[Raynaud's phenomenon and blood viscosity]. / Phénomène de Raynaud et viscosité sanguine.

Raynaud's phenomenon is mainly linked with cold provoked vasomotor perturbations, but also with rheological alterations since blood viscosity is enhanced by lowering temperature. Several methods are available for studying distal vascularization: peri-ungual capillaroscopy, digital plethysmography and laser-Doppler. Digital arteriography must be reserved to serious ischemia regarding the general anesthesia needed to avoid spasm. All these methods explore especially the vessel wall. Conservely, blood viscosity which has been developed for 25 years investigates the content of the vessel. Since 1965, numerous hemorheological studies pointed out the rheological disorders, especially those concerning plasma and blood viscosity. The most usual viscometry abnormalities revealed erythrocyte hyperaggregation, red cell hypodeformability, blood and plasmatic hyperviscosity. In a comparative study, 46 patients with Raynaud's phenomenon were studied: we performed peri-ungual capillaroscopy, plethysmography and viscosity measurements. The results demonstrated a link between capillaroscopy and thixotropy. Both investigations are never normal at the same time in connectivites and never abnormal at the same time in Raynaud's disease (primary Raynaud's phenomenon). In conclusion hemorheological studies showed nearly normal rheological parameters in Raynaud's disease, but abnormal rheological parameters in secondary Raynaud's phenomenon.

Capillary microscopy during eosinophilic fasciitis in 15 patients: distinction from systemic scleroderma.

PURPOSE: Eosinophilic fasciitis (EF) is a newly recognized syndrome that bears much discussion in regard to its distinction from progressive systemic sclerosis (PSS). In vivo microscopic examination of the nailbed capillaries has elicited the description of a characteristic vascular pattern seen in PSS dermatomyositis, and mixed connective tissue disease. To clarify the capillaroscopic aspects of this syndrome and to seek criteria distinguishing it from PSS, we performed nailbed capillary microscopy in 15 patients with EF and compared the results of this examination with those in 98 patients with PSS and those in 75 normal control subjects. PATIENTS AND METHODS: The diagnosis of EF was made in 15 patients aged 25 to 69 years (average 43 years) who had an acute course, with painful edema and subcutaneous sclerotic induration sparing the extremities. There was a peripheral hypereosinophilia in all 15 patients. Twelve underwent muscle or deep cutaneous biopsy, including the fascia. Nine of these had fascial thickening, and an inflammatory cell infiltrate was observed in eight. The diagnosis of PSS was made in 98 patients, according to the usual criteria. Seventy-five normal control subjects were examined. All the capillaroscopic examinations were performed by one observer. RESULTS: None of the patients in the EF group had a scleroderma-like capillaroscopic pattern. Thirteen had normal results of capillary microscopy. Two had a nonspecific organic microangiopathic picture. In the group of 98 patients with PSS, 89 had numerous megacapillaries (p less than 0.001), seven had a nonspecific organic microangiopathic pattern, and two had normal findings (p less than 0.001). In the whole group of 75 control subjects, the features were normal. CONCLUSION: Our results show a clear distinction between the results of capillary microscopy in cases of EF and PSS. The normal pattern in EF seems to be another argument for its differentiation from PSS.

[Nailfold capillaroscopy. Comparison of 100 subjects over 65 years of age and of 100 young adults]. / Capillaroscopie periungueale. Comparaison de 100 sujets âgés de plus de 65 ans et de 100 adultes jeunes.

Nailfold capillary microscopy patterns in 100 patients aged more than 65 years and free from inflammatory diseases were compared to those of 100 young healthy adults. A higher prevalence of arteriovenous sludge (36% vs 7%, p less than 10(-6)), increase in capillary loop length (12% vs. 0%, p less than 10(-3)) and especially prominent subpapillary plexus (63% vs 12%, p less than 10(-9)) was found in the geriatric group. Such capillary patterns cannot be considered as abnormal in patients aged more than 65 years. Enlargement of capillary loops and loss of capillaries were never encountered. Bushy capillary formations and hemorrhages were very uncommon in both groups.

[Diabetic microangiopathy. Role of capillaroscopy]. / Microangiopathie diabétique. Place de la capillaroscopie.

