Autoimmune progesterone dermatitis (APD) is a rare skin condition caused by sensitivity to high levels of progesterone secreted during the luteal phase of the menstrual cycle. This may be due to various pathophysiological mechanisms including a Type I and Type IV hypersensitivity reaction. Here we present the case of a patient with APD whose episodic flares were controlled by the addition of omalizumab, after a bilateral oophorectomy failed to resolve her symptoms. A 34-year-old female presented to our Endocrine clinic with marked Cushingoid features secondary to high-dose oral prednisone prescribed for APD diagnosed 6 years earlier. She first developed a pruritic maculopapular rash on her arms and legs just after the birth of her second child in 2009. The rash was also associated with headaches and diffuse angioedema. Symptoms occurred for 1–2 weeks, in a cyclical fashion, during the luteal phase of each menstrual cycle and subsided within a few days after menses. The severity of symptoms increased as time went on, and flare-ups began to also include dyspnea, nausea, vomiting and abdominal pain. Her symptoms improved with administration of oral prednisone, but she continued to experience breakthrough symptoms. After multiple failed treatment modalities, she elected bilateral oophorectomy in 2018. However, her symptoms of APD persisted and she still required high-dose oral prednisone. Her condition was further complicated by vasomotor menopausal symptoms and progressive iatrogenic Cushing’s syndrome. She eventually was started on Omalizumab, which suppressed further recurrences of APD symptoms and allowed her to wean off prednisone. Vasomotor menopausal symptoms responded well to the addition of conjugated estrogens with bazedoxifene. However, her symptoms of diffuse bony pain and arthralgias which started whilst on prednisone have persisted in spite of discontinuing prednisone. To our knowledge, this is only the third case of APD which was successfully treated with Omalizumab and the first case where a bilateral oophorectomy failed to resolve symptoms of APD in the literature. This case also demonstrates the complications of vasomotor menopausal symptoms secondary to a bilateral oophorectomy, as well as the adverse effects of long-term glucocorticoid therapy.

中文翻译：

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奥马珠单抗治疗双侧卵巢切除难治性自身免疫性黄体酮皮炎的疗效：1例报告

自身免疫性黄体酮皮炎 (APD) 是一种罕见的皮肤病，由对月经周期黄体期分泌的高水平黄体酮敏感引起。这可能是由于各种病理生理机制，包括 I 型和 IV 型超敏反应。在这里，我们介绍了一个 APD 患者的病例，在双侧卵巢切除术未能解决她的症状后，通过添加奥马珠单抗来控制其发作性发作。一名 34 岁女性就诊于我们的内分泌诊所，继发于 6 年前诊断为 APD 的大剂量口服泼尼松后，出现明显的库欣样特征。2009 年她的第二个孩子出生后，她的胳膊和腿上首次出现瘙痒性斑丘疹。皮疹还与头痛和弥漫性血管性水肿有关。症状在每个月经周期的黄体期以周期性方式发生 1-2 周，并在月经后几天内消退。症状的严重程度随着时间的推移而增加，突然发作还包括呼吸困难、恶心、呕吐和腹痛。口服泼尼松后，她的症状有所改善，但她继续出现突破性症状。在多次失败的治疗方式后，她于2018年选择了双侧卵巢切除术。然而，她的APD症状仍然存在，仍然需要大剂量口服泼尼松。她的病情因血管舒缩性更年期症状和进行性医源性库欣综合征而进一步复杂化。她最终开始使用奥马珠单抗，这抑制了 APD 症状的进一步复发，并让她摆脱了泼尼松。血管舒缩性更年期症状对添加结合雌激素和巴多昔芬反应良好。然而，尽管停用泼尼松，她在服用泼尼松期间开始出现的弥漫性骨痛和关节痛症状仍然存在。据我们所知，这只是第三例使用奥马珠单抗成功治疗的 APD 病例，也是文献中第一例双侧卵巢切除术未能解决 APD 症状的病例。该病例还证明了继发于双侧卵巢切除术的血管舒缩性更年期症状的并发症，以及长期糖皮质激素治疗的不良反应。尽管停用了泼尼松，但她在服用泼尼松期间开始出现的弥漫性骨痛和关节痛症状仍然存在。据我们所知，这只是第三例使用奥马珠单抗成功治疗的 APD 病例，也是文献中第一例双侧卵巢切除术未能解决 APD 症状的病例。该病例还证明了继发于双侧卵巢切除术的血管舒缩性更年期症状的并发症，以及长期糖皮质激素治疗的不良反应。尽管停用了泼尼松，但她在服用泼尼松期间开始出现的弥漫性骨痛和关节痛症状仍然存在。据我们所知，这只是第三例使用奥马珠单抗成功治疗的 APD 病例，也是文献中第一例双侧卵巢切除术未能解决 APD 症状的病例。该病例还证明了继发于双侧卵巢切除术的血管舒缩性更年期症状的并发症，以及长期糖皮质激素治疗的不良反应。