Abstract. GOMEZ, ANA MARÍA et al. Langerhans cell histiocytosis in children. A description of 10 cases. CES Med. [online]. , vol, n.2, pp La histiocitosis de células de Langerhans (HCL), anteriormente conocida como histiocitosis X, es una enfermedad poco frecuente caracterizada por la. La afectación cutánea en las formas agudas de histiocitosis de células de Langerhans (HCL) es en forma de pápulas eritematosas, aunque se han descrito .

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Hematol Oncol Clin North Am, 12pp. Specialty Hematology Langerhans cell histiocytosis LCH is a rare disease involving clonal proliferation of Langerhans cellscrlulas cells deriving from bone marrow and capable of migrating from skin to lymph nodes.

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Langerhans cell histiocytosis – Wikipedia

Juvenile xanthogranuloma Hemophagocytic lymphohistiocytosis Erdheim-Chester langernans Niemann—Pick disease Sea-blue histiocyte Benign cephalic histiocytosis Generalized eruptive histiocytoma Xanthoma disseminatum Progressive nodular histiocytosis Papular xanthoma Hereditary progressive mucinous histiocytosis Reticulohistiocytosis Multicentric reticulohistiocytosisReticulohistiocytoma Indeterminate cell histiocytosis.

Recommended articles Citing articles 0. Local steroid cream is applied to skin lesions.

Despite successive cycles of chemotherapy, the outcome was the death of the patient after five years, due to his liver disease. It langerans now considered a form of smoking-related interstitial lung disease. Clinical aspects of Langerhans cell histiocytosis. Full text is only aviable in PDF. When found in the lungs, it should be distinguished from Pulmonary Langerhans cell hystiocytosis—a special category of disease most commonly seen in adult smokers.

Guidelines for management of patients up to 18 years with Langerhans cell histiocytosis have been suggested. CS1 German-language sources de Infobox medical condition new All articles with unsourced statements Articles with unsourced statements from April Commons category with local link different than on Wikidata.


Am J Hematol, 47pp. Some affected people recover completely after they stop smoking, but others develop long-term complications such as pulmonary fibrosis and pulmonary hypertension. The frequency and natural history of diabetes insipidus in chindren with Langerhans-cell histiocytosis. Seen mostly in children, multifocal unisystem LCH is characterized by fever, bone lesions and diffuse eruptions, usually on the scalp hstiocitosis in the ear canals. It typically has no extraskeletal involvement, but rarely an identical lesion can be found in the skin, lungs, or stomach.

We use cookies to help provide and enhance our service and tailor content and ads. In the 10th episode of season 3 of House entitled “Merry Little Christmas”the primary patient is a girl with dwarfism who has a variety of symptoms, who is ultimately diagnosed with Langerhans cell histiocytosis.

Histiocytic sarcoma Langerhans cell sarcoma Interdigitating dendritic cell sarcoma Follicular dendritic cell sarcoma. Elsevier About ScienceDirect Remote access Shopping cart Contact and support Terms and conditions Privacy policy We use cookies to help provide and enhance our service and tailor content and ads.

Langerhans cell histiocytosis

Histiocigosis is clinically divided into three groups: Report from the International Registry of the Histiocyte Society”. Print Send to a friend Export reference Mendeley Statistics. The Spanish Association of Pediatrics has as one of its main objectives the dissemination of rigorous and updated scientific information on the different areas of pediatrics. Ten-year experience at Dallas Children’s Medical Center”.

Langerhans cell histiocytosis is occasionally misspelled as “Langerhan” or “Langerhan’s” cell histiocytosis, even in authoritative textbooks. We report five cases of LCH with the same histopathologic basis but different outcome.

Diagnosis is confirmed histologically by tissue biopsy. The Journal of Clinical Endocrinology and Metabolism. Current therapy for Langerhans cell histiocytosis.

The etiology of this disease is unknown. In other projects Wikimedia Commons. The disease spectrum results from clonal accumulation and proliferation of cells resembling the epidermal dendritic cells called Langerhans cellssometimes called dendritic cell histiocytosis. Among children under the age of 10, yearly incidence histiociyosis thought to be 1 in ,; [35] and in adults even rarer, in about 1 inRadiology will show osteolytic bone lesions and damage to the lung. Histiocytosis Monocyte- and macrophage-related cutaneous conditions Rare diseases.


Endocrine deficiency often require lifelong supplement e. Multifocal multisystem LCH, also called Letterer-Siwe diseaseis a rapidly progressing disease in which Langerhans Cell cells proliferate in many tissues. It can be a monostotic involving only one bone or polyostotic involving more than one bone disease. The latter may be evident in chest X-rays with micronodular and interstitial infiltrate in the mid and lower zone of lung, with sparing of the Costophrenic angle or honeycomb appearance in older lesions.

We present the case of a boy with acute disseminated Histiocitowis who, at the age of 16 months, began to experience outbreaks of seborrheic dermatitis-like skin lesions and progressive hepatic dysfunction. Hematoxylin-eosin stain of biopsy slide will show features of Langerhans Cell e. Journal of Clinical Pathology.

Xantomas en paciente con histiocitosis de células de Langerhans y cirrosis biliar – ScienceDirect

MRI and CT may show infiltration in sella turcica. Get Access Get Access.

New England Journal of Medicine. It is characterized by the accumulation and proliferation of histiocytes, eosinophils and Langerhans’ cells with Birbeck granules detected by electron microscopy. By continuing you agree to the use of cookies. We report a year-old man with LCH who presented an extensive cutaneous involvement and lymph node affectation. Peak onset is 2—10 years of age. Assessment of endocrine function and bonemarrow biopsy are also performed when indicated.