Addison's Disease

Look at this patient, who presented with weakness, loss of appetite and weight loss.

The striking abnormality is hyperpigmentation.

· Examine for hyperpigmentation:

-The hand: compare the creases with your own.

-The mouth and lips for pigmentation.

-Areas not usually covered by clothing: nipples, areas irritated by belts, straps,

collars or rings.

· Look for vitiligo.

· Tell the examiner that you would like to investigate as follows: -Examine the blood pressure, in particular for postural hypotension. -Look for sparse axillary and pubic hair.

-Examine the abdomen for adrenal scar (if the scar is pigmented, think of Nelson's syndrome and examine field defects).

· If you suspect Addison's disease, tell the examiner that you would like to do a short ACTH (1-24) test (Synacthen test).

This patient has postural hypotension, marked hyperpigmentation and sparse axillary hair (lesion) due to autoimmune Addison's disease (aetiology). The patient has marked hypoadrenalism (functional status).

The heart is small.

· Suntan.

· Race.

· Uraemia.

· Haemochromatosis.

· Primary biliary cirrhosis.

· Ectopic ACTH.

· Porphyria cutanea tarda.

· Nelson's syndrome.

· Malabsorption syndromes.

· Idiopathic (80%).

· Tuberculosis.

· Metastasis.

· HIV infection.

Graves' disease, Hashimoto's thyroiditis, primary ovarian failure, pernicious anaemia, polyglandular syndromes.

Addison's disease and hypoparathyroidism.

They are autoimmune in nature and are of two types:

· Type I: chronic mucocutaneous candidiasis, hypoparathyroidism and Addison's disease.

· Type II: Addison's disease, insulin-dependent diabetes and thyroid disease (hyper-thyroidism or hypothyroidism). This syndrome is also known as autoimmune polyendocrinopathy-candidiasis-ectodermal dysplasia.

Adrenal insensitivity to ACTH (resulting in cortisol deficiency), achalasia, alacrima and neurological disease.

· FBC (for lymphocytosis, eosinophilia).

· Electrolytes (for hyponatraemia, hyperkalaemia, hyperchloraemic acidosis, hypercalcaemia).

· Blood glucose, looking for hypoglycaemia.

· Short ACTH (1-24) (Synacthen) test; if positive, follow up with a prolonged ACTH stimulation test.

· ACTH and cortisol levels.

· Adrenal autoantibodies.

· CXR for tuberculosis.

· Plain radiograph of the abdomen for adrenal calcification.

· CT scan of the adrenals.

· Replacement steroids: prednisolone 5 mg maneand 2.5 mg nocte; adjust dose depending on serum levels and clinical well-being.

· Fludrocortisone 0.025-0.15 mg daily; adjust dose depending on postural hypotension.

· Give steroid card and Medic Alert bracelet.

· Stress the importance of regular therapy, and increase the dose in the event of stress such as dental extraction or urinary tract infection. It is also important to tell the patient that this therapy is lifelong and that an ampoule of hydrocortisone should be kept at home.

· Follow up every 6 months.

Note. In addisonian crisis, intravenous fluids and hydrocortisone should be admin-istered (after drawing a blood sample for cortisol determination).

Thomas Addison (1793-1860) qualified from Edinburgh and worked at Guy's Hospital, London. He wrote The Constitutional and Local Effects of the Disease of the Suprarenal Capsules. Armand Trosseau in Paris labelled the disease 'maladie d'Addison'. Addison also first described morphoea. The original description of the disease was reported in the following paper: Addison T 1855 Disease of the suprarenal capsules. London Med Gaz 43: 517.

John F. Kennedy, past president of the USA, reportedly had Addison's disease and was on replacement corticosteroid therapy.