1/6. Clinical association of autoantibodies to fibrillarin with diffuse scleroderma and disseminated telangiectasia.Circulating autoantibodies against a variety of nuclear and nucleolar antigens are characteristic serologic findings in systemic scleroderma. Some of these antibodies correlate with clinical subsets of the disease. We describe three patients with systemic scleroderma and high autoantibody titers against U3 ribonucleoprotein-associated fibrillarin, a recently identified 34 kD nucleolar protein. These patients showed a progressive course with multiple organ and diffuse skin involvement with disseminated telangiectasia.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/6. Acquired von Willebrand disease associated with an inhibitor to factor viii antigen and gastrointestinal telangiectasia.A patient with acquired von Willebrand disease and gastrointestinal telangiectasia is described. He presented with the recent onset of spontaneous hemorrhage and demonstrated a prolonged bleeding time, reduced factor viii coagulant (FVIII:C), and undetectable factor viii-related antigen (FVIIIR:AG) and ristocetin cofactor (FVIIIR:WF). Following transfusion of cryoprecipitate, there was a smaller than expected immediate increase in FVIII:C, FVIIIR:WF and FVIIIR:AG, with a rapid return to baseline levels and no secondary increase in FVIII:C. An inhibitor could be demonstrated in the patient's plasma which markedly decreased the level of FVIIIR:AG in normal plasma whereas it only weakly decreased the activity of FVIIIR:WF and FVIII:C. The inhibitor was contained in the immunoglobulin g(IgG) fraction of plasma and lacked precipitating properties. This inhibitor demonstrates a specificity not previously seen in spontaneous antifactor VIII antibodies. Our report also demonstrates that ristocetin-induced agglutination of platelets is not always the most sensitive method for detecting an inhibitor in acquired von Willebrand disease.- - - - - - - - - - ranking = 5keywords = antigen (Clic here for more details about this article) |
3/6. Coexistence of crest syndrome and primary biliary cirrhosis. Serological studies of two cases.Two patients with characteristic features of crest syndrome and primary biliary cirrhosis are reported. Sera of both patients contained autoantibodies to centromere and mitochondrial antigens. immunodiffusion analysis identified the specificities of precipitating antibodies to mitochondria of the first case as anti-M-A and M-C, and of the second case as anti-M-A and M-B antibodies. Simultaneous occurrence of 2 marker antibodies in an individual patient indicates that the 2 patients reported here have crest syndrome and primary biliary cirrhosis, both of which are considered as a distinct clinical entity.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/6. The crest syndrome: a distinct serologic entity with anticentromere antibodies.The crest syndrome is a variant of systemic sclerosis characterized by the presence of calcinosis. Raynaud's phenomenon, esophageal motility abnormalities, sclerodactyly and telangiectasia. The serums of 27 patients with the crest syndrome have been examined for the presence of antinuclear antibodies. Twenty-six of 27 (98 percent) serums contained high titers (> one:80) of an antibody that produces a discrete speckled pattern of immunofluorescence on a human laryngeal carcinoma cell line (HEp-2). The antibody has been shown to react with the centromeric region of metaphase chromosomes. This antibody was also found in three of 14 patients with Raynaud's disease, in one of 60 patients with systemic lupus erythematosus, in three of 26 patients with systemic sclerosis with diffuse scleroderma and in one of 15 patients with mixed connective tissue disease. The antibody was not detected in the serums of patients with rheumatoid arthritis. Sjogren's sicca complex or linear scleroderma. patients with osteoarthritis who were age- and sex-matched to the group with the crest syndrome did not have anticentromere antibodies. autoantibodies found in other connective tissue diseases (anti-dna, anti-RNP, sjogren's syndrome antigen B (anti-SS-B) were not found in serums from patients with the crest syndrome. A case report illustrating the appearance of the anticentromere antibody at a time when Raynaud's phenomenon antedated the clinical diagnosis of crest syndrome is presented.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/6. Hereditary progressive mucinous histiocytosis.We describe hereditary progressive mucinous histiocytosis, a rare autosomal dominant non-Langerhans cell histiocytosis, in a mother and daughter. Both had similar, progressive eruptions of skin-colored to red-brown papules on the nose, hands, forearms, and thighs. light microscopy showed small collections of epithelioid histiocytes and telangiectatic vessels in the upper dermis of early lesions. In the mid dermis of early and well-developed lesions, nodular aggregates of tightly packed spindle-shaped cells were seen. Moderate to extensive mucin production was demonstrated in epithelioid histiocytes and spindle-shaped cells. Electron microscopy of spindle-shaped cells revealed many dendritic histiocytes with abundant lysosomal storage organelles such as myelin bodies and zebra bodies. immunohistochemistry showed expression of macrophage antigens (CD68; MS-1 high-molecular-weight protein) in epithelioid histiocytes and in some of the spindle-shaped cells. The histologic and immunohistochemical features of hereditary progressive mucinous histiocytosis most closely resemble solitary histiocytoma/cellular-type dermatofibroma.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/6. Idiopathic solitary neuroma of skin with unusual histologic changes.We report herein a case of idiopathic solitary neuroma with vascular proliferation and neurofibroma-like features. Clinically, the skin lesion was a 0.7 cm nodule without tenderness on the forearm. Histologically, there was vascular proliferation in the upper dermis, some neural structures among the proliferative vasculature in the mid dermis and large bundles of neural structures extending in various directions in the mid and deep dermis (resembling neurofibroma). Special stains, such as Masson's trichrome and Luxol fast blue, and immunohistochemical studies, including S-100 protein, neuron-specific enolase (NSE), vimentin, desmin, factor-VIII related antigen and epithelial membrane antigen (EMA), were added for this very rare case.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |