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The disease spectrum results from clonal accumulation and proliferation of cells resembling the epidermal dendritic cells called Langerhans cells, sometimes called dendritic cell histiocytosis. These cells in combination with lymphocytes , eosinophils , and normal histiocytes form typical LCH lesions that can be found in almost any organ . [ 5 ]
Chronic multifocal Langerhans cell histiocytosis; Other names: Hand–Schüller–Christian disease: A child with Hand-Schüller-Christian Disease: Specialty: Dermatology: Symptoms: Triad of bulging eyes, breakdown of bone, diabetes insipidus [1] Other symptoms eg. bone pain, facial asymmetry, ear infections, teeth/gum problems, liver and lung ...
According to the Histiocytosis Association, 1 in 200,000 children in the United States are born with histiocytosis each year. [2] HAA also states that most of the people diagnosed with histiocytosis are children under the age of 10, although the disease can afflict adults. The disease usually occurs from birth to age 15. [3]
Letterer–Siwe disease, (LSD) or Abt-Letterer-Siwe disease, is one of the four recognized clinical syndromes of Langerhans cell histiocytosis (LCH) and is the most severe form, involving multiple organ systems such as the skin, bone marrow, spleen, liver, and lung. Oral cavity and gastrointestinal involvement may also be seen.
Hashimoto–Pritzker disease, [1] ... Congenital self-healing reticulohistiocytosis is a condition that is a self-limited form of Langerhans cell histiocytosis. [2]: ...
Histiocytic disorders refer to diseases that are caused by abnormal behavior of these cells. They include the following: Reactive diseases of Langerhans cells (most important feature in immune histochemistry is expression of E-cadherin) Histiocytomas; Reactive histiocytosis (immunohistochemical features show that interstitial/dermal DCs are ...
X-type histiocytoses are a clinically well-defined group of cutaneous syndromes characterized by infiltrates of Langerhans cells, as opposed to Non-X histiocytosis in which the infiltrates contain monocytes/macrophages. [1]: 720 Conditions included in this group are: [1]: 720–4 Congenital self-healing reticulohistiocytosis
Histiocyte Society logo. The Histiocyte Society is an international network of people that co-ordinate studies of the histiocytoses, which it has divided into Langerhans cell histiocytosis (class I) (previously known as Hand–Schüller–Christian disease and histocytosis-X), non-Langerhans cell histiocytoses (class II), and malignant histiocytosis (class III).