CASE REPORT: AUTOIMMUNE PROGESTERONE DERMATITIS

Autoimmune progesterone dermatitis is a rare skin condition with varying morphology, appearing on a monthly basis during the luteal phase of the menstrual cycle. Symptoms of APD correlate with progesterone levels during the luteal phase of the menstrual cycle. The first documented case of APD was in 1921, in which a patient’s premenstrual serum caused acute urticarial lesions. In addition, it was shown that the patient’s premenstrual serum could be used to desensitize and improve her symptoms. Progesterone levels gradually rise after ovulation, reaching a peak concentration about 7 days before menstruation, then decreasing 1 to 2 days after menses, when there is a spontaneous resolution of APD.4,5 In this condition, symptoms was found 5 days before menstruation, coincide with the peak progesterone’s concentration. The major mechanism is considered to be the exposure to exogenous and/or endogenous progesterone causing hypersensitivity reaction that leads to clinical manifestation of this disease.¹ The pathogenesis of APD remains unclear. Sexhormones modulate the immune and inflammation and inflammatory functions of cells, including mast cell degranulation. Several studies point to an autoimmune mechanism mediated by Th1 lymphocytes targeting endogenous progesterone, as in delayed hypersensitivity reactions. In women undergoing exogenous progesterone prior to APD diagnosis, Th2 cells and immunoglobulin IgE may also play a role.⁶

The clinical features of APD vary and thus far, about 80 cases have been reported in the literature. The most common clinical manifestations were eczema, urticaria, angioedema and erythema multiforme.5,7,8 Presentation of this case as an eczema is common symptom. Initially, this patient was considered to have allergic contact dermatitis. Because of its polymorphic characteristics, physicians can easily misdiagnose and thus treat this condition incorrectly.

The diagnostic criteria for APD proposed by Warin⁸ include 1) skin lesion associated with menstrual cycle (premenstrual flare); 2) a positive response to the progesterone intradermal test or reproducibility of the rash with the intramuscular test; and 3) symptomatic improvement after inhibiting progesterone secretion by suppressing ovulation. This patient has full 3 criteria for diagnosis. The intradermal test can support the diagnosis of APD based on the symptoms; on the contrary, in asymptomatic women, a positive intradermal test can result in false negative; therefore, this test might not be the most efficient diagnostic criteria for diagnosis of APD.

Treatment of APD is achieved mainly through suppressing ovulation. The first line of therapy is combined with oral contraceptives. The use of GnRH agonists has been reported successful in treatment. Another therapeutic agent used to suppress ovulation and improve symptoms is tamoxifen.⁵ Alkylated steroids, such as stanozolol and danazol, have been used to suppress ovulation, occasionally in combination with low dose corticosteroids.⁹ In this case, we have prescribed danazol to the patient 7 days before each menstrual cycle to 3 days after their period. The patient had a complete response and no relapse.