Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The distribution of S100 protein in normal skin and various tumors involving skin was assessed using rabbit antibody to S100 protein in an immunoperoxidase reaction. In normal skin, S100 protein was detected in the epidermis (melanocytes and Langerhans' cells), dermis (Schwann cells, Pacinian and Meissner's corpuscles, and interdigitating reticulum cells), cells of the sweat gland apparatus, and in chondrocytes. In tumors involving skin, S100 protein was present in nevi, malignant melanomas, histiocytosis X, mixed sweat gland tumors, neural tumors, chondromas, and chondrosarcomas. Detection of S100 protein by immunostaining was useful in understanding the histogenesis of various skin tumors and in assessing the diagnosis and prognosis of a variety of skin lesions encountered in surgical pathology.
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PMID:The value of immunohistochemical studies using antibody to S100 protein in dermatopathology. 637 34

The histological and immunohistochemical findings of 34 biopsy specimens from patients with Langerhans' cell histiocytosis (LCH) are reported, with special emphasis on the findings with CD1a mouse monoclonal antibody (MAb) O10 using paraffin-embedded material. Eighteen patients were treated in an adult hospital (mean age, 26.3 years), and the 16 others were children (mean age, 3 years) from a pediatric center. Specimens included 17 bone, 14 skin, two lung, and one lymph node. Tissue was fixed in formalin or Bouin's, and most bone samples were decalcified in nitric acid. Frozen sections were available for 16 cases and electron microscopy for one. Light microscopy was suggestive of LCH in all cases, characterized by large mononucleated cells with abundant eosinophilic cytoplasm and "coffee bean" nucleus. In 33 of the 34 paraffin-embedded LCH samples, mononucleate cells were stained by MAb O10. As controls, we investigated seven tumors expressing S-100 protein (three nevi, two melanomas, two neurofibromas): all were negative with MAb O10. Five non-Langerhans' cell histiocytoses (three juvenile xanthogranulomas and two Rosai-Dorfman lymphadenopathies) were also negative with MAb O10. The results show that in most cases a definitive diagnosis of LCH can be assessed on paraffin-embedded tissue specimens with the help of immunohistochemistry using MAb O10.
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PMID:Langerhans' cell histiocytosis. Definitive diagnosis with the use of monoclonal antibody O10 on routinely paraffin-embedded samples. 775 50

Four brief reports of combined skin lesions are presented: (1) combined basal cell epithelioma and histiocytosis X. (2) combined intraepidermal melanocytic neoplasia (melanoma in situ) and leukaemia cutis, (3) combined melanocytic naevus with pseudoepitheliomatous hyperplasia and desmoplastic trichoepithelioma, and (4) combined melanocytic naevus with lichenoid gold reaction. The importance of the occurrence of such lesions is discussed, as are the clinical and pathological diagnostic difficulties they sometimes pose.
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PMID:Combined skin lesions. 890

The latest studies regarding the controversial and concerning subjects of pigmented nevi and melanoma in children are reviewed. Additional topics covered include dermatologic conditions that feature genetic mosaicism, theories of pathogenesis of Langerhans cell histiocytosis, new clinical information regarding juvenile xanthogranulomas, and the unique features of pyoderma gangrenosum in infants and children.
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PMID:Pediatric dermatology. 900 65

This is a descriptive screening of 46 examples of childhood histiocytic lesions and some of their look-alikes using a monoclonal antibody, p55, to fascin. Fascin, an actin-bundling protein, identifies dendritic cells in the blood and in tissues. Our aim was to test the diagnostic utility of the antibody in various lesions at different sites and to see whether the staining patterns give insight into the cell types involved. Fascin intensely stained the cells of juvenile xanthogranulomas (JXG), Rosai-Dorfman lesions, and soft tissue dendrocytomas. Normal Langerhans' cells and the cells of Langerhans' cell histiocytosis were unreactive. Their lack of fascin staining may be relevant to fascin being maturation as well as lineage related. Epithelioid and palisading granulomas were unstained, though an example of Kikuchi lymphadenitis had large numbers of dendritic-type cells that stained strongly. A reticulohistiocytoma of the skin was also unstained and look-alike lesions, Spitz nevi, and mast cell lesions did not stain. Two of three large-cell lymphomas (both CD30+) also had fascin reactivity. Even though fascin is not specific to dendritic cells, staining other cell types as well (false positive), and not entirely sensitive, dendritic cells such as tissue Langerhans' cells are unstained (false negative), there seems to be a consistency of staining in childhood histiocytic lesions. This may be of diagnostic use when read in the context of the tissue differential diagnosis. Whether fascin can serve as a marker for the dendritic cell lineage, or at least for some phases of dendritic cell lifecycle, is not answered by this survey.
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PMID:Fascin and the differential diagnosis of childhood histiocytic lesions. 1046 81

