Addison's disease (Addison's syndrome)

Addison's disease - primary adrenal insufficiency; bilateral adrenal destruction by TB used to be most common cause, now only accounts for 7-20% of cases; now autoimmune disease 70-90%, remainder caused by infectious disease, mets or lymphoma, adrenal hemorrhage, infarction, or drugs.

Addison disease may coexist with diabetes mellitus or hypothyroidism in polyglandular deficiency syndrome. In children, the most common cause of primary adrenal insufficiency is congenital adrenal hyperplasia, but other genetic disorders are being increasingly recognized as causes.

Addison's disease. Hyperpigmentation

On the picture - Addison's disease. Hyperpigmentation representing an accentuation of normal pigmentation of the hand of a patient with Addison's disease (left). For comparison, the hand of a normal individual, matched for ethnic pigmentation, is shown on the right.

More details: 📖 Harrison’s Endocrinology 4th Edition

Thomas Addison (1795 - 1860), English physician.

Thomas Addison

The adrenal glands were described by Eustachius in 1714, but it was many years before their function was elucidated. Indeed, the offer of a substantial prize for an essay on adrenal physiology, made by the Académie des Sciences de Bordeaux in the eighteenth century failed to elicit any significant entries.

The story of Addison's disease begins with Addison's first description in a short note in an article in the London Medical Gazette entitled Anaemia - disease of the suprarenal capsules in which the disease is not distinctly separated from a new form of anemia.

This article was followed up in his monograph On the Constitutional and Local Effects of Disease of the Suprarenal Capsule which was published in London in 1855 and represented the beginning of the study of the endocrine glands. This work was much debated in England and Scotland and largely discounted, John Hughes Bennett (1812-1875) in Edinburgh denying the existence of the disease. Armand Trousseau (1801-1867) in Paris, however, was quick to recognise adrenal failure and gave it the eponym Addison's disease.

In his book Addisons points out that it was really under his attempts at elucidating the base of a peculiar form of anaemia that he happened to find pathological changes comprising both suprarenal glands. He maintains that the disease of the adrenal glands could not be connected with the anaemia, as he had previously thought. His descriptions of the symptoms of the eleven patients with enlarged adrenal cortex collected by himself and his younger associate Samuel Wilks is worth quoting:

"The leading and characteristic features of the morbid state to which I would direct your attention are, anaemia, general languor and debility, remarkable feebleness of the heart's action, irritability of the stomach, and a peculiar change of the colour in the skin, occurring in connection with a diseased condition of the suprarenal capsules . . .

The discoloration pervades the whole surface of the body, but is commonly most strongly manifested on the face, neck, superior extremities, penis, scrotum, and in the flexures of the axillae and around the navel."

One of the eleven patients in the monograph is of special interest. This patient had been treated by Bright, who had noted pigmentation of the skin, the irritability of the stomach, the emaciation, and the asthenia which quickly lead to the petient's death. He also described the characteristic changes: the very enlarged adrenal glands with deposits of a "scrofulous kind", and partial deterioration of the glands with deposits of pus.

Bright seems to have been confused by the fact that the patient also had tumors of the chest and a swelling of parotis, and he did not connect the subsequent classic symptology with the changes of the adrenal glands. This description came many years before Addion's monograph and, if Bright had understood the connection his name, not Addison's, would have been attached to the disease.

References

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