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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 ]
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.
Congenital self-healing reticulohistiocytosis is a condition that is a self-limited form of Langerhans cell histiocytosis. [2]: ...
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 is higher in white ...
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]
non-Langerhans-cell histiocytosis Hemophagocytic lymphohistiocytosis (HLH) II: D76.1: non-Langerhans-cell histiocytosis Niemann–Pick disease: II: E75.2: non-Langerhans-cell histiocytosis Sea-blue histiocytosis: II – non-Langerhans-cell histiocytosis Acute monocytic leukemia: III: C93.0: malignant histiocytic disorders Malignant ...
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
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]