ADRENOCORTICAL INSUFFICIENCY: Facts and Awareness

Adrenocortical insufficiency occurs when the adrenal cortex secretes inadequate amounts of adrenocortical hormones, primarily glucocorticoids and mineralocorticoids. The disorder occurs in two forms in adults and children. Primary adrenocortical insufficiency (Addison's disease) results from destruction and subsequent hypofunction of the adrenal cortex, usually caused by an autoimmune process. Presentation may be insidious or acute. Secondary adrenocortical insufficiency occurs because of adrenocorticotropic hormone (ACTH) deficiency from pituitary disease, or from suppression of the hypothalamic-pituitary axis by corticosteriods administered to treat nonendocrine disorders, which causes the adrenal cortex to atrophy.


Inadequate aldosterone produces disturbances of sodium, potassium, and water metabolism. Cortisol deficiency produces abnormal fat, protein, and carbohydrate metabolism. Absence of cortisol during a period of stress can precipitate addisonian (acute adrenal) crisis, an exaggerated state of adrenal cortical insufficiency, which is fatal if not immediately treated.



Physical findings may include the following:

• Patients with acute adrenal insufficiency generally present with acute dehydration, hypotension, hypoglycemia, or altered mental status. These signs usually occur in an acutely ill patient with sepsis or disseminated intravascular coagulation or in a patient after a traumatic delivery.
• Patients with chronic adrenal insufficiency may have increased skin pigmentation, particularly in the areolae and genitalia, as well as any scars or moles. Recent scars are typically affected most often. Areas unexposed to sun (eg, palmar creases, axillae, areolae) are often hyperpigmented, which may help to distinguish hyperpigmentation from sun tan. The patient may also have pigmentary lines in the gums. See the images below.
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  Left photograph shows hyperpigmentation on the dorsum of the hand before the treatment of primary adrenal insufficiency. Right photograph shows normal pigmentation after treatment.

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  Left photograph shows a patient with Addison disease who has prominent pigmentation in areas not exposed to the sun, such as the palmar creases. Right photograph shows normal pigmentation after treatment.

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  Left photograph shows vitiligo in a patient with autoimmune adrenalitis. Right photograph shows an area of hyperpigmentation surrounding the vitiligo.

• Signs of weight loss may be evident. If the patient is not frankly hypotensive, he or she may have orthostatic hypotension.
• Some patients lose pubic and axillary hair because adrenal androgens support growth of body hair in these areas.
• Wolman disease (OMIM 278000), an autosomal recessive disorder caused by a deficiency of lysosomal acid lipase, may present with adrenal calcifications. Adrenal calcifications may be seen on plain radiography or CT scanning of the adrenal glands.