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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Juvenile xanthogranuloma (JXG) is a disorder of histiocytes usually associated with cutaneous lesions. It may present a diagnostic dilemma in the absence of cutaneous lesions and when deeply located. Differentiation of JXG from other childhood histiocytosis syndromes, especially Langerhans' cell histiocytosis (LCH), is important. We describe an unusual case of deep JXG in a 27-month-old girl with multiple omental and peritoneal nodules presenting with ascites. Although a diagnosis of LCH was suspected clinically, the absence of Birbeck granules and S-100 protein and T6 antigen negativity, together with CD68 and factor XIIIa positivity, led us to a diagnosis of JXG. Physicians should be aware of the widening spectrum of manifestations of juvenile xanthogranuloma.
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PMID:Deep juvenile xanthogranuloma: an unusual presentation. 918 28

In the diagnosis of the Langerhans cell histiocytosis several monocyte and macrophag markers have been tested in the recent years. We compared the expression of macrophage and lymphoid markers in childhood and adult type Langerhans cell histiocytosis. Ten childhood and 11 adult cases were tested using paraffin sections of biopsy samples. We have examined 6 markers: the S-100, Lysozyme, CD68 macrophag and the CD1a, CD4, HLA-DR lymphoid markers. We have found that the CD68 marker was more frequently positive than the other examined macrophag markers, and proved to be almost as reliable as the recently discovered CD1a. The most interesting result was that the expression of the markers was different in the childhood and adult type of the disease. On the basis of our experience the possibility arise that the phenotype of the childhood and adult type of the Langerhans cell histiocytosis is different.
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PMID:[Expression of macrophage markers in childhood and adult Langerhans histiocytosis (LCH)]. 925 59

We present a 66-year-old man who had maculopapular pigmented lesions on the skin of the head, neck and trunk suggesting generalized eruptive histiocytoma (GEH). These lesions had a yellowish centre in a target-like pattern that has not been previously described. The patient suffered from diplopia and had a severe sensorimotor polyneuropathy causing progressive paresis of the limbs. The explorations performed disclosed the presence of specific xanthomatous infiltrates in the skin, lungs, respiratory tract, peripheral nerves and meninges, suggesting xanthoma disseminatum (XD) or juvenile xanthogranuloma. Multiple osteolytic lesions of large bones were also found. The infiltrate was CD68, MAC 387 and factor XIIIa positive and S-100 and CD1 negative. Some cells contained worm-like bodies visible by electron microscopy. Our patient presented clinical and immunohistochemical findings suggestive of GEH, juvenile xanthogranuloma or XD, supporting the idea of a wide spectrum of non-Langerhans cell histiocytosis. These specific target-like xanthomatous lesions seem to be unique for this new variant of XD.
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PMID:Systemic xanthohistiocytoma: a variant of xanthoma disseminatum? 958 Jan 48

Langerhans cell histiocytosis (LCH) is a disease with a broad spectrum of clinical presentations. All of the variants have in common the proliferation of cells which are morphologically, biochemically, and immunophenotypically indistinguishable from Langerhans cells. A retrospective study of three elderly patients revealed the unique presentation of cutaneous Langerhans cell histiocytosis limited to the genitalia. These cases produced a diagnostic challenge because of their unusual clinical presentation and their morphological similarity to certain other entities, including extramammary Paget's disease and malignant melanoma, which may also show S-100-positive atypical cells. All three cases showed infiltrates of histiocytic-appearing cells with folded nuclei and moderate amounts of cytoplasm which involved the epidermis, dermis, or both. Immunoperoxidase studies using antibody to S-100, CD1a and CD68 in each case showed positive staining.
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PMID:Cutaneous Langerhans cell histiocytosis of the genitalia in the elderly: a report of three cases. 976 22

Erdheim-Chester disease is a clinicopathologic entity defined by a characteristic pattern of symmetric osteosclerosis caused by an infiltrate of mononuclear cells that include prominent numbers of foamy histiocytes. About half of patients have extraskeletal manifestations, including involvement of the hypothalamus/posterior pituitary, orbit, retroperitoneum, skin, lung, and heart. Pulmonary involvement is an uncommon but important manifestation of Erdheim-Chester disease because it causes significant morbidity and mortality. A review of the Mayo Clinic files produced four patients with confirmed Erdheim-Chester disease in whom lung biopsy had been performed. One additional patient was included from the University of Pittsburgh. Four patients were women. The mean age was 53.6 years (range 25-70 years). All patients had bilateral and symmetric sclerotic bone lesions characteristic of Erdheim-Chester disease, although in three the skeletal abnormalities were discovered only after lung biopsy. Four patients had dyspnea, and one also had a dry cough. One patient died 17 months after diagnosis. Chest radiographs showed diffuse interstitial infiltrates in all patients, with an upper zone predominance in three. Thoracic computed tomography (CT) scans showed thickening of the visceral pleura and interlobular septa with patchy associated fine reticular and centrilobular opacities and ground glass attenuation. Lung biopsy specimens showed an infiltrate of foamy histiocytes, lymphocytes, and scattered Touton giant cells with associated fibrosis in a striking lymphatic distribution. The infiltrate involved visceral pleura, interlobular septa, and bronchovascular bundles. Immunohistochemical stains were positive for CD68 in all cases and S-100 protein in four cases. Stains for CD1a were consistently negative. Ultrastructural studies in one case showed no Birbeck granules. Although in bone the histologic features of Erdheim-Chester disease may overlap with Langerhans' cell histiocytosis, its expression in the lung is distinct. Lung involvement in Erdheim-Chester disease has emerged as a unique radiographic and histologic entity.
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PMID:Erdheim-Chester disease: clinical, radiologic, and histopathologic findings in five patients with interstitial lung disease. 988

