[Generalized leuko-melanodermal cutaneous amyloidosis].
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Biomedical subjects
Publications and source records attributed to C Cywiner-Golenzer.
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Neoplastic thrombosis of the pulmonary artery is a rare and little known cause of pulmonary arterial hypertension. The clinical picture is one of acute respiratory failure and progressive right ventricular failure caused by pre-capillary pulmonary hypertension. In the living patient there is no way of distinguishing this condition from that of subacute cor pulmonale due to embolism, especially as the primary tumour is not always found either because it is too small or because it has already regressed by the time it has metastasised. The diagnosis usually rests on histological examination of the lungs, and two pathological types can be distinguished: carcinomatous lymphangitis with secondary invasion and thrombosis of the pulmonary arterioles on the one hand, and the neoplastic arterial emboli of a chorio-epithelioma on the other.
The authors report a case of diaphyseal bony tuberculosis, in which the diagnosis was not made for a long time as bony tuberculosis is considered exceptional and owing to the presence of a staphylococcus which oriented the diagnosis towards osteomyelitis. Bony tuberculosis certainly affects more easily immigrant patients, in whom one may see multifocal forms, but also affects those who, by their environment, might imagine they are protected, and it is not exceptional to see unifocal forms.
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The authors have observed 3 cases of pure scleroderma and one case of scleroderma in which tendinous or subcutaneous nodular structures were present. To the author's knowledge, such nodules have never been described previously in cases of scleroderma. Their site and their histology identifies them as "rheumatoid" nodules and at the same time provides other valuable information. Scleroderma is thus, together with rheumatoid polyarthritis and lupus erythematosus, one of the possible causes of nodule formation, and the authors put forward the hypothesis that these nodules might represent one of the first lesions of the Thibierge and Weissenbach syndrome.
In scleroderma, the scapular striated muscles may be affected showing rapid fatigability, and sometimes, muscle wasting with EMG changes. The smooth muscle changes, well known in the esophagus, where they lead to loss of peristalsis, may also be marked in other parts of the digestive tract. They lead to various symptoms, such as cramps, vomiting and diarrhea.
The authors studied 61 bone marrow biopsies carried out in cancerous patients, presumably suffering from a bone metastasis and before any treatment. They feel that quantitative and qualitative changes in the bone marrow may be considered to be an indirect diagnostic indication of metastatic spread.