Histiocytosis X, which is a rare disease, combines 3 related but different in clinical manifestation, course and prognosis of the syndrome: eosinophilic granuloma (Taratynovs disease), Hand Schller Christian disease (syndrome) and Letterer Siw disease (syndrome). The clinical picture of Hend Schller Christian disease was first described by Hand in 1893, then by Schller in 1915 and Christian in 1920. The term histiocytosis X was first proposed in 1953 by Liechtenstein, who combined all 3 of the aforementioned diseases under this name. on the basis of the similarity of the clinical picture, the possibility of a mutual transition from one state to another and typical morphological manifestations consisting in the proliferation of histiocytes and the accumulation of various lipids in their cytoplasm. In 1965, this concept was confirmed in all three diseases, there are no special pentalaminar markers in the cytoplasm of histiocytes that are detected in Langerhans cells (hence the name of these inclusions - "Langerhans cell granules"), the origin and biological significance of which remains unclear. Histiocytes have no signs of atypia, although they infiltrate many organs and tissues retain their normal properties. Histiocytic proliferation can develop in various organs and tissues, causing them to corresponding functional and morphological changes.
Letterer-Siwe disease
