[Werner's syndrome with early onset with hyperandrogenism caused by ovarian hyperthecosis, acanthosis nigricans and peripheral neuropathy].
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Biomedical subjects
Publications and source records attributed to J Civatte.
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We report three cases of epithelio-lympho-histiocytic tumour with very original histological features. The patients were young adults presenting with a nodular lesion on the face which had been present for several years. No recurrence was observed after surgical excision. Histologically, the dermal lesion consisted of epithelial pouches edged with a few layers of basal-like cells and filled with lymphocytes and large clear cells. Relations were found between these lobules and hair follicles, and rudiments of piliary differentiation were noted. The stroma was dense and infiltrated by small lymphocytes and large clear cells. An immunohistochemical study showed marking of the large clear cells by the S 100 protein, which suggested that they were Langerhans cells. The lymphocytes were recognized by the common panleucocyte antibody. The KL 1 antibody marked a few isolated cells within the lobules, but not the basal-like cells. These cases seemed to be similar to the 7 cases reported in the literature by Santa-Cruz and Barr who used the term lymphoepithelial tumour of the skin. We felt justified in putting the stress on the histiocytic component of this tumour and calling it epithelio-lympho-histiocytic tumour. We agree with these authors that this is a tumour of the appendages of the skin and in particular the hair.
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Two cases of chronic urticaria associated with macroglobulinemia are reported, and the characteristics of 13 other cases are reviewed. This entity was described by Schnitzler in 1974 and has the following characteristics: chronic nonpruritic urticaria with leukocytoclastic vasculitis, bone pains with hyperostosis, intermittent fever, and a monoclonal IgM gammopathy. Liver, lymph node, and spleen enlargement may occur. Criteria for the diagnosis of Waldenström's disease are lacking (IgM level less than 10 gm/L, no overt lymphoid proliferation in bone marrow). Other immunologic findings (complement, C1 inhibitor, cryoglobulin, rheumatoid factor, antinuclear antibodies) are negative or normal. Evolution is long-term with a long follow-up period. In one case a lymphoplasmocytic lymphoma developed. No adequate treatment has yet been found. Pathogenesis is unclear but seems to be caused by skin deposits of the IgM paraprotein, as attested to by the direct cutaneous immunofluorescent findings in some cases.
A 58-year-old immunocompetent man presented with a 9-month history of several disseminated subcutaneous ulcerative nodules, fever and weight loss. Histopathological changes were not specific. X-ray of the chest disclosed a large right hilar density highly suggestive of a lung carcinoma. All these manifestations eventually proved to be caused by Mycobacterium tuberculosis, which grew from sputum and skin. We report this case because of its striking clinical features.
An overview is given on pemphigus diseases induced by certain drugs. Well known inducers are D-penicillamine, and pyritinol-chlorhydrate. Besides that in some rather rare cases other drugs were connected to pemphigus induction: rifampicin, INH, etambutol, practolol, propranolol, phenylbutazone, aurothiomalate, ibuprofen, heroin, penicillin, ampicillin, captopril, thiopromine, alpha-mercaptopropionylglycine and piroxicam.