Achard–Thiers syndrome

Overview

Achard–Thiers syndrome, also known as the diabetic-bearded woman syndrome, is a rare endocrine disorder that affects postmenopausal women. It is characterized by a combination of type 2 diabetes mellitus and signs of androgen excess, such as hirsutism (excess facial/body hair), deepening voice, and occasionally virilization. The syndrome represents a form of hyperandrogenism that develops later in life and may be associated with adrenal or ovarian dysfunction. It was first described in the early 20th century by French physicians Émile Achard and Joseph Thiers.

Causes

The exact cause of Achard–Thiers syndrome is not fully understood, but it is believed to result from increased androgen production by the adrenal glands or ovaries in postmenopausal women. The disorder is most often seen in women with underlying insulin resistance or diabetes, which may influence androgen metabolism. Potential contributing factors include:

• Adrenal hyperplasia or adenomas (benign tumors)

• Ovarian tumors or stromal hyperthecosis (increased ovarian stromal activity)

• Insulin resistance and metabolic syndrome

Symptoms

Achard–Thiers syndrome typically presents in postmenopausal women with a combination of metabolic and androgenic signs. Common symptoms include:

• Type 2 diabetes mellitus

• Hirsutism: Excess hair growth on the face, chest, or back

• Voice deepening

• Clitoromegaly: In some cases, as a sign of virilization

• Alopecia: Thinning or loss of scalp hair

• Obesity or central weight gain

• Hypertension

• Menstrual history: Typically postmenopausal at onset

Some women may also exhibit signs of Cushingoid features if cortisol production is involved.

Diagnosis

Diagnosis of Achard–Thiers syndrome involves a combination of clinical assessment, laboratory tests, and imaging studies. Key diagnostic steps include:

• Blood glucose tests: Fasting glucose or HbA1c to confirm diabetes

• Serum androgen levels: Elevated total testosterone or DHEA-S levels

• Hormonal profile: Including cortisol, LH, FSH, and insulin levels

• Imaging: CT or MRI scans of the adrenal glands and ovaries to detect tumors or hyperplasia

• Clinical history: Assessment of symptom onset, menstrual history, and family background

Treatment

Treatment of Achard–Thiers syndrome targets both the underlying endocrine disturbance and the metabolic complications. Management options include:

• Diabetes management: Lifestyle modification, oral antidiabetic drugs (e.g., metformin), or insulin therapy

• Anti-androgen therapy: Medications such as spironolactone or cyproterone acetate to reduce hirsutism

• Surgical removal: Of adrenal or ovarian tumors if identified and hormone-secreting

• Weight loss and diet: To improve insulin sensitivity and reduce androgen levels

• Laser hair removal or electrolysis: For cosmetic management of hirsutism

Prognosis

The prognosis for women with Achard–Thiers syndrome varies depending on the severity of diabetes and the underlying source of androgen excess. With appropriate management of blood glucose and hormonal imbalance, most women can achieve good control of their symptoms. If a tumor is the source of hormone production, surgical treatment often results in significant improvement. However, if left untreated, the syndrome may lead to complications associated with poorly controlled diabetes and cardiovascular risk.