1/54. Systemic sclerosis revealing T-cell lymphoma.We describe a case of systemic sclerosis (SSc) occurring together with malignant lymphoma. A 43-year-old man, who had noticed sclerodactyly 1 month before consultation, was admitted for progressive skin sclerosis on his forearms and chest. SSc was diagnosed. Immediately after admission, skin sclerosis rapidly extended to the neck and trunk, and subcutaneous tumors developed on the neck, chest and back. skin sclerosis was prominent at the sites where subcutaneous tumors were present. The tumors were diagnosed as non-Hodgkin's lymphoma of T-cell phenotype derived from soft tissue. Following 4 cycles of chemotherapy, he had complete remission and the skin sclerosis remarkably improved. It is possible that cytokines produced by T-cell lymphoma cells were responsible for the development of skin sclerosis in this case.- - - - - - - - - - ranking = 1keywords = chest (Clic here for more details about this article) |
2/54. Evidence of cell-mediated cardiac myocyte injury involved in the heart failure of a patient with progressive systemic sclerosis.A 54-year-old woman with progressive systemic sclerosis (PSS) was admitted to hospital because of dyspnea and chest pain. Echocardiogram revealed diffuse hypokinesis of the left ventricle (ejection fraction 24%). methylprednisolone, heparin, and diuretics were administered, without benefit. anemia, thrombocytopenia, and renal dysfunction rapidly progressed, and she died of heart failure on the 14th hospital day. Immunohistochemical study of the myocardial tissue showed mild to moderate cell infiltration, mainly consisting of natural killer (NK) cells, macrophages, cytotoxic T lymphocytes (CTLs), and T helper cells. perforin, a cytolytic factor, was expressed in the infiltrating CTLs and NK cells, indicating that these cells were activated killer cells. Furthermore, human leukocyte antigen classes I and II, intercellular adhesion molecule-1, as well as costimulatory molecules B7-1, B7-2, and CD40, all of which are known not to be expressed in cardiac myocytes under normal conditions, were moderately to strongly expressed in cardiac myocytes. There was no detectable level of enterovirus genomes in the polymerase chain reaction products from the myocardial tissue of this patient. These findings strongly suggest that the infiltrating killer cells recognized cardiac myocytes as target cells and directly damaged them by releasing perforin. Enhanced expression of these antigens may have played an important role in the activation and cytotoxicity of the infiltrating killer cells. Absence of enterovirus genomes in the myocardial tissue may suggest that this autoimmune process is primarily induced by PSS.- - - - - - - - - - ranking = 0.5keywords = chest (Clic here for more details about this article) |
3/54. Raynaud's phenomenon possibly induced by a compund drug of tegafur and uracil.We describe a 50-year-old woman who noted acral hyperpigmentation, sclerodactyly and Raynaud's phenomenon with 1:160 of antinuclear antibody titer after treatment with a compound drug with tegafur and uracil. Histological findings of the finger and palm included hyperkeratosis, vacuolar degeneration of basal cells, thickened collagen fibers in the dermis, and dilatation of capillary vessels, perivascular mononuclear cell infiltration and melanophages in the upper dermis. IgG, IgA, IgM, C3 and C1q were not deposited in the skin by direct immunofluorescence study. After cessation of the drug, Raynaud's phenomenon and hyperpigmentation disappeared within 1 month and 4 months, respectively, and antinuclear antibody turned negative within 4 months. These observations suggest that tegafur may have caused not only hyperpigmentation in the palms and soles, but also sclerodactyly and Raynaud's phenomenon in the present case.- - - - - - - - - - ranking = 0.27008366467545keywords = upper (Clic here for more details about this article) |
4/54. barium impaction as a complication of gastrointestinal scleroderma.Two patients with scleroderma of the bowel experienced life-threatening barium impaction after upper intestinal x-ray studies. Although the frequency of this complication is unknown, the difficulty of managing it when it occurs makes prevention imperative. X-ray studies should be performed only after careful consideration of the risks and benefits. When x-ray studies are performed, the patient should be vigorously purged soon thereafter, and a follow-up roentgenogram should be obtained to confirm adequate removal of barium.- - - - - - - - - - ranking = 0.27008366467545keywords = upper (Clic here for more details about this article) |
