Rapidly Progressive and Severe Hirsutism from Hyperreactio Luteinalis within a Background of Polycystic Ovary Syndrome

Abstract

Objective: Gestational trophoblastic disease and hyperreactio luteinalis (HL) are rare, but important, etiologies of hyperandrogenism that need to be further studied. Methods: We present a case of rapidly progressing hirsutism and marked biochemical androgen excess in the context of pregnancy. Results: A 26-year-old woman with a past medical history of obesity, prediabetes, and polycystic ovary syndrome presented with worsening hirsutism and markedly elevated testosterone levels. She was subsequently found to be pregnant, with extremely elevated levels of serum β-human chorionic gonadotropin. Subsequent work-up led to the identification of molar pregnancy and bilaterally enlarged ovaries, suggestive of HL. Following surgical intervention and therapy with methotrexate for invasive mole, she experienced improvement in both biochemical and clinical androgen excess features. Conclusion: With the prevalence of polycystic ovary syndrome, many women present to medical providers with hirsutism or other findings of hyperandrogenism. However, rapid progression of existing hirsutism or severe hirsutism should prompt more extensive evaluations to rule out rare etiologies. One such etiology found in pregnancy is HL, in which high levels of β-human chorionic gonadotropin can stimulate production of benign theca lutein cysts, leading to marked hyperandrogenism and virilizing symptoms. Abbreviations: 17-OHP = 17-hydroxyprogesterone; β-hCG = β-human chorionic gonadotropin; DHEA-S = dehydroepiandrosterone-sulfate; GTD = gestational trophoblastic disease; HL = hyperreactio luteinalis; MRI = magnetic resonance imaging; PCOS = polycystic ovary syndrome; SHBG = sex hormone-binding globulin; TSH = thyroid-stimulating hormone