Achard Thiers syndrome is an extremely rare endocrine disorder characterized by hyperandrogenism and hyperinsulinemia. It is primarily seen in postmenopausal women and was initially described as "diabetes mellitus in bearded women", reflecting the excess androgen manifesting as hirsutism. Additional symptoms include polyuria, polydipsia, weight loss, and menstrual irregularities - oligomenorrhea and amenorrhea. The diagnosis rests on a thorough clinical and biochemical assessment. The oral glucose tolerance test (OGTT) and a hormonal workup are crucial for discriminating this syndrome from classical diabetes mellitus or other hormonal disorders.
Described almost 100 years ago, Achard Thiers syndrome is now rarely mentioned in the literature, primarily because of its infrequent occurrence, underdiagnosis, and poor understanding of the pathogenesis [1] [2] [3] [4]. Some of the first authors who described it, termed the disorder "diabetes mellitus of bearded women", as women who suffered Achard Thiers syndrome had simultaneous problems with glucose metabolism and adrenocortical hormones (the name hyperinsulinemic hyperandrogenism is also mentioned in some studies) [1] [2] [3]. The clinical presentation of Achard Thiers syndrome is comprised of the main symptoms of diabetes on one hand (polyuria, polydipsia, etc.), and signs of hyperandrogenism on the other (hirsutism, especially on the head and neck; menstrual changes such as oligomenorrhea and amenorrhea, clitoral enlargement, infertility, and obesity) [5]. Postmenopausal women are identified as the main patient population [3] [5]. Some studies have emphasized the primary role of adrenocortical disease in the etiology of insulin resistance seen in patients with Achard Thiers syndrome, and have remarked that diabetes mellitus is not related to the changes that normally develop in the pancreas [4] [5]. In addition, psychiatric symptoms and disorders were noted in the first patients described with this condition [5]. Acanthosis nigricans is a common symptom of Achard Thiers syndrome as well. Another condition characterized by hyperandrogenism and hyperinsulinemia is the polycystic ovary syndrome or PCOS, which is usually identified in young women.
A detailed patient history and a thorough physical examination are the two crucial steps needed for the diagnosis of Achard Thiers syndrome. Physicians should inquire about the onset of abnormal hair growth and evaluate the onset, progression, and severity of additional symptoms. The physical examination should confirm the previously mentioned findings and provide solid evidence for a detailed laboratory investigation. The OGTT test is sufficient to detect hyperinsulinemia, while a complete androgen hormone panel is recommended for assessing the adrenal gland function. Because obesity and hypertension are frequently present, a comprehensive cardiologic examination through blood pressure measurements and electrocardiography (ECG) is often made. Imaging studies, such as ultrasonography or computed tomography (CT) may be of great benefit in determining the underlying cause of Achard Thiers syndrome, as intrinsic adrenocortical pathologies (e.g. carcinoma) have been described in its pathogenesis [5].