Symptoms of Itenko-Cushing's disease

In patients, the skin is thin, dry, on the face, in the chest and back of the purple-cyanotic color. The venous pattern on the chest and legs is clearly visible. There is acrocyanosis. On the skin of the abdomen, the inner surfaces of the shoulders, thighs, in the region of the mammary glands appear broad striae of red-violet. Often there is hyperpigmentation of the skin, often in places of friction. On the back, chest and face there are pustular eruptions, hypertrichosis. Hair on the head often falls out, and baldness in women is noted in the male type. There is an increased tendency to furunculosis and development of erysipelas.

There is an excessive deposition of fat in the neck, trunk, abdomen and face, which becomes a "full moon". In the area of the upper thoracic vertebrae, there are fat deposits in the form of a hump. The limbs are thin, lose their normal shape.

Obesity of varying degrees in the Itenko-Cushing disease is observed in more than 92% of cases. In patients with a lack of obesity, a redistribution of subcutaneous fat with a predominant location in the chest and abdomen is expressed.

Arterial hypertension is one of the earliest and permanent symptoms in the Itenko-Cushing disease. Both systolic and diastolic pressure increase. Often raising blood pressure for a long time is the only symptom of the disease. Changes in the cardiovascular system are often leading in the clinic of the underlying disease and are the cause of disability and the majority of deaths.

Often, the syndrome of hypertension is complicated by the damage to the vessels of the retina of the eyes, heart, kidneys, as in hypertension. Patients with Isenko-Cushing's disease, as a rule, experience cardiovascular insufficiency with tachycardia, arrhythmia, dyspnea, swelling, and enlargement of the liver. Metabolic and electrolyte shifts, predominance in the cardiac muscle of processes of protein breakdown and hypokalemia contribute in patients with hypertension to the rapid development of circulatory insufficiency.

In most patients, various ECG signs of metabolic disturbances in the cardiac muscle are detected, electrolyte-steroid cardiopathy is noted.

Osteoporotic skeletal lesion occurs in more than 80% of patients with Iscenko-Cushing's disease and is one of the more recent and severe manifestations of the disease. If the disease begins in childhood, there is a lag in growth, as cortisol inhibits the development of epiphyseal cartilage. The degree of development of osteoporosis in some cases determines the severity of the condition of patients, and bone fractures and severe pain syndrome are often the cause of excruciating suffering. Similar changes in the bone system in a number of cases occur as a complication of therapy with glucocorticoids of nonendocrine diseases. The thoracic and lumbar spine, ribs, sternum, skull bones are most often affected. In more severe cases, osteoporosis develops in flat and tubular bones. Expressed osteoporotic changes in the spine are accompanied by a decrease in the height of the vertebrae and their compression fractures, which can occur even with little physical exertion or trauma, and often without any apparent cause.

The formation of trophic ulcers, pustular lesions of the skin, the development of chronic pyelonephritis, sepsis occurs because corticosteroids suppress specific immunity. This leads to the development of secondary immunodeficiency. With the disease, the total number of lymphocytes decreases, their interferon activity decreases, the number of T and B cells in the blood and spleen decreases, and lymphoid tissue involution is observed.

In patients, carbohydrate metabolism is disturbed, often manifested by a decrease in glucose tolerance; Glucosuria, hyperglycemia and diabetes mellitus are noted. The diabetic type of the curve in the sample for glucose tolerance is revealed in half of the patients, and the apparent diabetes mellitus - in 10-20% of the total number of patients. Hyperglycemia with Isenko-Cushing's disease develops against a background of elevated levels of cortisol, glucagon, somatostatin and relative insulin deficiency. Glycosylated hemoglobin A as an indicator of the violation of carbohydrate metabolism is increased in the majority of patients with Isenko-Cushing's disease and is one of the earliest tests in the diagnosis of diabetes. Steroid diabetes caused by an excess of glucocorticoids differs from diabetes mellitus by insulin resistance, a very rare manifestation of ketoacidosis and relatively easily regulated by diet and administration of biguanides.

Sexual disorders are one of the early and permanent symptoms of the Itenko-Cushing's disease. They are associated with a decrease in the gonadotropic function of the pituitary gland and an increase in the secretion of testosterone by the adrenal glands. In women, there are violations of the menstrual cycle in the form of opsoniformes and amenorrhea. If the onset of the disease refers to the period of puberty, then menstruation either does not occur, or comes later. Often there is a secondary infertility. At the same time, some patients have an ovulatory menstrual cycle, pregnancy may occur. Violations of sexual function are often accompanied by acne, hirsutism, which is expressed in the growth of hair on the upper lip, chin, chest, back, limbs, along the white line of the abdomen, sometimes develops a virile type of build. The combination of pregnancy and Itenko-Cushing's disease is undesirable both for the health of the mother and for the fetus. Often there are spontaneous abortions in early pregnancy and premature birth.

After the disappearance of signs of hypercorticism, pregnancy and childbirth proceed more safely. In patients with Isenko-Cushing's disease, which have removed the adrenal glands, with adequate replacement therapy, pregnancy and childbirth do not cause major complications. Children, observed for two decades, do not have any deviations. Pregnancy in patients who underwent irradiation of the pituitary gland is recommended not earlier than 2 years later. Quite often after pregnancy, abortions and childbirth, relapse of the disease occurs. Men often suffer from impotence and decreased sexual desire. Hyperpigmentation of skin on the neck, elbows, abdomen occurs in the case of Itenko-Cushing's disease in 10% of cases. Excessive deposition of melanin in the skin is a clinical indicator of increased secretion of adrenocorticotropic and melanotropic hormone.

This disease is often accompanied by emotional shifts and mental disorders. They are very diverse - from mood disturbance to severe psychosis. Sometimes acute psychosis requires special treatment in conditions of psychosomatic hospitals. Treatment of the underlying disease usually leads to the normalization of mental activity.

Increased secretion of ACTH and its fragments, serotonin levels affect the memorization process, participate in the pathogenesis of certain behavioral disorders, reduce cognitive function. Correlations were found between the degree of severity of mental disorders and the blood levels of ACTH and cortisol.

Disease Itenko-Cushing can be mild, moderate and severe. The mild form is characterized by moderate severity of the symptoms of the disease. Some symptoms (osteoporosis, menstrual dysfunction) may be absent. For the disease of moderate severity, all the symptoms are typical, but no complications. In severe form, along with the development of all the symptoms of the disease, various complications are observed: cardiopulmonary insufficiency, hypertonic kidney, pathological bone fractures, the transition of steroid diabetes to a true, progressive muscle weakness associated with muscle atrophy and hypokalemia, severe mental disorders.

The course of the Itenko-Cushing's disease can be progressive and torpid. The progressive course is characterized by a rapid (within a few months) development and a further increase in all the symptoms and their complications. Patients quickly lose their ability to work. With torpid current there is a gradual development of the disease.

[1], [2], [3], [4]