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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 ...
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.
In the rare disease Langerhans cell histiocytosis (LCH), an excess of cells similar to these cells are produced. However LCH cells stain positive to CD14 which is a monocyte marker and shows a different, hematopoietic origin for the disorder. [12] LCH can cause damage to skin, bone and other organs. [citation needed]
First described in 1961 (where they were simply termed "characteristic granules"), [2] they are solely found in Langerhans cells. [1] Although part of normal Langerhans cell histology, they also provide a mechanism to differentiate Langerhans cell histiocytoses (which are a group of rare conditions collectively known as histiocytoses ) from ...
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]
A subset of cells differentiates into Langerhans cells; this maturation occurs in the squamous epithelium, lymph nodes, spleen, and bronchiolar epithelium. Langerhans cells are antigen-presenting cells but have undergone further differentiation. Skin Langerhans cells express CD1a, as do cortical thymocytes (cells of the cortex of the thymus gland).
Hashimoto–Pritzker disease, [1] ... Congenital self-healing reticulohistiocytosis is a condition that is a self-limited form of Langerhans cell histiocytosis. [2]: ...