Pulmonary langerhans cell granulomatosis (histiocytosis X)

Abstract

Pulmonary Langerhans cell granulomatosis is a rare disease. Its causes are unknown and accurate data on its prevalence are lacking. This disease is characterized by the formation of lung tissue granulomas consisting of activated Langerhans cells, lymphocytes, eosinophils, and macrophages. It is mainly encountered in young 20-40-year-olds, generally in heavy smokers. To establish its valid diagnosis, lung biopsy, followed thorough morphological examination of its obtained specimens, is needed for a valid diagnosis. Langerhans cell granulomatosis in its very advanced stage is very difficult to diagnose even histologically. Lung lesion may be primary and sporadic or concurrent with extrapulmonary lesions in other organs and tissues. The paper describe case of pulmonary Langerhans cell granulomatosis.