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Biomedical subjects

J Wechsler

Publications and source records attributed to J Wechsler.

At least 145 records · Page 8Linked to original sources
PubMed1993

Autoimmune pemphigus. A distinct staining pattern with an anti-desmoglein antibody.

BACKGROUND AND DESIGN: Diagnosis of autoimmune pemphigus is based on the immunodetection of IgG deposits in the epidermal intercellular spaces. Desmoglein is a desmosomal component that has been assumed to be the antigen of pemphigus foliaceus. We investigated the use of one monoclonal antibody (32-2B) to desmoglein 1 on paraffin sections. Twenty-nine pemphigus samples were studied (22 pemphigus vulgaris, seven pemphigus foliaceus). RESULTS: In 29 patients suffering from autoimmune pemphigus, the staining for desmoglein was represented by coarse granules along the cytoplasmic borders of epithelial cells in lesional and perilesional skin and in mucous membranes. This peculiar staining is different from the fine dots along the cytoplasmic membrane observed in normal skin. This abnormal staining seems to be specific for pemphigus. Hereditary acantholytic diseases and other diseases such as bullous pemphigoid, intraepidermal IgA dermatosis, eczema, herpes, and transient acantholytic dermatosis show a similar pattern to normal skin. CONCLUSION: The use of 32-2B on biopsy specimens in cases of autoimmune pemphigus demonstrated a specific staining pattern. It could be used on paraffin sections as a diagnostic test of autoimmune pemphigus. It can even be done retrospectively.

Antibodies, Monoclonal↗
PubMed1993

The spectrum of vasculitis in human immunodeficiency virus-infected patients. A clinicopathologic evaluation.

OBJECTIVE: To delineate the different types of inflammatory vascular diseases (IVD) occurring in patients with human immunodeficiency virus (HIV) infection. METHODS: Muscle, nerve, or skin biopsy specimens from 148 symptomatic HIV-infected individuals were reviewed, and subgroups of vasculitis were identified using the American College of Rheumatology (ACR) 1990 clinicopathologic criteria for the classification of vasculitis. RESULTS: IVD was documented in 34 patients (23%) and included necrotizing arteritis (3 patients), non-necrotizing arteritis (1 patient), neutrophilic IVD (7 patients), mononuclear IVD (17 patients), and other small vessel inflammatory changes (6 patients). According to the ACR criteria, 11 patients could be classified as having a distinct category of vasculitis, including polyarteritis nodosa (4 patients), Henoch-Schönlein purpura (1 patient), and drug-induced hypersensitivity vasculitis (6 patients), and 23 were classified in the group "other vasculitis, type unspecified." One patient had hepatitis B virus surface antigenemia, 2 had cryoglobulinemia, and 2 were coinfected by human T lymphotropic virus type I. Cytomegalovirus inclusions and antigens were found in endothelial cells in 1 patient. HIV antigens and genome were detected in perivascular cells of 2 of the 3 patients with necrotizing arteritis; in 1, HIV-like particles were seen by electron microscopy. Immune deposits were found in small vessel walls in 5 skin biopsy samples showing small vessel vasculitis and in the muscle of the 3 patients with necrotizing arteritis. CONCLUSION: A wide range of inflammatory vascular diseases may occur in HIV-infected individuals. Vascular inflammation appears multifactorial and may result from HIV-induced immunologic abnormalities and exposure to a variety of xenoantigens, such as HIV itself, other infectious agents, and drugs.

Adult↗
PubMed1993

Cutaneous side effects associated with interleukin 2 administration for metastatic melanoma.

