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Langerhans cell histiocytosis (LCH) is an abnormal clonal proliferation of Langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. Symptoms range from isolated bone lesions to multisystem disease . [ 1 ]
Sinus histiocytosis, a common feature in lymph node biopsies, is characterized by dilated sinuses containing variable numbers of histiocytes. [ 4 ] Histiocytosis is a rare disease, thus its diagnosis may be challenging.
Before the Histiocyte Society classified histiocytoses in the 1980s, the condition was also known as "Histiocytosis X", where "X" denoted the then unknown cause. [9] [10] [11] It is now known as chronic multifocal Langerhans cell histiocytosis, a subtype of LCH. [3] The disease is rare. [12] Most present between the ages of two and six. [2]
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
Non-Langerhans cell histiocytosis, also known as rare histiocytoses, comprise all histiocyte, macrophage, and dendritic cell proliferative disorders that are not categorized as hemophagocytic lymphohistiocytosis (HLH) or Langerhans cell histiocytosis (LCH). [1] The spectrum of non-langerhans cell histiocytoses include: Benign cephalic histiocytosis
Juvenile xanthogranuloma is a form of histiocytosis, classified as non-Langerhans cell histiocytosis. [1] [2] It is a rare skin disorder that primarily affects children under one year of age but can also be found in older children and adults. [3] It was first described in 1905 by Adamson. [4]
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.
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).