Hair

A Case of Absent Pubic and Axillary Hair — Donovan Hair Clinic


I enjoyed reading a quiz case in this month’s European Journal of Internal Medicine.

Let’s take a look at the quiz together!

The authors present the case of a 39-year-old female who presents with presents with  fatigue, extreme tiredness, muscle pain and shortness of breath about 6 months.

After obtaining more history from the patient, it was noted that the patient had not had menses for 19 years –  since her last pregnancy. She reported that she had a home delivery 19 years ago with post partum haemorrhage that required hospitalization and three units of blood transfusion.

Clinical examination showed skin pallor with no axillary hair and no pubic hair.

Lab tests showed hyperprolactinemia, secondary hypocortisolism, secondary hypothyroidism with a low FT4 and low FT3  and an inappropriately normal thyroid-stimulating hormone (TSH).  She also had central hypogonadism with a low estradiol an inappropriately low luteinizing hormone (LH)  and follicle-stimulating hormone (FSH)]. An MRI of the pituitary showed revealed a low volume pituitary with an empty sella.

The quiz question then asks readers – What is the diagnosis?

ANSWER:

The correct answer is Sheehan syndrome (SS). The patient was commenced on corticosteroids (hydrocorticosterone) and replacement of thyroid, oestrogen and progesterone hormones with dramatic improvement of her symptoms.

 

What is Sheehan Syndrome?

Sheehan syndrome refers to anterior pituitary necrosis that occurs after post-partum haemorrhage/hypovolemia.  The loss of blood leads to damage to the anterior pituitary gland and the result is that the anterior pituitary becomes impaired in its ability to make hormones.  Sheehan syndrome is also called post-partum pituitary necrosis.  The posterior pituitary is not affected by blood loss and hypovolemia in the same way and so Sheehan syndrome tends to affect the anterior pituitary only.

The clinical syndrome of hypopituitarism may occur immediately (Acute form) or years later (chronic form)

Advances in obstetrical care over the last decades have reduced in the incidence of Sheehan syndrome in the world.  However, some studies such as Karaca et al 2016 estimated the incidence of Sheehan syndrome as high as five patients out of 100,000 births. It may be higher in developing countries than developed countries.

 

Symptoms of Sheehan syndrome

 

Symptoms of Sheehan syndrome can best be understood by recognizing the hormones that are normally produced by the anterior pituitary gland. These include adrenocortico stimulating hormone (ACTH), thyroid stimulating hormone (TSH), growth hormone (GH), follicle stimulating hormone (FSH), luteinizing hormone (LH) and prolactin (PRL). A lack of these hormones leads to second deficiencies including hypothyroidism, hypoadrenalism and ovarian insufficiency.  There may be a true pan hypopituitarism syndrome where all hormones are affected or a more selective loss.

Remarkably there is a certain order that hormones are affected when necrosis occurs. Certain hormones are more likely to become deficient first and others become affected later.  These are (in order of first to last) GH affected first, followed by PRL, FSH, LH, ACTH, and then TSH last.

There are two presentations of Sheehan syndrome. There can be an acute presentation of Sheehan syndrome or a chronic presentation. The chronic presentation is more common meaning that the symptoms develop months to years after the post-partum hemorrhage. In the acute presentation, which is less common symptoms develop immediately after childbirth.

The authors point out that there can be a long diagnostic delay in identification of Sheehan syndrome. In developed countries this may be as much as 9 years on average and in developing countries as much as 20 years.

 

Acute Sheehan Syndrome

Of the most common symptoms of acute Sheehan syndrome is the inability to breastfeed, which is called agalactorrhea. Acute Sheehan syndrome can be very serious. Patients have persistently low blood pressure and tachycardia which often tricks the clinician into believing this is due to ongoing hypovolemia and shock. It’s only when the astute clinician recognizes that there is hyponatremia and hypoglycemia that there is a recognition of a more widespread endocrine problem and the diagnosis of Sheehan syndrome is finally made.

 

 

Chronic Sheehan Syndrome

 Typical symptoms of chronic Sheehan syndrome occurs months to years after the childbirth. Symptoms may include amenorrhea or oligomenorrhea (reduced LH, FSH), hot flashes (low estrogen), and/or decreased sex drive (low androgens). The lack of TSH may lead to hypothyroidism and this may often occur months or years after childbirth. Patients have symptoms of fatigue, bradycardia, hypotension, cold intolerance, weight gain, and constipation. Adrenal insufficiency can also occur with symptoms of fatigue and weight loss and hyponatremia, hypoglycemia and anemia.    

There may be loss of  axillary and pubic hair in Sheehan syndrome. The authors stated that the absence of adrenal and gonadal androgens that resulted in the lack of pubic and axillary hair.

Final Points

The key to this case is the reminder that irregular periods or absent periods are important to take note of. They are potentially an important sign of an underlying endocrine issue.

All women with irregular periods or absent periods need blood tests for CBC, TSH, estrogen, LH, FSH, prolactin, AM cortisol. Growth hormone and IGF-1 can be added.

In the case of suspected Sheehan syndrome,  other tests like sodium levels and blood sugar levels are added.

The finding a low basal hormone level along with a history and physical suggestive of Sheehan syndrome would help to make the diagnosis.

An MRI is often done to confirm the diagnosis. An empty sella is present in about 70% of patients, and a partially empty sella is present in about 30% of patients on a later MRI.

Treatment is with lifelong replacement of the deficient hormones.  

REFERENCE

Anand K Annamalai et al. Secondary amenorrhoea with absent axillary hair. Eur J Intern Med. 2023 Mar 11;S0953-6205(23)00082-1.



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