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Icenko-Cushing's syndrome.
Last reviewed: 04.07.2025
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A distinction is made between Itsenko-Cushing's disease, which has a hypothalamic-pituitary origin, and Itsenko-Cushing's syndrome itself - a disease associated with primary damage to the adrenal glands. This article only considers the cerebral form of Itsenko-Cushing's disease.
Causes of Itsenko-Cushing syndrome
In addition to ACTH-secreting pituitary tumors (usually basophilic adenomas), the disease very rarely develops as a result of neuroinfectious or post-traumatic damage to the hypothalamic-pituitary region. Decompensation of constitutional dysfunction of the hypothalamic-pituitary-adrenal axis under the influence of chronic stress, hormonal changes, intoxications, and infectious diseases is quite often detected.
Pathogenesis of Itsenko-Cushing syndrome
The clinical picture is determined by an increase in the content of ACTH and cortisol in the blood plasma. Hypertrophy of the adrenal cortex is of a secondary centrally conditioned nature. Hyperproduction of ACTH can be determined by both a predominant pathology of the hypothalamus (excessive production of corticotropin-releasing factor) and a primary lesion of the pituitary gland (ACTH-secreting pituitary adenomas). It is believed that in the genesis of ACTH-secreting pituitary adenomas, a decisive role is played by previous prolonged stimulation by excessively produced corticotropin-releasing factor.
Symptoms of Itsenko-Cushing syndrome
Selective localization of fat on the face (moon face), chest, abdomen, neck, above the 7th cervical vertebra ("climacteric hump") in combination with relatively thin limbs is noted. The face is purple-red, the skin is dry, flaky, there is numerous acne. Wide dystrophic stretch marks are characteristic, usually purple-bluish in color on the skin of the abdomen, thighs, shoulders, and mammary glands. The clinical picture includes arterial hypertension, diabetes mellitus, hirsutism combined with thinning hair on the head, osteoporosis and compression fractures, muscle weakness, capillary fragility, poor wound healing, and decreased resistance to infections.
Psychopathological disorders are of various nature - from hypersomnia to insomnia, from euphoria to depression, sometimes true psychoses occur. Changes in morphological and functional sexual characteristics are observed. In women, in addition to hirsutism, a coarsening of the voice, amenorrhea or oligomenorrhea, decreased sexual desire, masculinization are observed. In men, feminization phenomena, gynecomastia, testicular hypotrophy, decreased sexual desire and spermatogenesis are sometimes observed. Often, the skin of patients in areas experiencing pressure, around the nipples and in places where scars form, darkens.
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What tests are needed?
Differential diagnosis of Itsenko-Cushing syndrome
If signs of hyperadrenocorticism are present, it is necessary to exclude primary pathology of the adrenal glands, i.e. Itsenko-Cushing syndrome. For this purpose, the levels of ACTH and cortisol in the blood are determined and a dexamethasone test is performed. The dexamethasone test is based on the suppression of endogenous ACTH production according to the feedback principle. In Itsenko-Cushing disease, dexamethasone is prescribed orally at 2 mg every 6 hours for 2 days, while the excretion of 17-OCS in the urine decreases by more than 50%; in Cushing syndrome, the excretion of 17-OCS in the urine does not change.
It is necessary to exclude cases with ectopic ACTH production in small cell lung cancer, carcinoid tumors, and pancreatic islet tumors. In ectopic tumors, the course of the syndrome is more malignant, there is no significant increase in body weight, hypokalemia plays a leading role in the clinical picture; as a rule, the dexamethasone test is negative. ACTH- or corticotropin-releasing factor-secreting ectopic tumors are more common in men.
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Nelson's syndrome
In clinical manifestations, it is similar to Itsenko-Cushing disease. The appearance of ACTH-secreting pituitary tumors was detected after bilateral adrenalectomy performed for hyperplasia of the adrenal cortex. The increased production of corticotropin-releasing factor and increased adrenocorticotropic activity in the plasma that appears after adrenalectomy may contribute to the further development of ACTH-secreting pituitary tumors.
Madelung's disease
The main manifestation of the disease is a pronounced localized fat deposition in the neck area. The fat pad encircling the neck gives patients such a specific appearance that differential diagnosis is usually not required. Pathogenesis and etiology are not fully understood. It is referred to as the cerebral form of obesity.
Treatment: Sometimes surgical excision of fatty tissue is used.
Barraquer-Simons disease (progressive lipodystrophy)
Only women are affected, usually after reaching puberty. The disease manifests itself in uneven distribution of fat with the disappearance of the fat layer in the upper half of the body and excessive development of fat deposits in the lower half of the body. Fat deposits are observed mainly on the hips in the form of peculiar "breeches". Weight loss in the upper half of the body and face is very pronounced. Symmetrical forms of the disease are more often observed, but asymmetrical forms with a predominance of uneven distribution of fat on one side of the body can also be encountered. Local atrophic form of lipodystrophy is observed, as a rule, in patients with diabetes at the site of insulin injection. There are reports of bilateral or unilateral facial atrophy with atrophy of not only the subcutaneous fat layer, but also muscles, joining lipodystrophy.
Hereditary factors and mental trauma are of particular importance.
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Treatment of Itsenko-Cushing syndrome
In the presence of an ACTH-secreting pituitary tumor, appropriate surgical or radiation therapy is recommended. Of the pharmacotherapeutic agents, drugs are used that affect either the biosynthesis of cortisol in the adrenal glands (metapirone) or the metabolism of neurotransmitters in the central nervous system (parlodel, cyproheptadine, reserpine). Parlodel is used in a dose of 4 to 7.5 mg / day for 6-8 months; cyproheptadine (peritol, deseril) - in a dose of 25 mg / day. also for a long time; reserpine - in a dose of 2 mg / day. Along with the indicated therapy, traditional methods of weight loss are used.