In order to evaluate the significance of ungual and conjunctival microvascular abnormalities observed in diabetics prior to retinopathy, nailfold and conjunctival capillaroscopy was performed in 20 controls and 40 insulin-dependent diabetics of the same age. The diabetics were divided into 4 groups according to their state of retinopathy: absent, incipient, non-proliferative and proliferative. No difference was found between controls and diabetics and between groups of diabetics in the frequency of conjunctiva microaneurysms and specific nailfold microangiopathy, nor even in that of the so-called characteristic "fish shoal" image. The percentage of abnormalities detected was concordant with the results of previous studies in diabetics (12.5 per cent of non-specific organic microangiopathy), but it was 2 to 4 times higher than the frequency usually found in non-diabetic controls. This discrepancy could be due to the method used, since in contrast with earlier studies the operator did not know whether the subject was diabetic or not and was unaware of the patient's retina state. In the absence of other blind and prospective studies, capillaroscopic examination for diabetic microangiopathy should be reserved strictly to clinical research.

[Cutaneous microcirculation in infectious endocarditis]. / La microcirculation cutanée dans les endocardites infectieuses.

Nailfold capillary microscopy was used to study the microcirculation patterns in 26 adult patients with infective endocarditis. Abnormal patterns were found in 13 patients (50%). Enlargement of capillary loops was never observed. Significant correlations were found between the number of capillary abnormalities and both systemic involvement (cutaneous vasculitis, arthritis, splenomegaly and/or glomerulonephritis) and immunological disturbances (circulating immune complexes, rheumatoid factor and/or hypocomplementemia) (p = 0.02 and 0.003, respectively). Capillary abnormalities were significantly reduced in 14 patients studied 4 to 48 months after endocarditis was cured. However, due to the lack of specificity, nailfold capillary microscopy cannot be regarded as a useful tool for the diagnosis of infective endocarditis. Connective tissue disorders are not the sole diagnosis to be considered in patients with abnormal nailfold capillary microcirculation patterns.

[Ungueal capillaroscopy in fasciitis with eosinophilia: a distinctive feature of systemic scleroderma. Apropos of 15 cases]. / Capillaroscopie unguéale au cours de la fasciite avec éosinophilie: un élément distinctif d'avec la sclérodermie systémique. A propos de 15 cas.

Eosinophilic fasciitis (EF) is a recently described disease whose distinction from progressive systemic sclerosis (PSS) is still being discussed. PSS has a characteristic microcirculation pattern. We performed nailfold microscopy on 15 patients with EF and compared the results to those of 98 PSS patients and 75 normal control subjects. EF patients have a normal microcirculation pattern (13/15) or discrete, non-specific anomalies: none had the typical capillary pattern associated with PSS and associated diseases. The findings of this study justify making a distinction between EF and PSS and demonstrate that nail fold microscopy can be a useful tool for an early differential diagnosis between these two disorders.

[Lupus vasculitis]. / Vascularites lupiques.

Anatomical studies have demonstrated the high incidence of vasculitis in SLE, the appearances of which are variable and non-specific, ranging from necrotizing angiitis which is undistinguishable from periarteritis nodosa, to scarring lesions. Micro-angiitis is easily demonstrated in skin lesions and is also encountered to varying degrees in CNS, renal, cardiac, pulmonary and gastrointestinal localisations. Disease of large vessels is more rare and sometimes causes gangrene of the limbs. In SLE, vasculitis should be distinguished from thrombosis related to lupus anticoagulant and from atherosclerosis favoured by chronic steroid therapy but perhaps initiated by vascular deposits of immune complexes during the acute inflammatory stage. The treatment of lupic angiitis is mainly based on steroid therapy. The results are variable, probably due to the fibrous nature of some of the vascular lesions.

[Treatment of systemic scleroderma with ketanserin. Randomized, double-blind 6-months study of 27 cases]. / Traitement de la sclérodermie systémique par la Kétansérine. Etude randomisée, en double aveugle, sur 6 mois, de 27 cas.

Twenty-seven patients with systemic scleroderma and Raynaud's phenomenon underwent a randomised double blind therapeutic trial: monotherapy with Ketanserine (80 mg/day for 6 months) against Placebo. The secondary effects were comparable in both groups as were the withdrawals from the trial for aggravation of Raynaud's phenomenon (one in each group). No significant difference was observed between the two groups as regards the evolution of the Raynaud's phenomenon or skin changes. Dysphagia was improved in the Ketanserine group (p less than 0.05) but not in the Placebo group. Some patients in the Ketanserine group experienced an improvement in the Raynaud's phenomenon at the end of the trial period; there were no improvements in the Placebo group. Three haemorrheological parameters (total blood viscosity, plasma viscosity and thixotropism) were abnormal at the beginning of the trial and did not improve by the end in the Ketanserine group. The K infinity coefficient of Quemada's law was normal at the start of the trial and increased after treatment (p less than 0.05).