Langerhans cell histiocytosis is classically considered as totally different from Chester-Erdheim's disease which consists in the infiltration of various parenchymas by macrophagic CD68-positive histiocytes. We report the case of a 46-year-old woman with a long history of diabetes insipidus who presented typical lesions of Langerhans cell histiocytosis on vulvar and skin biopsies as well as bony cellular infiltrates characteristic of Chester-Erdheim's disease. A few months later she presented cerebellar disorders and died after an 18-month course. At autopsy the pons was enlarged, due to numerous cellular infiltrates which were also scattered in the middle cerebellar pedoncles, dentate nuclei, midbrain and hypothalamus. There were S100-protein positive Langerhans cells intermingled with numerous ovoid CD68-positive histiocytes. There are a few reported cases of Chester-Erdheim's disease presenting foci of Langerhans cells histiocytosis in other parenchymas. In addition, there are 10 reported cases with diabetes insipidus and bilateral infiltration of the brain stem and cerebellum, considered as presenting either one type of histiocytosis or the other. Our case demonstrates that both histiocytoses may coexist in the brain and thus correspond in fact to the same pathology in certain particular cases.
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PMID:Brain stem infiltration by mixed Langerhans cell histiocytosis and Chester-Erdheim disease: more than just an isolated case? 1039 77

Previous reports of patients with Langerhans' cell histiocytosis have shown characteristics of osteolytic lesion, visceral involvement and organ dysfunction. We report a 2-year-old boy who was diagnosed as Langerhans' cell histiocytosis with a prominent hepatomegaly. X-Radiogram, computed tomography and magnetic resonance imaging revealed the osteolysis of the right iliac bone, the absence of the left eighth rib as well as the right mandible, and an enhancing mass in the left cerebellum. The data of radiography were highly suggestive of Langerhans' cell lineage. The presence of large CD1a-positive mononuclear cells associated with inflammatory cells in peripheral blood smear and bone marrow aspirate further confirmed the diagnosis. In addition, expressions of S100, CD25, CD68, CD80, CD86, and Fas ligand were identified on these cells by immunocytochemical staining. The results indicate that although these cells are activated Langerhans' cells, progression of the disease and the bone destruction could be mediated by the overt FasL expression of the cells.
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PMID:Expression of Fas ligand in Langerhans' cell histiocytosis: A case report of a boy with multisystem involvement. 1044 Sep 12

A 26-year-old man presented with diabetes insipidus, sexual dysfunction and memory impairment. Nuclear magnetic resonance imaging and pathologic examination revealed Langerhans cell histiocytosis of the hypothalamus, which contained no eosinophils. The lesional cells were positive for S-100 protein and CD1a antigen, and negative for CD68 antigen. The possible pathogenesis, absence of eosinophils and prognosis are discussed.
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PMID:Hypothalamic Langerhans cell histiocytosis with no eosinophils. 1050 35

Hereditary progressive mucinous histiocytosis is a rare autosomal dominant non-Langerhans cell histiocytosis. We describe a sporadic case of this syndrome in a 64-year-old woman who had multiple dark-red dome-shaped papulonodules located mainly on the back of her hands, forearms and thighs. Light microscopy revealed a circumscribed upper dermal aggregate of ovoid or spindle-shaped histiocytes with abundant mucin deposition. Iron deposits and numerous mast cells were scattered throughout the tumour but giant cells were rare. Electron microscopy revealed a high number of zebra bodies and myeloid bodies in the cytoplasm of the histiocytes. Immunohistochemistry showed positive labelling with alpha-1 antitrypsin, Factor XIIIa and CD68, while CD1a, CD34 and S100 protein were negative. The differential diagnosis of histiocytic syndromes is discussed.
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PMID:A sporadic case of progressive mucinous histiocytosis. 1065 9

Benign cephalic histiocytosis (BCH) is best understood as a form of non-Langerhans cell histiocytosis, specifically as an early mononuclear variant of juvenile xanthogranuloma (JXG). However, the progression of BCH into JXG in the same patient has only been reported once before. We describe the case of a 2-year-old girl with asymptomatic, large, ill-defined infiltrated flat plaques over both cheeks, in addition to isolated papules. A punch biopsy of a plaque revealed dermal infiltration by vacuolated and scalloped histiocytes positive for CD68 KP-1, and that lacked expression of CD1a and S-100 protein, favoring macrophages over Langerhans cells. Electron microscopy study showed comma-shaped intracytoplasmic bodies in the histiocytic cells leading to the diagnosis of BCH. One year later, after an episode of varicella-zoster infection, the flat plaques over the cheeks became large reddish-yellow nodules, and in a second biopsy appeared to progress to JXG. Virus-related mechanisms of progression are discussed.
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PMID:Benign cephalic histiocytosis progressing into juvenile xanthogranuloma: a non-Langerhans cell histiocytosis transforming under the influence of a virus? 1069 21


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