CD163, a hemoglobin scavenger receptor, is expressed in monocytes and macrophages. Recent work has shown that this marker is specific for neoplasms of histiocytic differentiation. Our aim was to test the ability of CD163 to separate cutaneous histiocytomas from their morphologic mimics. We tested the expression of CD163 in 78 cases, including 19 xanthogranulomas, 16 atypical fibroxanthomas, 6 reticulohistiocytomas, 8 epithelioid cell histiocytomas, 9 cases of Langerhans cell histiocytosis, 10 xanthomas, and 10 intradermal Spitz nevi. CD163 expression was seen in all xanthogranulomas and reticulohistiocytomas, 4 epithelioid cell histiocytomas, 2 cases of Langerhans cell histiocytosis, and 8 xanthomas but was absent in atypical fibroxanthomas and Spitz nevi. CD163 is an excellent marker for confirming histiocytic differentiation and is useful in eliminating morphologic mimics such as Spitz nevi from the differential diagnosis. The lack of CD163 in atypical fibroxanthomas argues against a histiocytic origin for this tumor.
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PMID:CD163 expression is present in cutaneous histiocytomas but not in atypical fibroxanthomas. 2047 50

Multiple Spitz nevi are rare and may occur in agminated, widespread, or dermatomal distributions. Agminated Spitz nevi usually arise in children, presenting on grossly normal, hyperpigmented, or most rarely, hypopigmented skin. We present a child with Langerhans cell histiocytosis who developed bilateral agminated Spitz nevi in the inguinal area. Unusual features included the multifocal distribution, bilateral inguinal location, and co-occurrence with Langerhans cell histiocytosis.
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PMID:Acquired bilateral agminated Spitz nevi in a child with Langerhans cell histiocytosis. 2060 46

Diseases of the posterior compartment and the orbit are characterised by histological findings, most of which can be reproduced clinically. Examples are the examination of calcifications in retinoblastoma by ultrasonography. In the present review, histological findings of tumour and other diseases of the posterior ocular compartment and the orbit are presented and correlated with the clinical pictures and imaging techniques: uveal melanoma, choroidal nevus, choroidal metastases, choroidal hemangioma, retinoblastoma, Coat's disease, sympathetic ophthalmia, pleomorphic adenoma (benign mixed tumour) of the lacrimal gland, dacryoadenitis, lymphoma, rhabdomyosarcoma, Langerhans cell histiocytosis, orbital metastases, and phthisical eyes. Histopathology is usually the gold standard for a definitive diagnosis. It is very important for residents and those in training to become familiar with clinico-pathological correlations as these provide insight in pathophysiological processes. Regarding ophthalmic surgery, ophthalmic pathology offers the possibility to study wound healing and complications. A close collaboration between clinicians and ocular pathologists allows for an optimised processing of the submitted tissue and diagnosis. Thus, pre- and postoperative care can also be improved. This outstanding knowledge that ophthalmologists have gained over the last decades and beyond, should be preserved and passed on to the next generations in order to maintain a high standard in ophthalmological care.
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PMID:[Clinico-pathological correlations: posterior compartment of the eye and orbit]. 2283 34

The clonal/neoplastic nature of Langerhans cell histiocytosis (LCH) has recently been demonstrated by a high prevalence of BRAF mutations, including pulmonary LCH (PLCH). We hypothesized that BRAF-induced senescence, as demonstrated in nevi and melanoma, is involved in the pathogenesis of LCH and PLCH. In a series of pulmonary (19 cases) and non-pulmonary LCH (19 cases), including five aggressive cases, we investigated occurrence of the BRAF V600E mutation by molecular analysis and/or immunohistochemistry using a validated antibody (VE1). The expression of cell-senescence markers p16(INK4a) and p21(CIP1/WAF1) was also immunohistochemically investigated. We demonstrated that 6/19 cases of LCH and 12/19 cases of PLCH were VE1 positive, matching with molecular analysis, and in all cases both p16(INK4a) and p21(CIP1/WAF1) were expressed, irrespective of BRAF mutation status. Interestingly, all the aggressive cases did not express p16(INK4a), thus suggesting that loss of senescence control could be related to clinical aggressiveness of LCH, as in melanoma.
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PMID:Oncogene-induced senescence distinguishes indolent from aggressive forms of pulmonary and non-pulmonary Langerhans cell histiocytosis. 2447 9

Agminated flexural melanocytic nevi in children with a history of Langerhans cell histiocytosis (LCH) are rare and thought to be coincidental or related to systemic chemotherapy. We report on an 11-year-old boy in remission from LCH, treated with only topical steroids, who presented years later with an eruption of melanocytic nevi in the bilateral inguinal and axillary regions. Rather than coincidence, we hypothesize that agminated flexural melanocytic nevi are a late sequela of LCH, possibly resulting from immune tolerance or a reaction to local inflammation.
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PMID:Agminated flexural melanocytic nevi: a late sequela of Langerhans cell histiocytosis? 2578 31


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