INVOLVEMENT OF THE CENTRAL NERVOUS SYSTEM IN HAND‐SCHÜLLER‐CHRISTIAN DISEASE REPORT OF A CASE AND DISCUSSION ON THE ENTITY OF THE DISEASE–

Abstract

A case of Hand‐Schüller‐Christian disease with involvement of the central nervous system is reported. In addition to the visceral and bone lesions, the central nervous system were involved by granulomatous lesions with prominent astrocytic reaction and only few deposits of doubly retractile material in the form of foam cells. Literatures of the reported cases of Hand‐Schüller‐Chrlstian disease with Involvement of central nervous system are reviewed. Hand‐Schüller‐Christian disease is characterized by granulomatous lesion of the peripheral loose connective tissue of the organs where the lesions originate from the adventitial tissue. The major reticuloendothelial system organs such as the liver, spleen and lymph nodes are essentially spared. Deposition of cholesterol ester may be a secondary phenomenon. Hand‐Schüller‐Christian disease should be appreciated as a separate disease entity because of its particular pathomorphology.