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

We report the case of a 49-year-old woman suffering from a malignant neoplasm of Langerhans cells (LC), documented by immunohistochemical and ultrastructural analysis, and review the literature to examine and characterize the clinical and laboratory features, therapy, and prognosis of malignant neoplasms of LC. Langerhans cell histiocytosis is now regarded as a disorder of immune regulation or an inflammatory process, rather than as a malignant neoplasm. Although LC share certain features in common with ordinary histiocytes or interdigitating dendritic cells, they also differ significantly from these cells in other respects. Therefore, we propose designating a malignant neoplasm of LC 'malignant Langerhans cell tumour' and that it should be considered as a separate entity from Langerhans cell histiocytosis or other malignant histiocytoses.
Br J Dermatol 1992 Apr
PMID:Malignant Langerhans cell tumour. 157 Dec 64

Seven cases of congenital Langerhans' cell histiocytosis (LH) are reported, with emphasis on clinical and immunohistochemical features. This is a polymorphic disease at birth. In 4/7 cases, the diffuse, generalized rash could be classified as cutaneous Letterer-Siwe disease (LSD); 3/4 remained purely cutaneous and healed in less than 3 months; whereas the fourth-one persisted, pulmonary lesions appeared, and the infant died on his 40th day. In 3/7 cases, the clinical diagnosis at birth was either a Blueberry Muffin Baby (BMB) or Hashimoto-Pritzker type LH (HPLH); the lesions healed rapidly, although one cas was contradictory: typical BMB at birth, histology mimicking a monoblastic cutaneous leukemia, no T.O.R.C.H. syndrome, normal bone marrow, immunophenotyping of LH, auto-involution; 2/3 were MZ twins, both with few lesions. We would like to stress the fact that the clinical spectrum of LH should include BMB, which, however, in most cases must be considered a differential diagnosis. Regarding cutaneous congenital LH, an eponymic classification (LSD, HPLH) is difficult to follow strictly, because overlapping pictures are observed. There is a wide spectrum of cutaneous congenital LH. The main problem at birth is the lack of prognostic criteria. Neither the presence of the rash at birth, nor its type and extension, is necessarily evidence of risk of systemic disease. Cases of HPLH involute, as also do cases of cutaneous LSD, and the "Blueberry Muffin" type of LH; overlapping clinical aspects exist. Histopathological data, electron microscopy or immunohistochemistry, define LH, but they do not enable the outcome to be predicted.(ABSTRACT TRUNCATED AT 250 WORDS)
Ann Dermatol Venereol 1992
PMID:[Congenital cutaneous Langerhans histiocytosis. Apropos of 7 cases]. 160 6

Four cases of benign Langerhans cell histiocytosis limited to the skin were studied. In all three self-healing cases (cases 2, 3, and 4) many dense bodies, myelin bodies, and worm bodies were found. In one chronic case (case 1) none of these was identified. In all four cases, in addition to CD1, HLA-DR, and S-100 stains, interferon-gamma and S-100 beta-subunit were positive in the dermal tumor cells. Both interferon-gamma and S-100 beta-subunits were negative in the normal epidermal Langerhans cells. A comprehensive literature review yielded 87 cases of skin-limited Langerhans cell histiocytosis. These cases could be subgrouped into three categories: (1) those that resolved spontaneously, (2) those that responded to therapy and had no recurrence, and (3) those with persistent or recurrent lesions, not responding to therapy but still limited to the skin.
J Am Acad Dermatol 1991 Dec
PMID:Immunohistochemistry and electron microscopy in Langerhans cell histiocytosis confined to the skin. 168 9

Histiocytic cells infiltrating the lesions in eosinophilic granuloma of bone as well as in cutaneous histiocytosis X were studied using a murine monoclonal antibody (MA) produced with proliferating cells from an eosinophilic granuloma of bone. This MA reacts with Langerhans cells (LC) of normal human skin or mucous membranes and with proliferating cells of eosinophilic granuloma of bone and skin lesions of Letter-Siwe disease, as shown by immunohistochemistry and immunogold labelling. As other murine MA's obtained after immunization with human cortical thymocytes, this MA immunoprecipitates the 49-kDa CD1a antigen found on human LC and thymic-cell surfaces but not its breakdown product after treatment with trypsin, as demonstrated by analysis of immunoelectron labelling, cytofluorometry and gel electrophoresis. This first production of a CD1a MA from an eosinophilic granuloma supports the concept of Langerhans-cell histiocytosis.
Clin Exp Dermatol 1991 Sep
PMID:Eosinophilic granuloma of bone and biochemical demonstration of 49-kDa CD1a molecule expression by Langerhans-cell histiocytosis. 172 15

