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The authors study 86 cases of ischaemic problems affecting the upper limb associated with digital necrosis. They emphasise the wide range of aetiologies encountered and discuss the relationship between this syndrome and the main forms of arterial disease, principally inflammatory, scleroderma appearing to be the dominant though not sole aetiology. The aetiological results are also studied in relation to the extent of the necrosis and the sex of the patient. These data are compared with those found in the literature.

Cockett's syndrome (iliac compression syndrome) results from compression of the common iliac vein by the aortic bifurcation. The anatomical abnormality is frequent (15-20% of the population), but symptoms only appear when compression is pronounced. In a study of 16 patients, predominantly female, the following clinical presentations were observed: venous oedema of the left lower limb in 4 cases; symptoms suggesting iliac phlebitis in 3 cases; pulmonary embolism as first sign of peripheral venous thrombosis in 6 cases; and deterioration of angiodyplasia in 3 cases. In all patients, the diagnosis of Cockett's syndrome was confirmed by cavography. Surgical treatment, successfully performed in 13 patients, consists of uncrossing the artery and vein and placing a clip on the vena cava in case of pulmonary embolism

Thrombosis of the visceral veins is an extremely rare condition in cases of congenital and hereditary deficiency of antithrombin III, associated with recurring venous thorbosis of the limbs. The authors report such a case in a man of 40 years of age, who had this deficiency, associated with thrombosis of the portal and superior mesenteric veins and a portal cavernoma. They stress the frequent association of recurring peripheral vein thrombosis, portal vein thrombosis in adults, and thrombosis of the mesenteric vein, and the importance of systematic measurements of antithrombin III levels in these pathological conditions.

Retrospective analysis of the records of 86 patients with Takayasu's disease confirmed that the condition is more common in women, that it is frequently associated with a history of tuberculosis and that angiography is of considerable help in showing vascular lesions that are characteristic by their location (common carotid artery, post-vertebral subclavian artery) and by their diffusion to various areas. Histological examination of 20 surgical specimens confirmed the diagnostic value of sclerosis of the media and adventitia (associated or not with inflammatory lesions) and therefore radically different from the lesions of the intima observed in Horton's disease. Diagnostic criteria based on clinical, radiological and histological data are proposed.

The use of heparin by continuous infusion from a electric pump gives very good immediate results in the treatment of venous thromboses. In 58 patients, there was a constant improvement in clinical signs and recurrence of the venous thrombosis was exceptional (2%), pulmonary embolism rare (3.5%) and well tolerated. Constant flow and the surveillance of treatment led to the method ensuring stable blood heparin levels. Mid-term results (8 months) in 43 patients appeared to be less favourable, the prevalence of venous sequelae reaching 50% for iliac phlebitis, 28% for sural phlebitis. The respective advantages and disadvantages of heparin and fibrinolytic agents are discussed.

A prospective study of 100 consecutive patients hospitalised with a Raynaud phenomenone is presented. In 82 patients, a precise etiology was pound, most often a collagen disease (50 cases) and amongst this group generalised scleroderma was the most commun (n = 37). Other etiologies were very divers: inflamatory artertis, atheroma, local mecanical causes, emboligenic. In 18 patients, no etiology was found: the age, the sex, the context and the benign cours in this patients was such that a diagnosis of idiopathic Raynaud's phenomenone. This results lead to the suggestion of a method for the investigation of Raynaud's phenomenone. The consequences are therapeutic, the etiological context foring the base of the treatment prescribe.

Microangiopathy is an essential clinicopathological feature of systemic scleroderma, comprising capillary thinning, thickening of the basement membrane and abnormalities of the endothelium which has an abnormally high uptake of radioactive thymidine. The identification of a circulating cytotoxic factor for endothelial cells suggests that the microangiopathy may play a role in the physiopathogenesis of the disease. In practice, microangiopathy can be fully assessed by capillaroscopy in patients with systemic scleroderma.

Thirty one patients with generalised scleroderma underwent labial biopsy. Sixteen showed a nodular infiltration of the accessory salivary glands indicative of Sjögren's syndrome. In 21, intralobular fibrosis was present. More common in those forms with extensive cutaneous involvement, collagenous fibrosis would appear to be a direct manifestation of scleroderma. The frequency of these abnormalities poses the problem of immunological competence with respect to lymphocytic labial infiltrates in generalised scleroderma.

