During the past 40 years, three previously unrecognized disorders of the reticuloendothelial system have been described : Letterer-Siwe’s disease,’ Hand-Sch#{252}ller-Christian’s disease,24 and eosinophilic granuloma.5’6 Due largely to the influence of Lichtenstein,7 the opinion now prevails that these three disorders are fundamentally related. They differ from one another mainly in terms of pa...

BACKGROUND Langerhans cell histiocytosis is a rare systemic disease characterized by the abnormal overproduction of histiocytes that tend to infiltrate single or multiple organ systems leading to significant tissue damage. It mainly affects - by order of decreasing frequency - the bone, the skin, the lymph nodes, the liver, and lungs. Gastrointestinal tract involvement is extremely rare in adul...

INTRODUCTION Congenital Langerhans cell histiocytosis is usually limited to cutaneous lesions and has a good prognosis. In rare cases of gut involvement, mortality is high and early and aggressive treatment essential. MATERIALS AND METHODS We report a case of histiocytosis in a newborn with bowel involvement, and performed a literature review of 13 similar cases worldwide documented between 1...

Objective. To present a case of otic Langerhans' cell histiocytosis in an adult. Also included the diagnosis and management of the condition and a review of the relevant literature. Case Report. We report a case of a 41-year-old man with a history of persistent unilateral ear discharge associated with an aural polyp. Radiological imaging showed bony lesions of the skull and a soft-tissue mass w...

1. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy: a newly recognized benign clinicopathological entity. Arch Pathol. 1969;87 (1):63-70. 2. Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol. 1990;7(1):19-73. 3. Eisen RN, Buckley PJ, Rosai J. Immunophenotypic characterization of ...

Langerhans’cell histiocytosis (Histiocytosis X) is a rare disease of unknown cause characterized by oligoclonal proliferation of Langerhans cells. It occurs mostly in children and young adults and involves one or more body systems such as bone, hypothalamus, posterior pituitary gland, lymph nodes, liver or various soft tissues.The diagnosis is always made by a histological approach.We report a ...

Histiocytosis is a term applied to a group of rare disorders of the reticuloendothelial system. Eosinophilic granuloma, the most benign and localized of the three Langerhans cell histiocytosis entities, may be solitary or multiple. Eosinophilic granuloma can affect almost any bone, but commonly involves the mandible when the jaws are affected. Conventional treatment of LCH is with surgery, radi...

Langerhans' cell histiocytosis summarizes a spectrum of diseases on the basis of histogenetic criteria. These are characterized by an accumulation of cells with Langerhans' cell phenotype in one or multiple organs. Up to 50% of patients with either single or multi-organ manifestation of Langerhans' cell histiocytosis initially present with cutaneous symptoms. Nevertheless, cutaneous Langerhans'...

L angerhans’ cell histiocytosis (LCH), formerly known as histiocytosis X, is one of a group of poorly understood diseases of histiocytes. The clinical spectrum of disease ranges from the chronic, localized form to an acute leukemia-like disease with a fatal outcome. Alfred Hand was the first to report a case of histiocytosis in 1893.1 Later, in 1941, Farber described this condition when reporti...

familial defective apolipoprotein (apo) b 100 (fdb) causes early-onset coronary heart diseases (chd). it is produced by r3500q mutation of the apob gene resulting in decreased binding of ldl to ldl receptor. we screened the apo b gene for r3500q mutation in 130 hypercholesterolemic patients, among whom 30 patients met criteria of familial hypercholesterolemia (fh). the prevalence of r3500q alle...