Nelson's syndrome

Nelson syndrome is a neuroendocrine pathology characterized by chronic adrenal insufficiency, hyperpigmentation of the skin and mucous membranes, and the presence of pituitary adenoma (corticotropinoma). This syndrome can be considered as a complication of the treatment of Cushing’s disease by bilateral adrenalectomy, or long-term therapy with inhibitors of the adrenal cortex function.
The clinical pattern is characterized by corticotrophic hypersecretion of proopiomelanocortin, the precursor of ACTH (adrenocorticotropic hormone) and MSH (melanostimulating hormone).

Symptoms of Nelson’s syndrome

Skin hyperpigmentation is the earliest and most permanent symptom, because Nelson syndrome causes a significant increase in the production of ACTH and MSH. The severity of hyperpigmentation may decrease with adequate hormone replacement therapy.
Symptoms of chronic adrenal insufficiency include arterial hypotension, weakness, drowsiness, sometimes developing into the addisonic crisis signs (drop in blood pressure, fever, abdominal pain, severe weakness, nausea, etc.)

Treatment of Nelson’s syndrome

The main method of treating Nelson’s syndrome is surgery, with a combination of drug therapy (correction of adrenal insufficiency) and radiation therapy.