Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
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.
Int J Dermatol
PMID:The value of immunohistochemical studies using antibody to S100 protein in dermatopathology. 637 34

Two cases of adult histiocytosis X have been studied using monoclonal antibodies on skin sections by two techniques: indirect immunofluorescence and immunoperoxidase. This study confirm: --that histiocytosis X express two specific antigens Ia and T6, --the relations between Langerhans cell and histiocytosis X. Especially, it suggests that histiocytosis X cell would be a dedifferentiated cell with receptors OKT4 and OKT10.
Ann Dermatol Venereol 1984
PMID:[Value of correlated immunofluorescence and immunoperoxidase study of monoclonal markers in 2 adult cases of histiocytosis X]. 637 21

The ability to define subpopulations of immunologically competent lymphocytes has permitted an enhanced understanding of the interaction between functionally distinct components of the immune system. T cells can provide help in antibody formation or they may suppress antibody production. Abnormal immunoregulatory mechanisms have been demonstrated in the hyperimmunoglobulin E-recurrent infection syndrome. This disorder is associated with a marked elevation of IgE and specific elevations of IgE antibodies directed toward staphylococcal antigens. Abnormal T cell regulation of immune responses has been demonstrated. Graft-versus-host disease (GVHD) occurs in an immunodeficient patient who has received an infusion of immunocompetent cells. The diagnosis of graft-versus-host (GVH) reaction may be complicated by the protean manifestations of the disorder. The acute form, consisting of a maculopapular rash, fever, and diarrhea, may be confused with acute infection or drug reaction. Chronic GVHD has been incorrectly diagnosed as histiocytosis X, acrodermatitis enteropathica, or scleroderma. Utilizing chromosome markers and/or identification of histocompatibility antigens, the presence of circulating lymphocytes from donor immunocompetent cells (blood transfusion, maternal source) can be documented. The development of sensitive technics for identifying cells can establish a precise diagnosis. Certain immunodeficiency disorders can be identified by biochemical means. Biotin-dependent multiple carboxylase enzyme deficiency is associated with a chronic dermatitis, alopecia, ataxia, and secondary infection of the skin with Candida. The disorder responds promptly to the administration of biotin with correction of dermatologic, neurologic, and immunologic abnormalities.
J Am Acad Dermatol 1984 Oct
PMID:New insight into the causes of immunodeficiency disorders. 638 1

The case of an 81-year-old woman suffering for about 1 year from a typical form of Letterer-Siwe disease (LSD) is reported. The only important systemic involvement was hepatosplenomegaly. The patient was treated with steroids and vinblastine and then with thymopoietin pentapeptide. This last drug proved useful for decreasing the dose of steroid and thus for checking the appearance of new skin lesions. The ultrastructural investigations, carried out before and after vinblastine, showed the presence in some histiocytosis X cells (HXC) of coated Birbeck granules (BG). Immunoelectron microscopy showed about 90% of HXC to be OKT6+ and the OKT6 labeling of these cells to be similar to that of the Langerhans cells of normal skin. Five percent of OKT6+ cells apparently contained no Langerhans granules.
J Am Acad Dermatol 1984 Feb
PMID:Letterer-Siwe disease in an octogenarian. 654 37

Two adult patients with skin lesions typical of histiocytosis X were treated topically with mechlorethamine hydrochloride. In the patient whose disease was limited to the skin, a sustained remission was induced, but in the patient with extensive systemic disease, cutaneous remission lasted only six weeks.
Arch Dermatol 1983 Feb
PMID:Histiocytosis X in two adults. Treatment with topical mechlorethamine. 660 May 96

Morphologic similarities between histiocytosis X (HX) cells and epidermal Langerhans cells (LC) have led to the hypothesis that HX represents a proliferative disorder of LC. In order to prove the validity of this assumption, we tested single cell suspensions isolated from an eosinophilic granuloma type HX lesion for the presence of various antigenic determinants defined by monoclonal antibodies using an immunoelectron microscopic technique. An anti-Ia reagent reacted with essentially all histiocytic cells and a small portion of lymphocytes whereas plasma cells and eosinophils were negative. T6 antigen, in contrast, was disclosed exclusively on HX cells either with or without Birbeck granules. Pan-T cell-reagent OKT3 reacted only with small lymphocytes. The finding that HX cells from eosinophilic granuloma lesions are the only cells that have the identical surface marker equipment as epidermal LC (Ia antigens, T6 antigen, Fc-IgG, and C3 receptors) strongly supports the concept that these cells are derived from the LC lineage.
J Invest Dermatol 1983 May
PMID:Histiocytosis X cells in eosinophilic granuloma express Ia and T6 antigens. 660 81

A 2-year-old girl with extensive cutaneous xanthogranuloma and recurrent subdural effusions has been investigated. Differentiation from histiocytosis X depended upon light and electron microscopical studies of the cutaneous infiltrate and cells obtained from the cerebrospinal fluid. The recurrent subdural effusions responded well to cranial irradiation. Although juvenile xanthogranuloma is generally a benign cutaneous disorder, a spectrum of naevoid proliferative changes involving systemic organs may occur.
Br J Dermatol 1981 Jul
PMID:Juvenile xanthogranuloma with recurrent subdural effusions. 678 61

The case histories of two elderly patients with cutaneous histiocytosis X treated topically with nitrogen mustard are presented. The cutaneous lesions cleared within 2 to 3 weeks, and remission was maintained with daily topical administration of nitrogen mustard. The clinical impression of improvement was substantiated by light and electron microscopic studies prior to and after therapy.
J Am Acad Dermatol 1980 Jul
PMID:Histiocytosis X: treatment with topical nitrogen mustard. 696 94

A patient with histiocytosis X had nonhealing mouth lesions. Evaluation revealed diabetes insipidus and numerous bony lesions. Electron microscopy of the mouth lesions and lymph nodes did not indicate Langerhans cells, which have frequently been described as occurring in histiocytosis X. A survey of 59 patients examined during a 20-year period at UCLA reported the presence of oral lesions in 42% of the cases and confirmed the occurrence of a wide range of clinical patterns and variations in the course of the disease.
Arch Dermatol 1981 May
PMID:Histiocytosis X. 697 25

Six children with histiocytosis X, all in clinical remission, were investigated for antibody-dependent cytotoxicity mediated by monocytes and neutrophils. Monocytes demonstrated a reduced cytotoxicity and a normal Fc receptor activity. Judged by light microscopy the monocytes were normal. Preincubation with the patient's serum did not influence the cytotoxicity of normal monocytes. Neutrophils from patients with histiocytosis X showed a normal cytotoxic activity. We postulate a functional defect of the mononuclear phagocyte system in histiocytosis X.
Br J Dermatol 1981 Jul
PMID:Histiocytosis X--an immune deficiency disease? Studies on antibody-dependent monocyte-mediated cytotoxicity. 697 53


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>