Canadian Association of Pathologists - Case of the Month - July, 2008
"Forensic Pathology of Sudden Death"
Diagnosis:
Acute adrenal insufficiency (Addisonian crisis) arising from autoimmune adrenalitis.
Gross and Microscopic Description:
At autopsy, the only findings were paper-thin adrenal cortices bilaterally. No skin pigmentation or other abnormalities were noted. Microscopic examination of both adrenals showed thin cortices with associated chronic inflammation. The deceased was a healthy man with no previous medical history and was not receiving steroids.
Explanation:
Acute adrenal crisis is a life-threatening state caused by insufficient levels of cortisol, which is caused by primary and secondary conditions affecting the adrenal glands. Patients typically experience lightheadedness, weakness, sweating, abdominal pain, nausea and vomiting, and can lose consciousness.
Primary adrenocortical insufficiency is rare and it occurs at any age affecting males and females equally. Secondary adrenal insufficiency from abrupt withdraw of chronic exogenous steroid use is the most common cause of adrenocortical insufficiency. Other secondary causes are: acute exacerbation of chronic adrenal insufficiency, often caused by sepsis or post-operative surgical infection, infections (tuberculosis or meningococcemia leading to Waterhouse-Friderichsen syndrome), other diseases infiltrating the adrenal glands, and hemorrhage (commonly as a complication of anticoagulants). One of most frequent causes of primary adrenal insufficiency is idiopathic atrophy (as in this case), which is likely autoimmune in origin. Other causes of primary adrenal insufficiency are: hypothalamic-pituitary disease, congenital adrenal hyperplasia, enzyme inhibitors (e.g. metyrapone), or cytotoxic agents (e.g. mitotane).
References:
- http://www.emedicine.com/emerg/topic16.htm
- American Journal of Forensic Medicine & Pathology. 20(1):60-65, March 1999. Burke, Michael Philip M.B., B.S., B.Sc., F.R.C.P.A., Dip. Forens. Path.; Opeskin, Kenneth M.B., B.S., F.R.C.P.A.