone challenge test was performed (Fig. 1b). She was treated with topical administration of steroid ointment, and the eruption regressed with pigmentation a few days later (Fig. 1c). Since then, the fixed drug eruption-like erythema without anaphylaxis appeared on the same site of her left forearm at the beginning of each menstrual period. We performed follow up for 6 months after the challenge test, and the skin eruption was controlled well with the topical treatment of steroid in the meantime.