Chronic granulomatous disease: late onset of skin lesions only, in two siblings.
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Biomedical subjects
Publications and source records attributed to H Barriere.
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According to works of Penneys and Eaglstein on the management of pemphigus with gold compounds, the authors report one observation of chronic pemphigus, in which steroid therapy caused severe psychiatric disorders. The use of gold compounds permitted reduction of the dose of steroids and control of the pemphigus.
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Two characteristic cases encountered in young adults led the authors to present the hand foot and mouth syndrome. They report the characteristic distribution and vesicular appearance of the lesions. The course was benign. The viral origin of the disease was more or less easily confirmed by cell culture, inoculation in new born mice and demonstration of antibodies. Usually the virus was a Coxackie A 16. However in one of the authors cases, an Echo 11 was demonstrated. The apparent rareness of the disease may be explained by lack of recognition.
The authors report 5 cases whose main characteristics appeared very similar. Constantly, they found the same skin signs, urticaria without pruritus, recurring over a long period. The latter was accompanied by a very high E.S.R. and immuno-electrophoresis showed, in all cases, an increase in monoclonal IgB, permitting one to make the diagnosis of macroglobulinemia. In four cases out of five, this clinical picture was accompanied by bony pain associated with radiological signs of condensation. The symptoms were accompanied by prolonged fever and lymphadenopathy. After being well tolerated for a long period, the disease may become worse and lead to death. Thus this seems to be a true disease entity?
A case of Abrikossof tumor in a 33 year old patient presenting a progressive growth of a toe without associated pain or inflamation. There was an important hypertrophy of the affected toe with hyperkeratosis and pseudoelephantiasis. The histologic examination showed a massive infiltration of granular cells in the dermis. The exceptional localisation of the tumor in this case is emphasized.
The authors review critically a series of cases, followed up by the authors, of acrodynia and trophic ulcers occurring either in alcoholics (119 cases) or in diabetics (28 cases), who were also often alcoholic as well. The symptoms were similar in both groups. However, the poor nutrition was generally more market in the alcoholics. The trophic disorders were very similar, together with the sensory disorders. However, the deep sensitivity was less rarely involved in diabetics. Above all, the study of the rate of conduction of the motor nerve fibers showed axonal involvement in alcoholics and segmental demyelinisation in diabetics. The treatment was mainly dietary, together with proper hygiene and immobilisation of the foot.
This study of 38 cases observed over a period of 7 years emphasizes the constancy of rural contamination, whether professional or not. An enquiry carried out among veterinary surgeons in this region, showed the increasing frequency of the animal infection particularly in young oxen, hence the increased risk of kerions. The author recalls the clinical description of kerion of the scalp in children and sycosis barbae in adults, in which one may also encounter folliculitis of the limbs. The parasite isolated, included various strains of Trichophyton, e.g., Trichophyton Trimentagrophytes, Tr. verrucosum and, more rarely, Tr. magnini. On the other hand, bacteriological examination was usually negative, there were only two cases of secondary staphylococcal infection. The authors emphasize their concept of the disease which is probably an immune process. Finally, rather than the usual treatment, they emphasize the possibilities of social re-insertion; the disease should be recognised as an occupational disease.
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