We report a 13-year-old boy with localized Langerhans cell histiocytosis. The lesion was restricted to the skin of the mons pubis and clinically resembled lichen aureus. Histopathologic and electron microscopic examinations of a skin biopsy specimen of the lesion showed typical features of Langerhans cell histiocytosis. Extensive examination revealed no other organ involvement.
Pediatr Dermatol 1991 Sep
PMID:Langerhans cell histiocytosis masquerading as lichen aureus. 148 92

We describe an adult with progressive LCH who received oral etoposide as primary treatment. The response is documented and the strategical implications of this drug in Langerhans-cell histiocytosis discussed. Langerhans-cell histiocytosis (LCH) is the term recognized since 1987 for the group of diseases previously designated histiocytosis X. It is not now regarded as a malignant neoplastic process and there is some evidence to suggest that it is due to abnormal immunity, but cytotoxic drugs and steroids are still the mainstay of systemic treatment. Etoposide (VP16) is a semisynthetic epipodophyllotoxin derivative effective in the treatment of malignancies of the monocyte-macrophage lineage and used in resistant or relapsed childhood LCH. There are no previous reports of its use as firstline monotherapy in adults with LCH.
Clin Exp Dermatol 1991 Jul
PMID:Langerhans-cell histiocytosis--excellent response to etoposide. 179 74

We present a young man with the classical features of multisystem Langerhans cell histiocytosis (LCH), with emphasis on his disability and demonstrate his dramatic response to Etoposide when other treatments had clearly failed. We review the literature regarding oral and bone involvement in this disease and relate it to long term prognosis.
Clin Exp Dermatol 1991 Nov
PMID:Oral, skin and bone multisystem Langerhans cell histiocytosis and its response to etoposide--a case report. 180 25

An unusual adult case of Langerhans cell histiocytosis is presented. The case initially mimicked granuloma annulare, both clinically and histologically. The patient subsequently had development of extensive cutaneous involvement that pathologically revealed a diffuse infiltrate of CD1-positive histiocytic cells containing Langerhans granules. Extensive investigations failed to detect systemic involvement. The patient's cutaneous eruption did not respond to various therapeutic interventions, including phototherapy with oral psoralen with long-wave UV radiation in the A range (PUVA) and systemic vinblastine sulfate. Marked but temporary clinical and histologic improvement was achieved with total body electron beam radiotherapy. The nosology of this case is discussed in the context of the various histiocytic proliferative disorders.
Arch Dermatol 1991 Oct
PMID:Primary cutaneous Langerhans cell histiocytosis in an adult. 192 63

Langerhans cells histiocytosis, one of a group of histiocytosis syndromes characterized by Langerhans cell infiltration, has many clinical manifestations. In the past 30 years, numerous cases of presumed Letterer-Siwe disease, the acute multiorgan variant, have been reported in twins and siblings. Only recently has the Histiocyte Society established a criterion for a "definitive diagnosis" of Langerhans cell histiocytosis--the presence of Birbeck granules within the cells of the histiocytic infiltrate. We report the fatal outcome of Langerhans cell histiocytosis in monozygotic twin infants. There is no satisfactory explanation why Langerhans cell histiocytosis occurs concurrently in twins. We suggest that cytokines may provide an endogenous signal that triggers the pathologic proliferation of Langerhans cells.
J Am Acad Dermatol 1991 Jan
PMID:Langerhans cell histiocytosis in monozygotic twins. 199 26

The diagnostic reliability of ultrastructural and immunohistochemical examinations on routinely processed biopsy specimens of cutaneous histiocytic proliferations (histiocytosis X, n = 7; juvenile xanthogranuloma, n = 4; necrobiotic xanthogranuloma, n = 2; traumatic granuloma of the tongue, n = 1) was evaluated. S-100 protein, peanut agglutinin, and the antibody Mac-387 were used as markers for histiocytes. The frequency of Birbeck granule-containing cells in seven histiocytosis X lesions did not correspond with the number of S-100+ or peanut agglutinin+ cells. All neoplastic histiocytosis X cells were positive for S-100 protein and peanut agglutinin but were negative for Mac-387. Histiocytes of juvenile xanthogranuloma, necrobiotic xanthogranuloma, and traumatic granuloma were strongly positive for Mac-387 but were negative for S-100 protein and peanut agglutinin, except for the peanut agglutinin-reactive Touton giant cells. Mac-387 reliably differentiates histiocytic proliferations of the monocyte/macrophage system from those of the dendritic cell system. For the diagnosis of histiocytosis X, both S-100 protein and peanut agglutinin positivity in histiocytes is as reliable as ultrastructural demonstration of Birbeck granules.
J Am Acad Dermatol 1990 Nov
PMID:Immunohistochemical and ultrastructural study of histiocytosis X and non-X histiocytoses. 212 93


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