[Collagen diseases and microcirculation]. / Collagénoses et microcirculation.

The microcirculation (terminal vessels with a calibre of less than 30 mu) was examined by means of capillaroscopy which was correlated with histological studies. According to the classification of vasculitis, microvasculitis is a pathological process involved in a number of collagen diseases. The study of the cutaneous lesions of leukocytoclastic angiitis is one of the best method for approaching the study of these microangiopathies, especially in the context of hypersensitivity vasculitis. The study of the microcirculation may also be valuable in other connective tissue diseases (various forms of necrotic vasculitis, lupus, scleroderma, polydermatomyositis) and in Behçet's disease. However, this study is only in its preliminary stages and the data of capillaroscopy need to be interpreted critically.

[A case of purpura, or the return to natural diseases]. / A propos d'un cas de purpura ou le retour aux "maladies naturelles".

With reference to a case of vitamin C deficiency in a strict vegetarian, the authors recall the clinical findings and current diagnostic procedures in scurvy. Serum and urine ascorbic acid assays are now available and established the diagnosis. Management rests upon vitamin C given in a curative dosage of 1 to 2 g per day for 15 days followed by a preventive dosage of 10 mg per day.

[Periungual capillaroscopic aspects in Behçet's disease. Apropos of 30 cases]. / Aspects capillaroscopiques péri-unguéaux au cours de la maladie de Behcet. A propos de 30 observations.

Nailed capillaroscopy was systematically performed in 30 patients with Behcet's disease to search capillary dystrophies with paradoxal normal number of capillary loops; some indirect signs of microvascular disease were noted: pallor of the background; petechiae; sludge; abnormal visibility of the venous plexus and of irregularly arranged venules. Eight patients had a normal capillaroscopy: thirteen had direct signs of microvalvular abnormalities (nine cases with two direct signs and at least two indirect signs; and four cases with two direct and one indirect, or one direct and three indirect signs). Nine patients had only sludge and/or petechiae. We never observed any abnormality in the number of the capillaries, in the sweat droplets nor in the circulatory speed. Two patients had megacapillaries resembling those seen in scleroderma. The petechiae were observed in 50 p. 100 of the cases. We found no correlation between the capillaroscopic abnormalities and the age, sex, duration of the disease, or ethnic background. However, the presence of aphthosis the day of the capillaroscopy seems to be correlated with the cutaneous microvascular abnormalities. Thus, there exist frequent capillaroscopic abnormalities in Behcet's disease. Although aspecific, they point out the vascular tropism of this disease, resembling that of the vascularite. The prognostic importance of the capillaroscopy in Behcet's disease is under study.

[Long-term development of eosinophilic fasciitis. Study of 11 cases]. / Evolution à long terme des fasciites avec éosinophilie. Etude de 11 cas.

The long-term outcome of eosinophilic fasciitis (EF) described for the first time by Shulman in 1974 remains unclear. Its exact pathological classification is still contested. We reviewed 11 cases selected exclusively on their initial symptoms being compatible with EF. The outcome of the cutaneous lesions, the investigations of possible visceral involvement were analysed with a follow-up of 1 to 9.5 years (average 4.8 years). Regression of the cutaneous infiltration was complete in 5 cases and partial in 3 cases. No regression was observed in 3 cases. Steroid therapy was given in 10 patients: objective improvement was observed in 5 patients. There was no improvement in the other 5 cases. Ungual capillaroscopy was performed in 9 patients and showed none of the characteristic capillary changes of scleroderma. These results suggest that the prognosis and outcome of EF justify its distinction from systemic scleroderma. The effects of steroid therapy on the cutaneous lesions are very variable.

An investigation of the complement system in patients with periodic disease (results from 29 cases).

The complement system was investigated in 29 patients suffering from authentic periodic disease. A statistically significant increase in C4, also in total complement and C3 could be demonstrated. It is possible that the increase in C4 was due to the macrophages which are always present in the infiltrates of periodic disease. This biological observation is of clear practical importance for the diagnosis of the condition both before and after colchicine therapy.