[Lyme disease acrodermitis chronica atrophicans: misleading vascular signs]. / Acrodermatite atrophiante de Lyme : des signes vasculaires parfois trompeurs.

Lyme disease acrodermatitis chronica atrophicans is a tertiary form of Lyme borrelliosis. It occurs at least six months, but also up to several years, after a tick bite. This rare condition is probably underestimated because of the difficult diagnosis. Clinical presentations of acrodermatitis chronic atrophicans are quite variable depending upon the duration of the disease. Complimentary explorations are difficult to interpret and rarely specific. Only rare configurations allow formal diagnosis of Borrelia burgdoferi infection. We present a patient who exhibited an atypical clinical presentation of Lyme disease acrodermatitis chronic atrophicans. The clinical outcome was quite favorable with treatment, confirming the diagnosis. Such treatments, which are well tolerated and highly effective, are essential since an untreated disease can lead to potentially severe neurological involvement.

Use of bosentan for digital ulcers related to systemic sclerosis: a real-life retrospective French study of 89 patients treated since specific approval.

OBJECTIVES: Ischaemic digital ulcers (DUs) are a common complication of systemic sclerosis (SSc). This study aimed to characterize patients with SSc and ongoing DUs treated with the endothelin receptor antagonist bosentan in clinical practice in France. METHOD: An observational, retrospective, longitudinal study was conducted in 10 French expert centres. Medical records from randomly selected adult SSc patients who received treatment with bosentan for DU prevention from March 2007 to December 2010 were analysed. The primary objective was to determine the profile of patients at treatment initiation. Secondary objectives were to monitor bosentan dosing, treatment schedule, and reasons for treatment termination. RESULTS: The study included 89 patients (mean age 52 years, 69% female, 44% diffuse cutaneous SSc). At bosentan treatment initiation, the mean duration of Raynaud's phenomenon was 15 ± 12 years, and the mean time since first episode with DU was 6.5 ± 7 years. Most patients had a history of at least two episodes with DUs, separated by < 12 months (61%), and had received intravenous iloprost (63%). Previous DU complications included auto-amputation (8%), surgical amputation (6%), osteitis (6%), and gangrene (4.5%). Active smokers (25%) had a history of significantly more surgical amputation (p = 0.004) and osteitis (p = 0.004) than non-smokers. At least one active DU at bosentan initiation was detected in 82% of patients. Bosentan was used according to prescription guidelines and was well tolerated; six patients (7%) withdrew from treatment because of raised liver enzymes. CONCLUSIONS: Patients treated with bosentan for DU prevention in France have severe, refractory, ongoing ulcerative disease. Active smoking was correlated to a history of DU complications. Tolerance of bosentan was comparable to previous studies.

Cutaneous iontophoresis of treprostinil in systemic sclerosis: a proof-of-concept study.

Ischemic digital ulcer (DU) is a serious complication of systemic sclerosis (SSc). Intravenous prostanoids are the only approved treatment for active DUs, but they induce dose-limiting side effects and require hospitalization. Our objective was to evaluate the effect of iontophoresis (a noninvasive drug delivery method) of treprostinil in SSc patients. Three studies were conducted: a pharmacokinetic study in 12 healthy volunteers showed that peak dermal concentration was reached at 2 hours, whereas plasma treprostinil was undetected. Then, a placebo-controlled, double-blind incremental dose study assessed the effect of treprostinil on digital skin blood flow in 22 healthy subjects. The effect of the highest dose was then compared with that of placebo in 12 SSc patients. Treprostinil significantly increased skin blood flow in healthy subjects (P = 0.006) and in SSc patients (P = 0.023). In conclusion, digital iontophoresis of treprostinil is feasible, is well tolerated, and increases digital skin perfusion. It could be tested as a treatment for SSc-related DUs.

[Secretan's syndrome: myth or pathomimia?]. / Le syndrome de Secrétan : mythe ou pathomimie ?

UNLABELLED: Secretan's syndrome is a rare condition involving generally trauma-induced hard edema of the dorsal aspect of the hand. The cause is poorly understood but factitious trauma is often suspected. CASE REPORT: A 42-year-old woman presented with a fortuitous edema on the back of the right hand. The minimally depressible edema was associated with moderately intense mechanical pain. Routine laboratory tests were normal. An extensive imaging work-up (bone x-ray of the hand and wrist, bone scintigraphy, computed tomography phlebography, lymphoscintigraphy, magnetic resonance imaging) was equally non-contributive. The diagnosis of self-inflected trauma was suggested by the atypical nature of the edema, the absence of any organic disorder on the tests performed, and the patient's attitude concerning her disease. In this clinical context, the diagnosis of Secretan's syndrome was retained. Outcome was compatible, with secondary development of complex regional pain syndrome. DISCUSSION: Three forms of Secretan's syndrome have been recently described: benign; hyperplastic; and mixed. The cause remains poorly defined. Certain authors report that it is most likely related to pathomimia. Treatment can combine physiotherapy and psychological counseling. CONCLUSION: Secretan's syndrome is a poorly-understood and rarely-described condition that may be underdiagnosed. Physicians specialized in vascular medicine should be aware of this syndrome and its difficult diagnosis by elimination.

