Hand-Schiller-Christian Disease

Hand-Schiller Christian disease (HSCB) is a rare disease associated with excessive granulocyte activity and damage to the vascular endothelium, which leads to hemodynamic and thrombotic microcirculatory disorders. This syndrome describes a hereditary inflammatory process, which is characterized by hemorrhagic diathesis, the development of giant cells with lipid contents and vasculitis, and progressive renal failure. The prevalence of the syndrome in the world is from 1:40,000

Hand-Schüller-Christian disease is a rare, hereditary connective tissue disease of unknown etiology, manifested by slowly progressive hyperemia of the skin with bluish-purple foci of varying sizes. In most cases, small nodules simultaneously appear under the mucous membranes of the oral cavity, nose and pharynx, gums, tongue and larynx, which also represent a tumor of unclear consistency and color - the so-called. “crumbs” covered with crusts. The speech of patients is difficult due to narrowing of the larynx and hypoglossia. Death occurs as a result of respiratory failure due to swelling and obliteration of the larynx or from compression of the subcortical formations of the brain.