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

A case of Langerhans' cell histiocytosis with unusual skin manifestations in the form of multiple large skin tumors is described. The skin lesions responded partially to chemotherapy with etoposide and prednisone, and residual lesions were excised surgically. The patient developed central diabetes insipidus during treatment.
Arch Dermatol 1990 Dec
PMID:Unusual skin tumors in Langerhans' cell histiocytosis. 186 87

We describe an infant who presented at birth with numerous haemorrhagic and encrusted skin lesions, hepatomegaly, lymphadenopathy, raised hepatic transaminases, leucopenia and thrombocytopenia. The diagnosis of Langerhans cell histiocytosis was confirmed by immunohistochemistry, which demonstrated the presence of CD1, S-100 and DR positive cells in the skin infiltrate. The skin lesions resolved spontaneously after 6 weeks but recurred at 3 months and again were self involuting with resolution by 9 months. Persistent circulating T-cell abnormalities, including T-cell lymphopenia and the presence and persistence of peripheral blood CD1 + cells were noted throughout the first year of life.
Br J Dermatol 1990 Apr
PMID:Congenital self-healing Langerhans cell histiocytosis with persistent cellular immunological abnormalities. 233 23

Histiocytosis X and mastocytosis are proliferative processes that may have similar cutaneous manifestations. However, a positive Darier's sign (urtication on stroking of the lesion) is thought to reliably distinguish between these two diseases. We recently studied a 13-year-old girl with a 2-year history of extensive skin lesions and a positive Darier's sign. Routine histopathologic studies revealed a polymorphous cutaneous infiltrate composed of histiocytes, mast cells, eosinophils, and lymphoid cells. Electron microscopic studies demonstrated Langerhans granules in some of the histiocytes, and immunologic studies of frozen tissue showed that a significant subpopulation of the histiocytes marked as Langerhans cells. Giemsa staining of specimens from eight other cases of cutaneous histiocytosis X from our files revealed mast cells in all of the lesions, although none showed the abundance of mast cells present in the case with urtication. Our studies emphasize the often polymorphous nature of the cell population in cutaneous histiocytosis X and demonstrate that confusing clinical findings can result when the mast cell population in histiocytosis X produces urtication.
J Am Acad Dermatol 1986 May
PMID:Urticating histiocytosis: a mast cell-rich variant of histiocytosis X. 242 66

We compared peanut agglutinin and S100 stains on paraffin embedded material from a variety of sites involved in Langerhans cell histiocytosis (LCH). The two techniques were comparable in terms of ease of performance, time taken and cost. Peanut agglutinin produced dense cell surface and paranuclear staining of the characteristic LCH cells seen in lesions, which was easier to distinguish than the more diffuse cytoplasmic staining produced by the S100 technique. This characteristic staining pattern is more specific than that for the S100 protein which is present in a variety of different cells, including some malignant histiocytes. We recommend that peanut agglutinin be used as a routine diagnostic test for all samples suspected of being LCH.
Br J Dermatol 1988 Oct
PMID:Comparison of peanut agglutinin and S100 stains in the paraffin tissue diagnosis of Langerhans cell histiocytosis. 246 Dec 16

The histiocytic syndromes are currently divided into two major categories: Langerhans cell histiocytosis and non-Langerhans cell histiocytosis. The disease entities recognized under these categories are discussed. The discussion includes clinical features, histopathology, and treatment.
Dermatol Clin 1989 Jul
PMID:Histiocytic syndromes. 254 4

Langerhans cell histiocytosis (LCH), or histiocytosis X, is now generally considered to be a non-malignant condition. A flow cytometric (FCM) study of a single case has, however, been published which claimed to provide evidence to contradict this. The presence of DNA-ploidy as detected using this technique is a feature of malignant and pre-malignant disease. In this reported single case, DNA-ploidy was present but the clinical features of this patient were atypical for LCH. We have performed a FCM study of the DNA of nine biopsies of LCH lesions from six patients with well-established disease. In addition, in one of these, fresh tissue studies including the use of an anti-CD I monoclonal antibody to specifically label the LCH cells were performed. In all cases the DNA content of the cells was entirely normal. We therefore found no evidence that LCH is a neoplastic disorder.
Br J Dermatol 1989 Apr
PMID:A flow cytometric study of Langerhans cell histiocytosis. 265 63

