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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 ]
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.
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]
Lesion on a nose. Human eosinophilic granuloma is characterized by abnormal proliferation of Langerhans cells (LCs). LCs are antigen-presenting cells derived from dendritic cells. In humans, eosinophilic granulomas are considered as a benign tumors that occurs mainly in children and adolescents. EG is a quite rare condition, and its incidence ...
A dermatofibroma, or benign fibrous histiocytomas, is a benign nodule in the skin, typically on the legs, elbows or chest of an adult. [3] It is usually painless. [3] It usually ranges from 0.2cm to 2cm in size but larger examples have been reported. [3] It typically results from mild trauma such as an insect bite. [3]
Langerhans cell is represented by a yellow oval; blue arrows correspond to is_a relations, and orange arrows correspond to develops_from relations. Only a subset of Langerhans cell parent types are included in the figure. [1] A Langerhans cell (LC) is a tissue-resident macrophage of the skin [2] once thought to be a resident dendritic cell. [3]
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]
Langerhans cell sarcoma (LCS) is a rare form of malignant histiocytosis. It should not be confused with Langerhans cell histiocytosis, which is cytologically benign. [1] It can present most commonly in the skin and lymphatic tissue, but may also present in the lung, liver, and bone marrow. [2] [3] Treatment is most commonly with surgery or ...