Symptoms of Icenko-Cushing's disease

The skin of patients is thin, dry, and purple-cyanotic on the face, chest, and back. The venous pattern on the chest and limbs is clearly visible. Acrocyanosis is observed. Wide red-violet striae appear on the skin of the abdomen, inner shoulders, thighs, and in the area of the mammary glands. Hyperpigmentation of the skin is often observed, most often in areas of friction. Pustular rashes and hypertrichosis appear on the back, chest, and face. Hair on the head often falls out, and baldness in women is noted according to the male pattern. There is an increased tendency to furunculosis and the development of erysipelas.

There is excessive fat deposition in the neck, trunk, abdomen and face, which takes on the appearance of 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, losing their normal shape.

Obesity of varying degrees in Itsenko-Cushing's disease is observed in more than 92% of cases. In patients without obesity, there is a pronounced redistribution of subcutaneous fat with a predominant location in the chest and abdomen.

Arterial hypertension is one of the early and constant symptoms of Itsenko-Cushing's disease. Both systolic and diastolic pressure increase. Often, an increase in blood pressure over a long period of time is the only symptom of the disease. Changes in the cardiovascular system are often the leading ones in the clinical picture of the underlying disease and are the cause of disability and most deaths.

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

Most patients show various ECG signs of metabolic disorders in the heart muscle, and electrolyte-steroid cardiopathy is noted.

Osteoporotic skeletal damage occurs in more than 80% of patients with Itsenko-Cushing's disease and is one of the later and more severe manifestations of the disease. If the disease begins in childhood, growth retardation is observed, since cortisol inhibits the development of epiphyseal cartilage. The degree of osteoporosis development in some cases determines the severity of the patient's condition, and bone fractures and severe pain syndrome are often the cause of excruciating suffering. Similar changes in the skeletal system in some cases also occur as a complication of glucocorticoid therapy for non-endocrine diseases. Most often, the thoracic and lumbar spine, ribs, sternum, and skull bones are affected. In more severe cases, osteoporosis develops in flat and tubular bones. Pronounced 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 minor physical exertion or injury and often for no apparent reason.

The formation of trophic ulcers, pustular skin lesions, 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 involution of lymphoid tissue is observed.

Patients have impaired carbohydrate metabolism, often manifested by decreased glucose tolerance; glucosuria, hyperglycemia, and diabetes mellitus are noted. The diabetic type of curve in the glucose tolerance test is detected in half of the patients, and overt diabetes mellitus - in 10-20% of the total number of patients. Hyperglycemia in Itsenko-Cushing's disease develops against the background of increased levels of cortisol, glucagon, somatostatin, and relative insulin deficiency. Glycosylated hemoglobin A as an indicator of impaired carbohydrate metabolism is elevated in most patients with Itsenko-Cushing's disease and is one of the earliest tests in the diagnosis of diabetes. Steroid diabetes caused by excess glucocorticoids differs from diabetes mellitus in insulin resistance, a very rare manifestation of ketoacidosis, and is relatively easily regulated by diet and the administration of biguanides.

Sexual dysfunction is one of the early and constant symptoms of Itsenko-Cushing's disease. It is associated with a decrease in the gonadotropic function of the pituitary gland and an increase in testosterone secretion by the adrenal glands. Women experience menstrual irregularities in the form of opsomenorrhea and amenorrhea. If the onset of the disease occurs during puberty, menstruation either does not occur or occurs later. Secondary infertility often occurs. At the same time, some patients have an ovulatory menstrual cycle, pregnancy may occur. Sexual dysfunction is often accompanied by acne, hirsutism, expressed in hair growth on the upper lip, chin, chest, back, limbs, along the white line of the abdomen, and sometimes a virile body type develops. The combination of pregnancy and Itsenko-Cushing's disease is undesirable for both the health of the mother and the fetus. Spontaneous early terminations of pregnancy and premature birth often occur.

After the signs of hypercorticism disappear, pregnancy and childbirth proceed more successfully. In patients with Itsenko-Cushing's disease, whose adrenal glands have been removed, pregnancy and childbirth do not cause major complications with adequate replacement therapy. Children observed for two decades do not have any abnormalities. Pregnancy in patients who have undergone pituitary irradiation is recommended no earlier than 2 years later. Relapses of the disease occur quite often after pregnancy, abortions and childbirth. Impotence and decreased sexual desire are often observed in men. Hyperpigmentation of the skin on the neck, elbows, and abdomen occurs in Itsenko-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 hormones.

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

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

Cushing's disease can be mild, moderate, or severe. The mild form is characterized by moderate severity of the disease symptoms. Some symptoms (osteoporosis, menstrual dysfunction) may be absent. Moderate severity of the disease is characterized by the severity of all symptoms, but the absence of complications. In the severe form, along with the development of all the symptoms of the disease, various complications are observed: cardiopulmonary insufficiency, hypertensive kidney, pathological bone fractures, the transition of steroid diabetes to true diabetes, progressive muscle weakness associated with muscle atrophy and hypokalemia, severe mental disorders.

The course of Itsenko-Cushing's disease can be progressive and torpid. The progressive course is characterized by rapid (within several months) development and further increase of all symptoms and their complications. Patients quickly lose their ability to work. In the torpid course, the disease develops gradually.

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