5/54. Digital necrosis in a woman over eighty.Chronic vascular disorders of the upper limbs are not frequently encountered in women; in addition, advanced age of onset tends to suggest the presence of common diseases such as arteriosclerosis or microembolization. Nonetheless, diagnosis is not always straightforward and in some cases both-laboratory and instrumental findings can do no more than arouse a diagnostic suspicion. We present a case of acro-localized disorder, which proved difficult to diagnose and which we believe may be attributed to a slowly progressing form of scleroderma of the extremities which, in our case, determined positivity of anticentromere autoantibodies.- - - - - - - - - - ranking = 0.27008366467545keywords = upper (Clic here for more details about this article) |
6/54. Secondary amyloidosis in progressive systemic sclerosis.Progressive systemic sclerosis (PSS) is a connective tissue disease that may affect many organs, including the kidneys. It is quite rare to see secondary amyloidosis due to PSS. We present a patient with a 9-year history of PSS who developed nephrotic syndrome, and whose renal biopsy was compatible with secondary amyloidosis. He died from massive upper gastrointestinal bleeding caused by oesophageal telangiectasia.- - - - - - - - - - ranking = 0.27008366467545keywords = upper (Clic here for more details about this article) |
7/54. Keloidal scleroderma.A 53-year-old woman with systemic sclerosis developed numerous cutaneous nodules, resembling keloids, on her anterior chest wall. The nodules failed to respond to therapy with topical steroid, calcipotriol, or extended photochemotherapy. This is a rare, disfiguring variant of scleroderma, unresponsive to treatment, and poorly understood.- - - - - - - - - - ranking = 0.5keywords = chest (Clic here for more details about this article) |
8/54. Idiopathic myelofibrosis associated with classic polyarteritis nodosa.A woman with scleroderma and classic polyarteritis nodosa (PAN) who developed idiopathic myelofibrosis (IM) is reported. The patient presented with a one-year history of weakness, polyarthritis, Raynaud phenomenon, dry cough, and epigastralgia. The diagnosis of scleroderma with visceral involvement was made and treatment with prednisone subsequently started, with good clinical response. Six years later, fever, weight loss, livedo reticularis, and dysesthesias developed. Electromyographic studies were consistent with sensory neuropathy and a sural nerve biopsy yielded the diagnosis of PAN. The patient received cyclophosphamide plus prednisone with a favorable response, but 11 years later she was admitted because of weakness, constitutional symptoms, and abdominal pain due to spleen infarcts. Marked anemia, with aniso-poikilocytosis, tear-drop cells, immature myeloid precursors in the peripheral blood, and an increased serum LDH, was observed and the diagnosis of IM established by bone marrow biopsy. This case represents a new association between IM and an autoimmune disease and supports the hypothesis of an immune basis of IM in some patients.- - - - - - - - - - ranking = 0.87000354968688keywords = abdominal pain (Clic here for more details about this article) |
9/54. Nodular scleroderma in systemic sclerosis under D-penicillamine therapy.A case of systemic sclerosis (SS) which developed keloidal lesions (nodular scleroderma) on the chest during D-penicillamine (DPC) therapy is reported. The 36-year-old woman showed rapidly progressing skin sclerosis with lung and esophageal involvement, and DPC was started at the age of 38. skin sclerosis as a whole had improved to some extent, when keloidal nodules developed on the upper chest at the age of 44. Since there were no other findings suggestive of adverse reactions caused by DPC, we speculate that activation of the fibroblasts in these lesions occurred despite the suppressive effect of DPC.- - - - - - - - - - ranking = 1.2700836646754keywords = chest, upper (Clic here for more details about this article) |
10/54. Silica and trichloroethylene-induced progressive systemic sclerosis.Several environmental factors and chemicals have been described as being able to induce systemic scleroderma and scleroderma-like diseases. The present work reports 2 male patients with progressive systemic sclerosis and pulmonary silicosis. Both patients had occupational histories of exposure to silica and one of them of handling trichloroethylene as a degreasing agent. The clinical and analytical findings could not be distinguished from those present in idiopathic systemic scleroderma with the exception of interstitial images with calcified hilar lymph nodes in the chest X-ray suggestive of pulmonary silicosis.- - - - - - - - - - ranking = 0.5keywords = chest (Clic here for more details about this article) |
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