Hyperpigmentation with hypoglycemia

The child has 2 episodes of hypoglycemia associated with hyperpigmented skin. Thus one must consider Addison’s disease or hypocortisolism in this child. It is unlikely to be congenital adrenal hyperplasia as there is no blood pressure abnormality, dehydration or ambiguous genitalia. The serum cortisol in this child was undetectable (<1mcg/dl, normal 5-25 mcg/dl) and serum ACTH was elevated to ten times normal value (664pg/ml, normal 10-85pg/ml). Thus, the diagnosis was confirmed to be a primary adrenal insufficiency. Her CT adrenals were normal. Her serum cholesterol was 116mg/dl ruling out Wolman’s disease.