During the last few years increasing attention has been focused on Langerhans cells and their peculiar granules (1-5). These organelles were considered specific products of the intraepidermal dendritic cells (1-3), but recently identical structures have been observed in osseous and pulmonary lesions of histiocytosis X (6). The Langerhans cell granule resembles a tennis racket. It is rodlike wit...

Langerhans cell histiocytosis (LCH), formerly known as histiocytosis X, refers to a spectrum of diseases characterized by idiopathic proliferation of histiocytes that produce either focal (localized LCH) or systemic manifestations (Hand-Schüller-Christian disease and Letterer-Siwe disease). Localized LCH accounts for approximately 60-70 % of all LCH cases. Osseous involvement is the most common...

BACKGROUND Congenital self-healing Langerhans cell histiocytosis (Hashimoto-Pritzker disease) is the rarest form of Langerhans cell histiocytosis, usually confined to the skin and/or mucous membranes. Cutaneous eruption is mostly generalized, papular, nodular or vesicular. Despite impressive clinical presentation in a newborn it infrequently spreads to internal organs (which then portends a gra...

Fixation of epidermis with a mixture of osmium tetroxide and zinc iodide (OsO(4)-ZnI(2)) for 24 hr renders the central periodic lamella of the Langerhans cell granule (LCG), the Golgi region, and the nuclear envelope of epidermal Langerhans cells preferentially visible. The use of this technique on Langerhans cells in normal epidermis and in epidermis of patients with histiocytosis (Letterer-Si...

Histiocytosis is a heterogenous group of disorders that are characterized by proliferation and activation of mononuclear phagocyte system. Langerhans Cell Histiocytosis (LCH) or Class I histiocytosis is a rare disorder of unknown etiology with proliferation of Langerhan cells which may infiltrate a single or multiple organs. This disease is more common in infants and children. It is usually spo...

A four-year-old male child presented with increased Typically the lesions are round or oval-shaped with wellurine output and excessive thirst for eight months. On defined margins and bevelled edge. Individual lesions examination he had multiple scalp swellings with mild may coalesce giving a geographic appearance to the skull hepatosplenomegaly. Urine specific gravity was decreased suggestive o...

The records of 70 patients presenting to this hospital since 1961 with histiocytosis X confirmed by biopsy examination have been reviewed. The patients were subdivided into three groups: group A, those under 2 years of age at diagnosis; group B, those between 2 and 5 years; and group C, those over 5 years. All eight patients who died (11% overall mortality) were under 2 years of age at diagnosi...

Over a 29-year period, 43 cases of histiocytosis X presented in children under the age of 12 years. 29 patients (67%) have survived, and of these, 15 (52%) have a detectable disability. It was confirmed that young age at presentation and evidence of soft tissue involvement were associated with a worse prognosis. The majority of deaths were associated with pulmonary involvement. 14 patients deve...