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

Abstract

Autoimmune progesterone dermatitis that changed its clinical manifestation from anaphylaxis to fixed drug eruption-like erythema Dear Editor, 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 lL of progesterone (10 lg/lL) 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.Skin immune responses are dynamic. 2,3 It has been suggested that both T-helper (Th)1-and Th2-polarized immune responses to endogenous progesterone are involved in the pathogenesis of APD. Immunoglobulin E antibodies to progesterone were detected in some cases of APD with anaphylaxis, 1,4 while progesterone-specific Th1 or cytotoxic T cells were detected in other APD patients with erythema multiforme-like lesions. 5 In our case, the patient first developed APD with urticarial rash and severe respiratory distress, which developed into fixed drug eruption-like erythema after an i.d. challenge test. It remains unclear whether a shift of immune responses (e.g. from Th2 to Th1) occurred in the current case, because we cannot provide evidence to supp...

Autoimmune progesterone dermatitis mimicking fixed drug eruption

“…Marked by great clinical variability, the most common manifestations of APD are urticaria and erythema multiforme. Fixed drug eruption-like lesions, as in our patient, have previously only been reported in two Japanese patients [ 3,4 ] . Fixed drug eruption-like lesions, as in our patient, have previously only been reported in two Japanese patients [ 3,4 ] .…”

“…Other presentations previously described include eczema, pompholyx, stomatitis, erythema annulare centrifugum, and even anaphylaxis [ 2 ] . Fixed drug eruption-like lesions, as in our patient, have previously only been reported in two Japanese patients [ 3,4 ] . Such lesions require taking a detailed medication history as well as a progesterone challenge test in order to differentiate between APD and FDE.…”

The histopathologic features of autoimmune progesterone dermatitis

“…The most commonly reported clinical presentations include eczematous dermatitis, urticarial lesions or erythema multiforme. Clinical features of fixed drug, erythema annulare centrifugum, vesicular, bullous, angioedema and anaphylaxis have been less commonly reported . Mucosal lesions have been commonly reported and occasionally are the sole area involved but more frequently the mucosal surfaces are uninvolved.…”

Presentation and natural history of progestogen hypersensitivity

“…Respiratory and vascular involvement ranges from bronchospasm 6 to anaphylaxis. 7,40,46 Cutaneous symptoms may be concurrently present 22,40,47 or absent. There are no reports of isolated gastrointestinal symptoms or gastrointestinal and cutaneous symptoms without respiratory involvement.…”