Primary adrenal insufficiency (Addison disease) - is a consequence of primary adrenal disease, which destroyed more than 90% of the cells of the cortical substance, secreting corticosteroids. In primary adrenal insufficiency gradually developed generalized hyperpigmentation, which is often mistaken for surviving the summer tan. The skin becomes even bronze color, darken the areola, the skin of the external genitalia, birthmarks, wrinkles on the palms and soles, as well as more recent scars. At the solid sky and on the gums appear blue-black spots giperpigmentirovannye. It is believed that the hyperpigmentation is caused by excessive secretion of MSH. Pubic hair in the armpits thinning. At the nails may appear dark giperpigmentirovannye band. Primary adrenal insufficiency is often combined with vitiligo.
The most common cause of primary adrenal insufficiency is autoimmune lesion of the adrenal cortex (approximately 80% of cases). Autoimmune adrenal failure may be associated with other autoimmune endocrine diseases, such as insulin dependent diabetes mellitus and hypothyroidism, and sometimes hypogonadism gipoparatireozom and autoimmune gastritis (APGS type I).
Primary adrenal insufficiency leads to hypoglycemia, extremely high sensitivity to insulin, intolerance of stress, anorexia, weight loss, nausea and a pronounced weakness. In patients with Addison disease have low blood pressure, as well as reduced glomerular filtration rate and ability to cope with the load with water. Often there craving for salt. The level of Na in the plasma of these patients decreased, and the level of K increased, increased the number of lymphocytes in the blood. These patients are often enhanced pigmentation of the skin and mucous membranes, due to a compensatory increased secretion of ACTH and related gene products POMK.
