Arman Manjikian, Kush Kapadia, Yi McWhorter, Kord Strebel
Progestogen hypersensitivity (PH), historically termed autoimmune progesterone dermatitis, is an exceedingly rare condition documented almost exclusively in reproductive-age women. Although the pathophysiology remains incompletely understood, affected patients typically develop cutaneous and mucocutaneous findings, bronchospasm, and/or anaphylaxis following exposure to endogenous progesterone or exogenous progestins. Diagnosis rests on clinical history and characteristic physical findings, supported where available by progesterone skin testing, which is not standardized and has limited sensitivity and specificity. Cases in men are exceptionally rare, and the resulting scarcity of literature contributes to frequent misdiagnosis. We report a 56-year-old man with coronary artery disease, prior resection of an intestinal carcinoid tumor, and recurrent documented episodes of PH since 2006. He presented to the emergency department with a diffuse rash, facial angioedema, and nausea, and received intravenous fluids, epinephrine, antihistamines, and corticosteroids. Because his symptoms persisted and further epinephrine dosing was limited by chest pain in the setting of coronary artery disease, he was admitted to the intensive care unit for airway monitoring. Inpatient management consisted of dexamethasone, cromolyn sodium, testosterone cypionate, analgesia, and supportive care, with rapid improvement and discharge on hospital day three. This report describes a symptom-directed approach to the management of PH in a male patient in the critical care setting and considers the comorbidities and possible mechanisms that may underlie his presentation. Given the rarity of the condition and the limited literature in men, effective management currently depends on an individualized, symptom-directed strategy.