Absence of human herpesvirus-8 DNA in angiosarcoma.
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Biomedical subjects
Publications and source records attributed to I Umbert.
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A patient with necrobiotic xanthogranuloma (NXG) and paraproteinaemia, who was followed-up for several years, and treated with low-dose chlorambucil, died as a result of a respiratory illness. The significant findings at autopsy were a xanthogranuloma of the spleen and giant cell myocarditis. The myocardial lesions were composed of macrophages, giant cells and lymphocytes. This finding is important because four of five known autopsied patients with NXG have had giant cell myocardial disease, and an effort at antemortem diagnosis should be made.
The L-tryptophan eosinophilic myalgia syndrome (EMS) clinically has some similarities with idiopathic eosinophilic fasciitis (EF). In order to study the pathology of both syndromes, we analyzed 21 biopsies of patients with EMS and 8 with idiopathic EF. In both diseases there is dermal and fascial mucin and dermal edema, but this was more common in EMS. EMS is also characterized by dilated lymphatics, dermal and septal sclerosis and macrophage-rich inflammation. Neural inflammation was seen in 4 of the cases with EMS and in none with idiopathic EF. In both syndromes, there are many histopathological similarities. The differences may be due to sampling and to sample size. The nerve lesions of EMS may result from the nature of lymphocyte-macrophage inflammation, or the effect of the eosinophil neurotoxin and may not be a primary event.
We report a 63-year-old man with violaceous nummular patches on the trunk. Histopathologic studies were consistent with a diagnosis of malignant angioendotheliomatosis or angiotropic lymphoma. Immunohistochemical study of skin was positive for UCHL-1 antigen and leukocyte common antigen and negative for L-26, Ulex europaeus lectin I, vimentin, cytokeratin, and epithelial membrane antigen. Ultrastructural study ruled out an endothelial origin of the neoplastic cells. These data confirmed the diagnosis of malignant proliferative angioendotheliomatosis. Five years before, a soft tissue lymphoma had been excised. This is an unusual case of malignant angioendotheliomatosis for the following two reasons: (1) a previous association with a soft tissue lymphoma and (2) the rarely described T immunophenotype of neoplastic lymphoid cells.
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We reviewed 15 cases of reticulohistiocytoma cutis (RHC). Three cases were of solitary lesions. Four cases were associated with a destructive arthritis but no other systemic features. One additional patient had a destructive arthritis and lesions of reticulohistiocytoma in synovium, larynx, and mucosa. Three patients had associated xanthelasma. Two cases were associated with internal malignancy (metastatic malignant melanoma, adenocarcinoma of the bowel). Two cases were sequentially associated with systemic vasculitis (Wegener's granulomatosis, periarteritis nodosa). This occurrence of systemic vasculitis in RHC patients is unique. RHC may have variable cutaneous and systemic manifestations and associations with malignancy and immunoinflammatory diseases, including vasculitis.
A case of multiple glomus tumour is described in a 33 year old man. The clinical and histopathological features are discussed, with the review of the literature. We discuss the types of glomus tumour and its differential features.
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