Tuesday, August 14, 2012

Addison's Disease

The patient who is shown below, a female of 59 years of age, presented with following signs and symptoms in Medical ward in one of the leading government hospitals in Islamabad:

* Fatigue
* Muscle weakening particularly of lower limbs
* Loss of Appetite
* Weight Loss
* Vomitting
* Constipation
* Persistent Hypotension (low blood pressure)
* Altered taste and sense of smell
* Depression
* and the most striking one was the altered skin colour. Hyperpigmentation of skin of both exposed and unexposed parts. On examination, the hyperpigmentation was appreciated more on knuckles, elbows, lips, buccal mucose and also on the palmer creases. 

According to the patient and as seen on a picture brought by the her that was taken about two years back, her skin colour was normal. She complained of generalized blackening of skin that started to appear throughout her body at the same time.


Diagnosis: Adreno-Cortical Insufficiency or Addison's Disease.

Addison's Disease, also known as Adreno-Cortical Insufficiency, is due to the destruction or dysfunction of the entire adrenal cortex. It affects particularly glucocorticoid and mineralocorticoid function. The onset of disease occurs when 90% or more of both adrenal cortices are destroyed or dysfunctional. It tends to be more common in females and children.

The most common cause of Addison's Disease is Idiopathic autoimmune adrenocortical insufficiency resulting from autoimmune atrophy, fibrosis and lymphocytic infiltration of the adrenal cortex usually with sparing of Adrenal Medulla. Idiopathic autoimmune adrenocortical atrophy and Tuberculosis account for nearly 90% of cases of Addison's disease.

Due to destruction and dysfunction of Adrenal cortex, there is diminished synthesis of glucocorticoids, mineralocordicoids and androgens. Decrease in the level of cortisol sends impulses to pituitary gland which produces ACTH in more than normal amount in order to compensate for diminished levels of cortisol in the body. But since the cortex is dysfunctional, elevated levels of ACTH are of no use. ACTH affects the melanocytes by a complex molecular mechanism which causes hyperpigmentation of the entire body.

The signs and symptoms of Addison's Disease are as follows, compare them with those of the above mentioned patient:

* Hyperpigmentation of skin - most obvious on mucous membraness, palmer creases, knuckles and extensors.

* Musle waekness, fatigue

* Weight loss, decreased appetite

* Low Blood pressure

* Craving for salt

* Nausea, Vomitting and occasional diarrhea.

* Depression

* Loss of pubic and axillary hair

* Loss of Libido.

* Hyperkalemia

* Hyponatremia

Although very rare, Addison's disease is a very important clinical condition and a case in general ward in a hospital of Islamabad, Pakistan, suggests that this autoimmune disease is no longer a rare one.

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