Achard-Thiers Syndrome

Achard-Thiers Syndrome

Article
Skin Care
Women's Health
+3
Contributed byMaulik P. Purohit MD MPHMar 18, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • Diabetes in Bearded Women
  • Diabetic-Bearded Woman Syndrome 

What is Achard-Thiers Syndrome? (Definition/Background Information)

  • Achard-Thiers Syndrome is a rare, uncommon hormonal disorder that primarily affects postmenopausal women. It is also known as Diabetes in Bearded Women
  • The syndrome is characterized by the presence of the following features:
    • Type 2 diabetes (high sugar level)
    • Excess androgen
    • Hirsutism: Excessive growth of facial or body hair in women      
    • Deep masculine voice
    • Acne
    • Enlarged clitoris
    • Obesity
    • Hyperplasia or adenoma of the adrenal gland. The adrenal glands are small, paired glands present atop the kidneys
  • This syndrome appears to be transmitted within families, but the exact mechanism of genetic transmission is not yet discovered
  • The diagnosis of Achard-Thiers Syndrome is based on the symptoms manifested by the disorder
  • The treatment of Achard-Thiers Syndrome includes managing diabetes and the use of hormonal therapies. With appropriate and effective treatment the prognosis of the condition is very good 

Who gets Achard-Thiers Syndrome? (Age and Sex Distribution)

  • Post-menopausal women are primarily affected by Achard-Thiers Syndrome
  • There is no racial, ethnic, and geographical predominance 

What are the Risk Factors for Achard-Thiers Syndrome? (Predisposing Factors)

The risk factors associated with Achard-Thiers Syndrome include:

  • Postmenopausal women
  • Obese or overweight women 

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider 

What are the Causes of Achard-Thiers Syndrome? (Etiology)

Achard-Thiers Syndrome appears to be transmitted within families. But the exact mechanism of genetic transmission is not yet understood. The possible contributing factors may include:

  • Diabetes is the main cause for this disorder and this may be due to:
    • Use of certain chemicals and drugs such as thiazide diuretics, adrenal corticosteroids, and hormonal contraceptives
    • Physiological  and emotional stress 

What are the Signs and Symptoms of Achard-Thiers Syndrome?

The common signs and symptoms associated with Achard-Thiers Syndrome include:

  • Onset of diabetes
  • Frequent urination
  • Excessive thirst and hunger
  • Weight loss

The signs and symptoms caused by overproduction of androgens may include:

  • Hirsutism: Development of excess body hair on the face, chest, and back
  • Receding hairline
  • Deepening of the voice
  • Enlargement of the clitoris
  • Infertility
  • Obesity
  • Oligomenorrhea: Infrequent or very light menstrual periods
  • Amenorrhea: Absence of menstrual periods
  • Facial hypertrichosis (excess hair on the face in males)
  • Adrenal cortical hyperplasia or adenoma 

How is Achard-Thiers Syndrome Diagnosed?

Achard-Thiers Syndrome is diagnosed using the following methods:

  • Complete physical exam with thorough medical history: The diagnosis is based on the symptoms manifested by the condition
  • The disorder can be confirmed by a 2-hour oral glucose tolerance test, which is done to check abnormally elevated glucose levels in blood
  • Blood levels of androgen, estrogen, hemoglobin A1c
  • Complete blood count
  • Thyroid function test
  • Liver function test
  • Ultrasound scan of abdomen
  • CT abdomen

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis. 

What are the possible Complications of Achard-Thiers Syndrome?

Complications due to Achard-Thiers Syndrome may include:

  • Amenorrhea: Absence of menstrual periods
  • Hypertension
  • Osteoporosis: Brittle or weak bones that can fracture easily after mild trauma
  • Atrophy or sclerosis of the ovaries; abnormal ovaries
  • Pancreatic defects
  • Abnormal liver function, liver cirrhosis
  • Signs and symptoms related to thyroid gland function

How is Achard-Thiers Syndrome Treated?

The various treatment methods for Achard-Thiers Syndrome include:

  • Diabetes may be managed through proper diet or intake of insulin or other related medications such as sulfonylureas, meglitinides, biguanides, alpha-glucosidase inhibitors, and thiazolidinediones
  • Cosmetic measures, such as waxing and electrolysis, may be helpful in removing excess hair
  • Hormone replacement therapy is often prescribed for post-menopausal women with Achard-Thiers Syndrome
  • Anti-androgens  can also be used to treat symptoms of androgen excess
  • Hormonal therapy to rectify endocrine imbalances caused by systemic diseases may also be considered 

How can Achard-Thiers Syndrome be Prevented?

Currently, there are no definitive methods available to prevent Achard-Thiers Syndrome. However, the following measures can be helpful:

  • Maintaining the correct body weight is important
  • Avoiding drugs that may cause diabetes
  • During pregnancy, care should be taken to control glucose levels and ensure proper weight management
  • Reducing cholesterol and triglyceride levels can also be beneficial
  • Reducing smoking will help to avoid complications and prevent heart diseases
  • Dietary education and weight reduction are very important factors in controlling blood pressure and diabetes. These are important contributing factors for Achard-Thiers Syndrome
  • Doing regular exercise will help to prevent obesity

What is the Prognosis of Achard-Thiers Syndrome? (Outcomes/Resolutions)

  • With proper and effective treatment the prognosis of Achard-Thiers Syndrome is very good
  • Maintaining normal glucose levels  will help in preventing complications such as retinopathy, nephropathy, and neuropathy
  • Controlling diabetes, blood pressure, and reducing or stopping smoking can result in lesser complications and a better prognosis 

Additional and Relevant Useful Information for Achard-Thiers Syndrome:

  • Emile Charles Achard and Joseph Theirs were the first to describe the syndrome, and hence, the disorder is named after them
  • Disorders that are related to Achard-Thiers Syndrome include:
    • Acquired adrenogenital syndrome
    • Empty Sella Syndrome
    • Diabetes    
    • Polycystic ovary syndrome
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Maulik P. Purohit MD MPH picture
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Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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