[Myeloblastic invasion of bone marrow in 15 days in a patient with lymphopathy].
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Biomedical subjects
Publications and source records attributed to D Colomb.
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A dermal duct tumor which clinically resembled an intradermal nevus and developed on the lower back of a 59-year-old woman is described. The diagnosis could only be reached by microscopic examination. The dermal duct tumor appears to originate from cells with differentiation towards the intradermal portion of the eccrine sweat duct.
A case of diffuse plane xanthomatosis assoicated with systemic amyloidosis and multiple myeloma at its outset is reported. Plane xanthomatosis is certainly an autonomous entity in comparison with systemic amyloidosis, for there are no amyloid deposits in xanthoma. The patient had lambda type IgG paraproteinemia, with Bence-Jones proteinuria. Lipid tests were considered as normolipemic though some levels recall a type IV hyperlipoproteinaemia. A review of literature about the association "xanthomatosis-multiple myeloma" was made, after the important work of Bazex, Dupré and Mrs. Christol-Jalby. It allows us to distinguish two differnet descriptions: 1. When there is hyperlipoproteinemia, all clinical types of xanthomas may exist; multiple myeloma is generally typical (but sometimes not very progressive). 2. When there is normolipidemia, the main clinical type is diffuse plane xanthomatosis; multiple myeloma is atypical and often only a monoclonal gammapathy is found. 3. However in both cases, the outstanding clinical type is diffuse plane xanthomatosis: whether normo- or hyperlipemic, this therefore indicates a possible underlying disease, and above all a multiple myeloma.
The authors report five new cases associating basal cell epithelioma and multiple Pinkus fibro-epithelial tumors on the spine after radiation damage to the skin. One of them had previously published three similar observations: so his experience refers to eight cases. The authors recall similar cases of the literature, they discuss the relations between basal cell epitheliomas and Pinkus premalignant fibro-epithelial tumors of the skin. Histologically they saw several aspects of transition between the two types of tumors. They study the patterns of cancers found after radiation therapy, influence of type of radiations, dose, age, and intervals since X-rays. As regards such cases, the necessity of systematically searching for previous radiations by X-rays in the affected zone is emphasized.
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Among the systemic manifestations of Behçet's syndrome, venous and arterial involvements are discussed. Venous thrombosis is a common manifestation and may be considered as a cardinal sign in the diagnosis. Venous and arterial thrombotic complications may be regarded as agents of morbidity and mortality. The pathogenesis of the occlusive process is still some what obscure, although vasculitis and impaired local vascular fibrinolytic activity play a part in it. Therapeutic measures using fibrinolytic enhancing agents may prevent and control the thrombotic complications of the disease.
The fragility of epidermal cells in Hailey-Hailey's disease end even more in Darier's disease seems to favour the development of viral infections. The authors present six cases of herpes virus infections in three siblings with an intermediate form between Darier's disease and Hailey-Hailey's disease, both having a tendency to recur. One of the flares demonstrated signs of Kaposi and Juliusberg's pustulosis vacciniformis which is often found in the literature as a complication of Darier's disease. The authors discuss the frequency of the recurrences, which could be due to different viruses. Herpetic virus is often found, less frequently vaccinial virus, but sometimes also Coxsackie A 16 virus.
The authors demonstrate, with the help of electron microscopy, the location of the polymorphonuclear neutrophils and of the keratinocytes in the Kogöj's pustule. Unlike to the classical ideas, the polymorphonuclear neutrophils are not located in the epidermal cells but in the intercellular spaces. The lesions of keratinocytes, the content of the spaces and the histogenesis have been studied. The results of this study are compared to thoses formed in the literature.
The authors have studied clinically and histologically two cases of " erythrosis interfollicularis colli de Leder ". It's a frequent affection, principally between 40 and 50 years of age but not very known because the patients rarely consult for it. Clinically they insist on the respect of a triangulary and lozengical median zone, and on the sight in "plucked chicken skin"; histologically on the dilatation of sebaceous glands, the frequency of a folliculary corneous plug and the elastic tissue alterations. On the etiological plan, the part of the age, the sun exposure, and in their two cases on the possible photosensibilisative part of the scents.
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On the basis of three cases, the authors describe the main changes seen in the nails in Bourneville tuberose sclerosis, not only in fingers with a Koenen tumour but also in association with simple cuticular hyperkeratoses and in fingers with no apparent lesion of the matriceal region. They feel that the description of these ungual lesions should take its place alongside the major cutaneous signs of this disorder.
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The authors report 4 cases of multiple tumors of the lumbo-sacral region with, on histology, a mixture of basal cell carcinomas and fibro-epithelial Pinkus tumours. They were struck by the fact that three of these four patients had received, in this region, radiotherapy numerous years previously. They discuss the role of this radiotherapy in the onset of the disease and the relative importance of this factor and of constitutional factors (Hamartomas). They recall the characteristics of multiple basal cell carcinomas and of Pinkus fibro-epithelial tumours, together with the close relationship between these two types of tumour.
The possibility of cutaneous manifestations in Down's syndromes not a surprise because we known that this affection affects the three embryonic parts. This cutaneous disorder is described by many authors but it is variable and not specific. The authors had occasion to see four cases of alopecia areata in mongolian children. They do an analytic study of variable manifestations. They insist on frequency of hyperkeratotic lesions, alopecia areata, alterations of vasomotricity and elastic dystrophies. In the group of chromosomic aberrations, down's syndrome is alone where the cutaneous manifestations are studied because the patients have a good survival.