Herein, we describe familial autoimmune progesterone dermatitis in 3 sisters and possibly their mother. To our knowledge, no familial cases have been previously reported.
A 41-year-old white woman first experienced intermittent urticarial eruptions at around age 25 years. She described the cyclic eruptions on her extremities and trunk as brief urticarial episodes that would resolve spontaneously then reappear several weeks later. A 3-month course of oral steroids administered at around age 35 years temporarily suppressed the episodes, but once treatment was discontinued, the eruptions returned with greater severity. An intradermal skin test was performed with both progesterone and the progesterone vehicle. The patient had a positive reaction to progesterone only. It was characterized by a wheal and flare initially followed by 2 weeks of indurated erythema. The patient discontinued taking triphasic oral contraceptive pills (OCPs) and began treatment with Seasonique (Duramed Pharmaceuticals, Cincinnati, Ohio) (levonorgestrel/ethinyl estradiol combination tablets and ethinyl estradiol tablets), an extended-cycle OCP. The patient's symptoms markedly improved thereafter.
Chawla SV, Quirk C, Sondheimer SJ, James WD. Autoimmune Progesterone Dermatitis. Arch Dermatol. 2009;145(3):341-342. doi:10.1001/archdermatol.2008.605