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Author: Dr Amanda Oakley MBChB FRACP, Dept of Dermatology Waikato Hospital, 2004.
Addison disease is a hormonal disorder resulting from a severe or total deficiency of the hormones made in the adrenal cortex. The adrenal cortex is the outer portion of the adrenal gland, of which there are two, located above each kidney. Two important hormones made by the adrenal cortex are cortisol and aldosterone. They are both essential for the normal functioning of the body. In particular, aldosterone regulates salt and water levels which affect blood volume and blood pressure.
Near total or total destruction of the adrenal glands results in neither of these hormones being produced and a condition called primary adrenal insufficiency or Addison disease (first described by Dr Thomas Addison in 1855).
There are several causes of Addison disease:
The signs and symptoms of Addison disease are generally non-specific and include fatigue, weakness, weight loss, nausea, abdominal pain, diarrhoea, vomiting and mood disturbances. These symptoms steadily worsen over time due to the slowly progressive loss of cortisol and aldosterone production.
The following skin signs may be indicative of Addison disease and should prompt further investigations and appropriate tests.
Due to the slowly progressive chronic symptoms, the diagnosis is often not made until abnormal results from routine blood tests are found. If the diagnosis has not been suspected, the patient may have an Addisonian crisis, triggered from a sudden event like a ‘flu virus, accident, or surgery. An Addisonian crisis is a medical emergency and requires immediate treatment with intravenous hydrocortisone.
If Addison disease is suspected, tests measuring cortisol and aldosterone blood and urine levels must be performed to make a definitive diagnosis.
Treatment of Addison disease is with replacement steroid hormones that are tapered to suit the physiological requirements of the individual patient.
Treatment with replacement hormones is life-long and can never be stopped. Routine care should include regular doctor's visits, special attention and possibly extra medication during times of illness, and an awareness of circumstances that may trigger an adrenal crisis, e.g. dehydration/salt loss during vigorous exercise.
Patients with Addison disease should wear an identification bracelet or necklace (e.g. Medic Alert) stating that they have the disease, to ensure proper emergency steroid treatment is given when necessary.
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