Nasal oral fistula revealing Langerhans' cell histiocytosis in adult patient: case report
A. MzabiM. ThabetTaghrid TliliHend ZorgatiJ. AnounI. Ben HassineMonia KarmeniF. Ben FredjMoncef MokniChadia Laouani
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Abstract:
Langerhans cell histiocytosis (LCH) is a rare systemic disease caused by proliferation of mature histiocytes; its association to histiocyto fibroma is rarely reported. It rarely affects adults. We report a case of systemic LCH, in an adult patient with osteolytic lesion causing a fistula between the left nasal cavity and hard palate, involving the bone, lung, lymph node and associated to multiple histiocyto fibroma. The patient was operating for a fistula, and he was treated by chemotherapy and corticosteroids. Langerhans´ cell histiocytosis is a rare case, especially in adult patient. The diagnosis was based on histological and immunohistochemical analyses. This patient was treated by steroids and chemotherapy.Keywords:
Langerhans Cell Histiocytosis
Rare disease
Langerhans cell histiocytosis (LCH) in the mandible is a rare disease.LCH was formerly known as histiocytosis X. Histiocytosis X is a granulomatous condition that derives form a clonal proliferation of histiocytes.It has a variable clinical course.LCG may be local or disseminated.It is a reactive disorder or a truly malignant process.This article describes a case of a 45 -year old man with Langerhans cell histiocytosis in his jaw (Fig. 4, Ref. 11).
Langerhans Cell Histiocytosis
Histiocytosis X
Mandible (arthropod mouthpart)
Malignant histiocytosis
Eosinophilic granuloma
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Non-Langerhans cell histiocytosis is a collective term encompassing a vast group of benign proliferative disorders of histiocytes, macrophages and dendritic cells that do not meet the criteria of Langerhans cell histiocytosis. We describe a case of juvenile xanthogranuloma with an unusual clustered distribution.
Juvenile xanthogranuloma
Langerhans Cell Histiocytosis
Histiocytosis X
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Langerhans cell histiocytosis of bone causes bone destruction and frequent bone reactions that mimic benign and malignant neoplasms as well as osteomyelitis. The bony lesion of Langerhans cell histiocytosis consists of histiocytes with variable numbers of eosinophils, lymphocytes, and neutrophils. We present a rare case of Langerhans cell histiocytosis of bone, including imaging studies and pathology. In addition, we discuss the classification and pathology of Langerhans cell histiocytosis.
Langerhans Cell Histiocytosis
Histiocytosis X
Malignant histiocytosis
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Abstract Langerhans‐cell histiocytosis is a proliferative disorder of histiocytic cells that usually affects children during the first years of life. An unusual case of skin limited Langerhans‐cell histiocytosis in a 81‐year‐old man is presented. Mono‐organic involvement seems to be a sign of good prognosis, as is old age in such cases.
Langerhans Cell Histiocytosis
Histiocytosis X
Skin lesion
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Langerhans Cell Histiocytosis
Birbeck granules
Monozygotic twin
Malignant histiocytosis
Infiltration (HVAC)
Histiocytosis X
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Abstract: Langerhans cell histiocytosis is a rare proliferative disorder where pathologic Langerhans cells accumulate in a variety of organs. Historically, the nomenclature regarding this entity has been confusing because the disease had been subcategorized simply based upon the different clinical manifestations. In the following article, we summarize the current recommendation of the Histiocyte Society regarding the classification, evaluation, prognosis, and treatment of Langerhans cell histiocytosis.
Langerhans Cell Histiocytosis
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Abstract Background. The proliferation of non‐Langerhans cell histiocytes is a poorly understood process of unknown cause. Variation in the clinical features and/or histopathology of histiocytic proliferation has led to subclassification of the general category of non‐Langerhans cell histiocytes. Although the current classification may provide some useful generalizations in regard to the anticipated clinical course, wide variability in presentation and outcome make this classification less than optimal when dealing with individual patients. The objectives of the study were to present four cases of generalized non‐Langerhans cell histiocytosis. Materials and Methods. Medical records and slides of four patients diagnosed with non‐Langerhans cell histiocytosis at the Cleveland Clinic are reviewed. Results. The patients exhibit features of more than one subtype of non‐Langerhans cell histiocytosis. Conclusion. The overlap among the clinical and histologic features of the generalized cutaneous non‐Langerhans cell histiocytic disorders suggests that they represent one disease entity with a wide spectrum of presentations rather than many distinct disorders.
Langerhans Cell Histiocytosis
Histopathology
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Langerhans Cell Histiocytosis
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Langerhans cell histiocytosis (LCH) is a rare proliferative disorder in which pathological Langerhans cells (LCs) accumulate in a variety of organs. Historically, the nomenclature regarding LCH has been confusing because the disease had been sub-categorized simply based upon different clinical manifestations. Herein, we report a child with the classic finding of disseminated LCH and summarize the current recommendation of the Histiocyte Society regarding the classification, evaluation, prognosis and treatment of LCH.
Langerhans Cell Histiocytosis
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