• More recently, histiocytic diseases have been reclassified into five groups: (1) Langerhans-related, (2) cutaneous and mucocutaneous, (3) malignant histiocytosis, (4) Rosai-Dorfman disease, and (5) hemophagocytic lymphohistiocytosis and macrophage activation syndrome. (medscape.com)
  • Cutaneous histiocytoses are classified according to the type of histiocyte within the skin. (dermnetnz.org)
  • Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the presence of cells with characteristics similar to bone marrow-derived Langerhans cells juxtaposed against a backdrop of hematopoietic cells, including T-cells, macrophages, and eosinophils. (medscape.com)
  • The term Langerhans cell histiocytosis is generally preferred to the older term, histiocytosis X. This newer name emphasizes the histogenesis of the condition by specifying the type of lesional cell and removes the connotation of the unknown ("X") because its cellular basis has now been clarified. (medscape.com)
  • Non-Langerhans cell histiocytosis refers to a family of histiocytosis characterized by the absence of Langerhans cells. (wikipedia.org)
  • The spectrum of non-langerhans cell histiocytoses include: Benign cephalic histiocytosis Generalized eruptive histiocytoma Indeterminate cell histiocytosis Juvenile xanthogranuloma Progressive nodular histiocytoma Necrobiotic xanthogranuloma (Giant Cell) Reticulohistiocytoma Multicentric reticulohistiocytosis Rosai-Dorfman disease Xanthoma disseminatum Kikuchi disease Erdheim-Chester disease. (wikipedia.org)
  • Broadly speaking, they can be divided into Langerhans and non-Langerhans cell histiocytoses. (thedoctorsdoctor.com)
  • Langerhans cell histiocytoses are all defined by the presence of a unique ultrastructural organelle, the Birbeck granule. (thedoctorsdoctor.com)
  • Currently, the preferred term is Langerhans cell histiocytosis. (thedoctorsdoctor.com)
  • Langerhans cell histiocytosis presenting in the neonatal period: a retrospective case series. (thedoctorsdoctor.com)
  • OBJECTIVES: To describe the morphologic characteristics of skin lesions, extent of extracutaneous disease, and outcomes in patients with neonatal presentation of Langerhans cell histiocytosis (LCH), and to examine clinical predictors of disease prognosis. (thedoctorsdoctor.com)
  • Epidemiologic study of Langerhans cell histiocytosis in children. (thedoctorsdoctor.com)
  • Class IIa dermal dendritic cell histiocytosis is a reactive increase in the number of non-Langerhans' cell histiocytes. (dermnetnz.org)
  • The working group of the Histiocyte Society divided histocytic disorders into three groups: (1) dendritic cell histiocytosis, (2) macrophage-related disorders, and (3) malignant histiocytosis. (medscape.com)
  • A congenital, self-healing form called Hashimoto-Pritzker disease has also been described. (medscape.com)
  • The intermediate clinical form called Hand-Schüller-Christian disease is characterized by multifocal, chronic involvement and classically presents as the triad of diabetes insipidus, proptosis, and lytic bone lesions. (medscape.com)
  • A histiocytosis is a disease in which there are too many histiocyte cells in the skin and other organs. (dermnetnz.org)
  • Many patients suffering from histiocytoses eventually develop neurodegenerative symptoms or behavioral deficits. (uni-bonn.de)
  • Normal histiocyte cells are originally formed within the bone marrow and then migrate throughout the body via the bloodstream. (dermnetnz.org)
  • Most forms of histiocytosis are rare. (dermnetnz.org)
  • Her attempt to manipulate microglia, as the brain-specific macrophages are called, were stimulated by the findings in patients suffering from a rare form of cancer called histiocytosis. (uni-bonn.de)
  • STUDY DESIGN: We used a case-control study design to obtain data from parents of children with LCH (n = 459) who were members of the Histiocytosis Association of America and Canada. (thedoctorsdoctor.com)
  • The working group of the Histiocyte Society divided histocytic disorders into three groups: (1) dendritic cell histiocytosis, (2) macrophage-related disorders, and (3) malignant histiocytosis. (medscape.com)
  • Since we last met in Stockholm in 2004, our field has advanced tremendously with new insights into disease pathogenesis and new, effective targeted therapies for all forms of histiocytoses. (wildapricot.org)
  • The intermediate clinical form called Hand-Schüller-Christian disease is characterized by multifocal, chronic involvement and classically presents as the triad of diabetes insipidus, proptosis, and lytic bone lesions. (medscape.com)
  • It is part of a group of clinical syndromes called histiocytoses, which are characterized by an abnormal proliferation of histiocytes (an archaic term for activated dendritic cells and macrophages). (springer.com)
  • This year's program continues to offer the cutting edge of clinical and scientific advances related to the histiocytoses. (wildapricot.org)
  • These diseases are related to other forms of abnormal proliferation of white blood cells, such as leukemias and lymphomas. (springer.com)
  • However, in contrast to pediatric histiocytoses, which involve multiple organs, PLCH usually manifests in a single organ-the lung. (medscape.com)