BACKGROUND: Cutaneous changes associated with recombinant interleukin 2 (r IL-2) administration are frequent but have been rarely studied in a large series. OBJECTIVE: We analyzed these clinical, microscopic, and immunologic changes. METHODS: Patients with metastatic melanoma treated with r IL-2 were studied. The eruption was scored as mild or severe. Biopsy specimens were obtained for histopathology, ultrastructural analysis, and immunophenotyping. Chi-square and t tests were used for statistics. RESULTS: Twenty-five patients were included. Eruptions were observed in 56 of 78 cycles (72%); 53 were mild with a burning pruriginous erythema, and 3 were severe with urticaria, necrotic lesions, and blisters. Regression was constant without sequelae. Pathologic changes were mild with a mononuclear cell infiltrate of activated helper T phenotype, expressing LFA-1. Keratinocytes and endothelial cells displayed intercellular adhesion molecule-1 and HLA-DR. Cells rarely expressed CD25. CONCLUSION: Administration of r IL-2 triggers non-treatment-limiting cutaneous inflammation.

Adult↗
PubMed1993

A homozygous insertion-deletion in the type VII collagen gene (COL7A1) in Hallopeau-Siemens dystrophic epidermolysis bullosa.

The Hallopeau-Siemens type of recessive dystrophic epidermolysis bullosa (HS-RDEB) is a life-threatening autosomal disease characterized by loss of dermal-epidermal adherence with abnormal anchoring fibrils (AF). We recently linked HS-RDEB to the type VII collagen gene (COL7A1) which encodes the major component of AF. We describe a patient who is homozygous for an insertion-deletion in the FN-4A domain of the COL7A1 gene. This defect causes a frameshift mutation which leads to a premature stop codon in the FN-5A domain, resulting in a marked diminution in mutated mRNA levels, with no detectable type VII collagen polypeptide in the patient. Our data suggest strongly that this null allele prevents normal anchoring fibril formation in homozygotes and is the underlying cause of HS-RDEB in this patient.

Base Sequence↗
PubMed1993

Acquired C1 inhibitor deficiency revealing systemic lupus erythematosus.

We report the case of a previously healthy 54-year-old woman, without any family history, who developed an angioneurotic edema with acquired C1 inhibitor deficiency and systemic lupus erythematosus. The search for a lymphoproliferative disorder was negative. Hydroxychloroquine therapy induced simultaneous resolution of lupus and angioedema. The pathophysiology of this association is discussed.

Angioedema↗
PubMed1993

[Diffuse cholesterol embolism after fibrinolysis for myocardial infarction].

The authors report the case of a 57 year old man admitted to hospital 3 hours after the onset of an infero-latero-basal infarct who underwent thrombolytic therapy with intravenous streptokinase, in whom the immediate outcome was complicated by multiple cholesterol embolisation which cause renal failure, mesenteric ischemia, medullary ischemia and livedo reticulosis of the lower half of the body complicated by gangrene of the toes, despite the fact that cardiac catheterisation had not been performed. This case (fifth reported case) of multiple cholesterol embolisation from the abdominal aorta caused by thrombolysis is the earliest presentation of this complication in the literature.

Cholesterol↗
PubMed1993

Impaired antigen presentation in toxic epidermal necrolysis.

BACKGROUND AND DESIGN--The pathophysiology of toxic epidermal necrolysis (TEN) remains largely unknown. Toxic epidermal necrolysis is considered a hypersensitivity reaction to drugs, but direct evidence of an immunologic mechanism is still lacking. Lymphopenia and a decrease in the numbers of circulating CD3- and CD4-positive lymphocytes have been reported in acute phase, but, to our knowledge, no study of cellular immune functions of patients with TEN has been reported so far. Herein, we investigated several T-cell functions in a series of 11 patients with TEN. Peripheral blood mononuclear cells (PBMC) obtained in the acute phase were tested together with PBMC obtained after the patient's recovery and compared with those of age- and sex-matched healthy control subjects. RESULTS--Phytohemagglutinin-induced proliferations and lymphocyte responses in allogeneic mixed lymphocyte reactions were not impaired in the acute phase compared with those after recovery in the same patients and with those in control subjects. In contrast, natural killer cytotoxicity and allogeneic cytotoxic responses were significantly decreased in early TEN. The most striking feature was the significantly impaired ability of acute-phase lymphoid cells to activate allogeneic T cells. Patient PBMC in acute-phase TEN did not inhibit the proliferation induced by patient PBMC after recovery, suggesting that their defect was not related to the presence of radioresistant suppressor cells. The phenotypic expression of HLA-DR, -DQ, and -DP antigens on circulating peripheral blood lymphocytes was then assessed by immunoalkaline phosphatase staining and flow cytometry. Results showed decreased percentages of HLA-DR-positive mononuclear cells and a decreased density of HLA-DR antigens, mainly on monocytes, in acute-phase TEN. CONCLUSIONS--These results demonstrate that peripheral blood lymphocytes of patients with TEN have an impaired ability to activate allogeneic T cells. This defective antigen presentation is not secondary to the presence of suppressor lymphocytes, but it is probably related to a decreased expression of HLA-DR antigens on circulating mononuclear cells in acute-phase TEN.