The physiology of the histiocyte (macrophage) in health and disease is reviewed briefly. An overview of the so-called primary malignant, pseudomalignant, and benign histiocytic disorders, excluding histiocytosis X, is presented. The malignant histiocytosis with erythrophagocytosis, the pseudomalignant histiocytic diseases (such as sinus histiocytosis with massive lymphadenopathy and regressing atypical histiocytosis), and the solitary lesions with histologic malignant and atypical storiform histiocytosis are described. Two groups of adult histiocytic diseases are reviewed; one is characterized by nonfamilial and familial histiocytic dermatoarthritis and the other by multiple widespread benign lesions, such as xanthoma disseminatum, generalized eruptive histiocytoma, nodular non-X histiocytosis, and various xanthomatous eruptions associated with paraproteinemia. Finally, multiple benign cutaneous histiocytic lesions of childhood, such as juvenile xanthogranuloma and congenital self-healing histiocytosis, are included.
Arch Dermatol 1985 Dec
PMID:Primary histiocytic dermatoses. 299 86

Cutaneous histiocytosis may take two principal forms. It is either a benign proliferative process or a relentless, progressive process with a poor prognosis. In histiocytic medullary reticulosis, histiocytes demonstrate nuclear atypia and the outcome is uniformly fatal. Benign cephalic histiocytosis X causes lesions similar to those of histiocytosis X, but Langerhans' cells are absent. In congenital self-healing histiocytosis X, the Letterer-Siwe-like cutaneous infiltrate contains Langerhans' cells, but the lesions heal spontaneously without treatment. The nodular cutaneous lesions of juvenile xanthogranuloma appear in infancy and resolve without treatment; however, the higher percentage (10%) of associated ocular lesions may lead to glaucoma and blindness. In histiocytosis X, the cutaneous lesions show a marked proliferation of Langerhans' cells, with prognosis dependent on the patient's age and the extent of organ dysfunction. Patients who survive the acute form of the disease may develop diabetes insipidus, growth retardation, pulmonary fibrosis, and biliary cirrhosis. A subtle immunologic defect has been identified in patients with histiocytosis X, yet the pathogenesis of the disease is still speculative. Familial disease occurring in early infancy should be differentiated from complete or partial immunodeficiency syndromes. Guidelines for evaluating patients with cutaneous histiocytosis are reviewed.
Pediatr Dermatol 1985 Nov
PMID:Cutaneous histiocytosis syndromes. 299 40

Biopsy of a solitary tumor of the buttock in a 3-month-old girl was diagnosed as a histiocytic proliferation suggestive of histiocytosis X. Electron microscopy showed Birbeck granules and dense myelinlike bodies within the cytoplasm of the tumor cells. An immunoperoxidase study, using a panel of monoclonal antibodies (OKT6, OKT4, and OKM1) and a polyclonal anti-S100 protein antibody, showed positive staining for OKT6 and OM1 and moderately positive staining for OKT4 and S100. After surgery to remove the tumor, visceral involvement could not be demonstrated during a 20-month follow-up.
Arch Dermatol 1986 Sep
PMID:Solitary Langerhans cell histiocytoma. 301 34

Plasminogen activators (PA) play an important role in cell migration and tissue degradation. Considering the strong epidermotropism of atypical mononuclear cells in histiocytosis X (HX) skin infiltrates leading to intraepidermal abscess formation, it was the purpose of this study to look for tissue-type PA (t-PA) and/or urokinase-type PA (u-PA) on HX cells. Four monoclonal antibodies against PA were used, employing the alkaline phosphatase anti-alkaline phosphatase (APAAP) technique on cryostat sections from four patients with HX. Twenty percent to 40% of infiltrating cells in the epidermis expressed the t-PA antigen. t-PA+ cells were present in the follicular centers of human tonsil, absent in normal epidermis and scanty in cutaneous infiltrates from mycosis fungoides and lupus erythematosus. Double labeling with anti-PA and T6 (CD1) or S100 protein revealed some of the HX cells to express both antigens (t-PA+ CD1+ or t-PA+ S100+). We conclude that cutaneous infiltrates of HX contain PA+ dendritic cells which are different from normal Langerhans cells and which may be responsible for the strong epidermal alterations in HX.
Arch Dermatol Res 1987
PMID:Cutaneous infiltrates of histiocytosis X contain plasminogen activator-bearing epidermotropic dendritic cells different from Langerhans cells. 311 52


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