Abstract
Langergans cell histiocytosis (LCH) is a rare orphan pathology. Even rarer the clinicians see histiocytic lesions of the lungs and skin, the share of cases varies from 20–40% in case of multisystem LCH. Two clinical cases of LCH with lesions of the lungs, skin, pituitary gland and bones which differ in the manifestation and progression speed of the disease, are presented. The most characteristic clinical, radiological and functional features of pulmonary LCH and morphological changes are reviewed in detail. The clinical manifestations of skin histiocytosis are nonspecific, making it difficult to diagnose LCH. Currently, the frequency of this pathology is on the grow, which increases the responsibility for its timely diagnosis, based on clear clinical and morphological comparisons. For proper diagnosis and timely treatment, a biopsy of the pathological focus is necessary, followed by histological and immunohistochemical examination with antibodies to protein S100, CD1a, CD68, CD207 (langerin).