Adolescent↗
PubMed1992

Sclerodermatous chronic graft-versus-host disease. Analysis of seven cases.

BACKGROUND: Sclerodermatous chronic graft-versus-host disease is a disabling complication after allogeneic bone marrow transplantation from HLA-identical sibling donors. Only a few series of patients have been reported and the dermatologic features have never been extensively described. OBJECTIVE: The purpose of the study was to describe clinical and biologic features of chronic sclerodermatous graft-versus-host disease and to compare them with scleroderma. METHODS: We reviewed 196 patients grafted between April 1973 and July 1987 with survival times sufficient to be at risk of chronic graft-versus-host disease. Seven had the sclerodermatous form. RESULTS: Most patients had disseminated sclerosis of the trunk and the proximal portions of the limbs. In two cases, atrophy of the skin was predominant, corresponding with a severe clinical evolution. Periorbital pigmentation was observed as an initial manifestation in three cases. Visceral manifestations resembled those observed in scleroderma but histologic and immunologic studies demonstrated clear differences. Response to therapy was variable. CONCLUSION: Chronic sclerodermatous graft-versus-host disease may realize two different patterns. Major atrophy is associated with a more severe progression.

Adolescent↗
PubMed1992

Intraepidermal localization of the clone in cutaneous T-cell lymphoma.

BACKGROUND: No immunohistologic techniques are currently available to demonstrate clonality of T-cell lymphomas. Monoclonal antibodies to the variable region of the T-cell receptor (TCR) have been produced that identify minor populations of normal peripheral blood T lymphocytes. OBJECTIVE: We investigated the expression of TCR V-region genes in cutaneous lymphomas to determine whether immunostaining with these antibodies may be a simple method to detect clonal T-cell proliferations and help to distinguish benign lymphoid infiltrates from malignant lymphoma. METHODS: Cutaneous samples were obtained from 18 cutaneous T-cell lymphomas (14 mycosis fungoides, 1 Sézary syndrome, 2 pleomorphic T-cell lymphoma, and 1 large cell anaplastic lymphoma) and 8 benign lymphoid infiltrates. Frozen sections were incubated with monoclonal antibodies and stained by the alkaline phosphatase-antialkaline phosphatase technique. Staining was performed with a panel of 7 anti-TCR V-region antibodies, 6 T-cell markers, 1 anti-beta chain antibody, and 1 anti-delta chain antibody. RESULTS: Clonality could be demonstrated in 2 of 18 cutaneous lymphomas. We observed the strictly intraepidermal localization of clonal proliferation in one case of early-stage mycosis fungoides. CONCLUSION: Anti-TCR V-region antibodies may identify a strictly epidermotropic clone in early mycosis fungoides. However, the panel of antibodies currently available stains only a minority of cutaneous T-cell lymphomas. The usefulness of these antibodies as a clonotypic marker needs to be reevaluated when a larger panel of antibodies becomes available.

Adult↗