The disease refers to disorders of lipid metabolism. It is assumed that reticulosis develops primarily, and secondarily, due to a violation of cell permeability, lipids are excessively deposited. Violation of cellular metabolism plays an important role.
It comes to light on the 3rd year of life of the child. Such dwarfs have a puppet appearance, normal intelligence. Most often, changes in the periodontium are found in the group of pyridium with dwarfism with delayed sexual development and with hypothyroidism. In children suffering from dwarfism at the age of 3 to 6 years, periodontopathic syndrome was not detected. It is generally accepted that the main lesion is located in the anterior pituitary gland and secondarily involved in the process of the adrenal cortex, sex glands and other parts of the endocrine system. In the epididymis of the brain with Itsenko-Cushing's disease, an intrasaddle tumor develops - a small adenoma of basophilic cellular elements that secrete a gonadotropic secret.
Gradually, malaise develops, the child quickly gets tired, becomes irritable, his appetite decreases, up to anorexia, abdominal pain, nausea, and constipation appear. The most typical is the triad of signs: 1) multipledestructive changes in the bones of the skull, spine, pelvis, ribs, limbs; 2) exophthalmos; 3) diabetes insipidus. Patients can drink and excrete up to 10-12 liters of fluid per day. They vomit, lose body weight, up to complete exhaustion, growth slows down, and there is a lag in physical and mental development. Skin changes are noted in 1/3 of patients: dryness, follicular bright red, and then yellow, slightly scaly papules, petechial elements, hemorrhagic crusts, punctate scars. The picture is very similar to rashes in Abt-Letterer-Siwe disease. They have the same, but more limited localization, damage to the nail plates is also possible. Diagnosis of Hand-Schuller-Christian disease.