Histiocytosis-x disease: histopathology and histochemical study

ABSTRACT

Eight cases of histocytosis X disease [H and-Schuller-Christian disease]; 2 cases-Eosinophilic granuloma; 2 cases-Letterer-Siwe disease; 4 cases] with oral lesions were studied histologically and histochemically. Histochemical evaluation of mucopolysaccharides was undertaken by application of PAS stain in an effort to establish, if any, histochemical criteria for microscopic differentation .The main proliferating cells were different in every tumor type; many eosinophils in eosinophilic granuloma, numerous histiocytes with foam cells in Hand-Schuller-Christian disease, large number of undifferentiated reticuloendothelial cells in Letterer-Siwe disease. The histochemical investigations revealed that strong PAS positive material could be observed in eosinophilic granuloma, while the other two forms showed faint reaction. Large number of cases are required to confirm such differences. Interestingly, dental tissue changes were observed in Letterer-Siwe disease; it comprised an increase in the width of secondary dentin and the interglobular dentin, hydropic degeneration of odontoblasts of the dental pulp. Since oral lesions might be the first manifestation of the disease, histologic differentiation betwewn these forms is considered to be an important factor in the diagnosis because of the extreme variability of prognosis and management