Langerhans cell histiocytosis (LCH) is a rare disorder characterised by increased production of Langerhans-type histiocytes. It more common in the pediatric age group with predilection for osseous involvement, though any organ may be involved. A 10-year male child was brought to neurosurgical clinic slow growing painful tender mass on head. Initial attempt biopsy lesion failed due excessive ble...

Abstract Background. Langerhans cell histiocytosis is a rare reactive proliferative disorder marked by excess proliferation and accumulation of mononuclear phagocytes in tissues organs. Usually, organs systems where cells are normally found involved. Lesions may be limited to one system single- or multisite, many The etiology not fully known. According the hypotheses, immune dysfunction due exu...

We report Langerhans cell histiocytosis involving the sphenoid sinus and extending to the orbital apex. MR and CT imaging, particularly with contrast, both were helpful in defining the extent of the lesion before treatment. MR was a useful examination for follow-up after treatment. Langerhans cell histiocytosis should be considered in the differential diagnosis of lesions of this region in the ...

Progressive mucinous histiocytosis is a very rare, benign, non-Langerhans' cell histiocytosis limited to the skin. In total ten patients (all women) in four families and three sporadic cases have been reported. We report here the first published case of a male patient with progressive mucinous histiocytosis. The multiple red papules on the scalp and forearms were asymptomatic and had slowly inc...

PURPOSE To describe the involvement of the cerebellum by a gliotic and demyelinating process in Langerhans cell histiocytosis. METHODS A retrospective analysis of all (N = 30) cases of Langerhans cell histiocytosis followed at our institution since 1975 yielded four patients with CT and/or MR evidence of cerebellar abnormalities. RESULTS Four patients manifested strikingly similar findings ...

In this issue of Blood, Hervier et al has identified that cooccurrence of Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD) in the same patient is not a rare event. Mixed histiocytosis (MH) highlights existence of a link between distinct groups of histiocytic disorders and suggests presence of a common progenitor cell. Today, histiocytic disorders are classified into 3 group...

Adult pulmonary Langerhans’ cell histiocytosis is a rare disorder of unknown aetiology that occurs predominantly in young smokers, with an incidence peak at 20–40 yrs of age. In adults, pulmonary involvement with Langerhans’ cell histiocytosis usually occurs as a single-system disease and is characterised by focal Langerhans’ cell granulomas infiltrating and destroying distal bronchioles. High-...

Introduction: Erdheim-Chester disease (ECD) is an uncommon non Langerhans cell histiocytosis. Bilateral symmetric sclerosis of the long bones and probable multiorgan involvement are main manifestations this condition. Here, we report a case ECD showing diffuse thickening aorta’s entire wall pericardial effusion.

Smoking-induced diffuse interstitial lung processes include respiratory bronchiolitis, respiratory bronchiolitis-associated interstitial lung disease (RBILD), desquamative interstitial pneumonia (DIP) and Langerhans’ cell histiocytosis. The histological, radiological and clinical features of respiratory bronchiolitis, RBILD and DIP are reviewed, with particular reference to management issues; L...