Forty one patients with systemic sclerosis were studied after separation into three groups according to Barnett's classification. A multi-dimensional statistical analysis eight serum proteins revealed a difference between control patients and patients with type I and type II scleroderma. Type I scleroderma was characterised by a rise in alpha 2 macroglobulin and in the C4 fraction of complement, whilst in type II scleroderma all the proteins studied were raised, with the exception of CO complement, which was normal, and transferrin which was markedly decreased.

Both of these arterial diseases may involve the aorta and the major arterial trunks. Two cases of subclavian involvement are used to contrast them from a histopathological standpoint. In giant cell arteritis, the lesions affect above all the internal elastic layer and the inner part of the media, destroyed by an inflammatory infiltrate with giant cells. In Takayasu's disease, the lesions involve the adventitia, the site of fibrosis and of inflammatory islets with the vasa vasorum at the centres. Involvement of the media is predominantly in its outer part, the internal elastic layer being intact. A histopathological definition of these arterial diseases may be envisaged on the basis of these facts.

To better understand the putative association between insulin and atherosclerosis many experimental studies have explored the metabolic effects of insulin upon the arterial intima-media (which is essentially made up of smooth muscle cells). This tissue appears to be insulin-sensitive in vivo but not in vitro, suggesting that the actions of insulin on arterial metabolism are probably indirect. Yet, cultured arterial smooth muscle cells do increase their anabolism when physiological concentrations of insulin (10-250 microU/ml) are added to the medium. To account for these discrepancies we have proposed a haemodynamic explanation : hydrostatic forces present in vivo but absent in vitro, would be necessary for insulin to reach the cells of the media and act upon them. We demonstrated such an effect using an isolated rat aorta perfused under variable pressure. But only highly unphysiological concentrations of insulin (0.1 mU/ml) could stimulate the metabolism of intima-media significantly. The insulin-sensitivity of the target tissue for atherosclerosis remains an enigma. We speculate that the variable phenotype of arterial smooth muscle cells (either contractile or synthetic) might be an interesting clue to this problem.

In patients with arteriosclerosis obliterans of the lower limbs immediate (operative risk) and long term prognosis depend on the diffusion of the atheroma to other arterial beds (coronary and carotid). Basic investigations were completed by doppler ultrasonography of the cervical arteries, exercise electrocardiogram and lateral aortogram. Doppler ultrasonography revealed significant degrees of stenosis of the cervical arteries in 67 out of 402 patients (16.6 p. 100). The stenosis were totally latent clinically in 13 patients. Valid results were obtained in 72 p. 100 of 140 after an exercise electrocardiogram. It was positive in 19 p. 100 of patients without clinical or electric sign of coronary heart disease. Lateral aortogram in 125 patients showed atheromatous lesions in 39 cases (31 p.cent). These were often multiple and involved the coeliac and superior mesenteric arteries in 13 cases. These investigations are of great importance in making the therapeutic decision, indications related with risk and resulting priorities. Alarming coronary or carotid lesions may indeed require treatment before arterial disease of the lower limbs.

The authors report six new cases of necrosis of the fingers occurring during the course of a malignant condition. None of the patient was suffering from any other disease which could explain the digital necrosis. They discuss the mechanism of the relationship between the two conditions : blood hyperviscosity, thrombocytosis, polycythaemia, cryoglobulin and the production of immune complexes. The particular role of bleomycin is mentioned. The possibility of a true paraneoplastic syndrome is suggested.

This paper describes a case of disseminated gonococcal infection, a rare disease in France. A 41 year-old woman was hospitalised with acute polyarthritis and characteristics skin lesions. Jaundice and liver function abnormalities are difficult to interpret due to a preexisting alcoholic cirrhosis. The possibility of an endocarditis is raised because of a systolic murmur heard at the base of the heart. Bacteriological identification of N. Gonorrhoeae is carried out in blood culture; it has also been recovered by scrapings of a cutaneous bullae by staining only. Therapy was instituted by daily intravenous penicillin G sodium 50 000 000 u. and intramuscular gentamicin 160 mg for 45 days. There resulted good clinical and bacteriological response. The elements of clinical and bacteriological diagnosis, as well as the therapy are discussed.

The results of nailfold capillary microscopy were compared in 107 lumberers who had been using a chain saw for more than 3 years and 115 manual workers who had never used vibrating tools. The prevalence of Raynaud's phenomenon was 61.7% in lumberers and 5.2% in the control group. The mean time of exposure to risk before the condition developed was 7.86 years. The syndrome was usually mild, but the patients had an abnormally high incidence of accidents at work, including wounds and cut off fingers. The number of capillary loops was significantly reduced. The lumberers affected showed an abnormal spasm of the digital artery in response to cold; the predictive value of this test was 88%. It is suggested that nailfold capillary microscopy should be systematically used in industrial medicine for monitoring workers using a vibrating tool.