[Minimal work-up for Raynaud syndrome: a consensus report. Microcirculation working group of the Société française de médecine vasculaire]. / Bilan étiologique minimal du phénomène de Raynaud : un consensus d'experts.

About ten to fifteen percent of the French population suffer from Raynaud's phenomenon. Most of the time, it is considered as primary Raynaud's phenomenon, without underlying disease. The aim of this expert consensus from the "microcirculation group" for the French Society of Vascular Medicine and the French Society for Microcirculation, was to define clinical guidelines in patients consulting for Raynaud's phenomenon. The recommended minimal screening includes clinical examination, nailfold capillaroscopy and antinuclear antibodies. In particular, the aim of this screening is to identify patients with a significant risk for scleroderma, who would need a careful follow up.

[Chromametry, a promising technique for the quantification of skin changes in chronic venous disorders]. / La chromamétrie, une technique prometteuse pour la quantification de l'atteinte cutanée dans l'insuffisance veineuse chronique.

OBJECTIVE: The development and validation of new clinimetric tools is essential for the progress of clinical research in the field of chronic venous insufficiency. Chromametry is a simple, quick and non-invasive technique that measures the color of the skin. The aim of this study was to evaluate the ability of this technique to quantify skin pigmentation as a marker of severity of chronic venous disease and to assess the variability of measurements obtained in this condition. METHODS: Chomametry was performed on three different sites on each lower limb in 42 patients undergoing a spa treatment in La Léchère (Savoie) for chronic venous disorders (CVD). Four series of measurements were taken by two investigators for each patient, at two sessions two to four days apart. RESULTS: The chromameter readily measured the pigmentation index (PI). The PI increased with higher clinical class (CEAP classification) for measurements made at the malleolar level (r=0.48; P<0.001) and the supra-malleolar area (r=0.55; P<0.001), but not at the level of the anterior tibial tuberosity (r=-0.09; P=0.45). The repeatability and the intra- and inter-observer reproducibility of this PI index were 15%, 18% and 21% respectively of the mean of the observed difference at the malleolar level. The chromameter also provided an erythema index, which appears to be less relevant and more variable than the PI, but which might add potentially useful information regarding the characterization of skin inflammation related to the venous disease. CONCLUSION: This study shows that chromametry can be used in clinical research studies to quantify skin changes associated with CVD. Whether it can also be useful for early detection and follow-up of patients with venous trophic changes remains to be investigated.

[Minimal work-up for Raynaud syndrome: a consensus report. Microcirculation Working Group of the French Vascular Medicine Society]. / Bilan étiologique minimal du phénomène de Raynaud : un consensus d'experts.

About ten to fifteen percent of the French population suffer from Raynaud's phenomenon. Most of the time, it is considered as primary Raynaud's phenomenon, without underlying disease. The aim of this expert consensus from the "microcirculation group" for the French Society of Vascular Medicine and the French Society for Microcirculation, was to define clinical guidelines in patients consulting for Raynaud's phenomenon. The recommended minimal screening includes clinical examination, nailfold capillaroscopy and antinuclear antibodies. In particular, the aim of this screening is to identify patients with a significant risk for scleroderma, who would need a careful follow up.

Acceptability and practicability of elastic compression stockings in the elderly: a randomized controlled evaluation.

BACKGROUND: Compression stockings are the cornerstone of the treatment of chronic venous disorders, but practical acceptability is an important limitation in the elderly. OBJECTIVE: To evaluate the practicability of compression stockings in elderly patients. METHODS: Twenty women aged 68-85 years without major disability were asked to put on, wear for three hours and take off Solegg® and Solegg® Fine compressive stockings (15-20 mmHg) in random order on different days, and to rate through questionnaires the difficulties and discomfort they experienced in comparison with their usual non-compressive stockings (controls). RESULTS: Foot and heel insertions of the compression stockings, as well as their removal, were found significantly more difficult, whereas comfort when they were on was higher. In the whole, the compression stockings were found to be more agreeable than the controls. CONCLUSION: Difficulties regarding putting on and removal of the compression stockings remain significant but are counterbalanced by a better comfort when they are on.