Two patients are presented as new cases of secondary syphilitic hepatitis. The closely resemble descriptions in the literature with the same clinical and laboratory "profile" and the same course spectaculary influenced by anti-syphilitic penicillin therapy. The presence of treponemes in the liver, which would definitely confirm the diagnosis, was not demonstrated. This is a particularly important diagnosis, which should not be missed since it leads to treatment of fundamental importance to the patient's future.

The case of an 18 year old Algerian girl with acrocyanosis of the four extremities since childhood and presenting a with 6 months history of oedema of the left hand resulting in severe functional handicap, is reported. Clinically, the oedema suggested a lymphatic origin. However, its localised, strictly unilateral distribution, the normality of vascular investigations, the absence of pain and regression of the oedema during the period of hospitalisation suggested autoaggression. The discovery of tourniquet marks on the skin of the thigh confirmed the diagnosis of Charcot's blue oedema. The patient has a hysterical personality, was of higher than normal intelligence but immature, and could only express herself through her functional handicap, a cry for affection in a rigid family environment only concerned by her scholastic achievements. To our knowledge, there have been 27 other reports of this condition since Charcot's first description in 1890. The majority concerned young intelligent women. Diagnosis is difficult and can only be confirmed by the finding of tourniquet marks on the skin. Psychotherapy is often useful as an underlying neurotic or psychotic personality is commonly found.

Takayasu's disease is a syndrome of inflammatory arteritis involving the aorta and its branches due to varying etiologies. The diagnosis is usually made after clinical, radiological and biological investigations, but the pathognomonic sign of the disease is inflammatory sclerosis of the adventitial media. There appear to be five prognostic factors: hypertension, arterial aneurysm, aortic regurgitation, coronary insufficiency, and retinopathy.

We studied the glucose oxidation to CO2 and the incorporation of glucose into lipids by thoracic aorta from streptozotocin-diabetic rats in two circumstances: 9 minutes exposure of the isolated aorta to insulin, perfused in situ; and one-week treatment with either low--4 units (U)--or high--12 U--daily doses of insulin. Diabetes inhibited the glucose metabolism of media and adventitia. Perfusion with insulin did not influence the depressed metabolism of media but increased that of adventitia. Four units insulin treatment normalized the synthesis of glucose-derived lipids by the media, whereas 12-U treatment brought it to levels significantly higher (+23%) than those found in nondiabetic rats. We conclude that: (a) a haemodynamic explanation cannot account for the in vitro insulin resistance of media found in diabetes; (b) insulin treatment exerts a dose-dependent lipogenic effect upon the media, which might favour an atherogenic role for therapeutic hyperinsulinaemia in insulin-dependent diabetics.

A study of salivary immunoglobulins revealed the presence of IgM in 11 out of 17 patients suffering from progressive systemic sclerosis. The presence of IgM was frequently accompanied by an increase in IgA and less often by IgG. Immunofluorescence examination of labial biopsies showed comparable modifications in the immunocyte populations; the presence of IgM cells, sometimes in large numbers, and an increase in IgA and IgG cells. All patients with a nodular lymphoplasmocyte infiltration of the minor salivary gland of the lip have salivary IgM. The presence of IgM in the saliva is a diagnostic criteria of Sjögren's syndrome. The absence of a correlation between immunoglobulin concentrations in the saliva and the serum and correlation between the salivary IgM concentration and the number of IgM immunocytes, demonstrate that the presence of IgM is related to the glandular synthesis of this enzyme.

Biopsies of the lips were performed on 27 patients (7 male and 20 female; mean age, 58 years) with diffuse scleroderma and on 10 healthy subjects serving as controls. The degree of intralobular sclerosis in labial salivary glands was measured on tissue sections by an automated morphometric analysis technique. Collagenous compared with controls. It was independent from the type of cell infiltrating the gland and from the evolution potential of the disease, but it correlated with its duration and seemed to be specific to scleroderma.

Thrombolysis occurred in five out of eleven patients treated with intra-arterial urokinase for recent raterial obliteration. These positive results were accompanied by perfect tolerance and absence of systemic fibrinolysis. In some cases, therefore, surgical revascularization can be associated with fibrinolytic treatment.