Paroxysmal vascular acrosyndromes are related to a peripheral vasomotor disorder and presented as paroxysmal color changes of the fingers. They include primary Raynaud\'s phenomenon (RP), which is the most common, secondary RP and erythermalgia. They are to be distinguished from non-paroxysmal acrosyndromes such as acrocyanosis and chilblains, which are very frequent and often associated with RP, digital ischemia and necrosis, spontaneous digital hematoma and acrocholosis. The challenge of a consultation for a vascular acrosyndrome is to make positive diagnosis through history and clinical examination, and to specify its nature, to prescribe complementary exams. In any patient consulting for RP, assessment includes at least an antinuclear antibody test and capillaroscopy. For erythermalgia, a blood count and even a search for JAK2 mutation are required. A thryoid-stimulating hormon assay, a test for antinuclear antibodies, and a search for small fiber neuropathy are also performed. The treatment of RP is essentially documented for secondary RP where calcium channel blockers are indicated in first line, and iloprost in severe cases. The treatment of primitive erythermalgia is based on sodium channel blockers such as mexiletine or lidocaine infusions, and on drugs effective on neuropathic pain, such as gabapentin or amitryptiline, in case of erythermalgia associated with small fiber neuropathy. The treatment of erythermalgia associated with myeloproliferative syndromes is based on etiological treatment and aspirin.

BACKGROUND: This is a clinical case illustrating a diagnosis of an IgG4 related-disease (IgG4-RD) diagnosed in a vascular context.
METHODS: A 47-year-old man with no past medical history consulted for a recent and disabling Raynaud phenomenon without trophic disorder. Vascular examinations revealed multiple arterial thromboses with no abnormal finger and toe pressures. Secondly, weight loss and submandibular glands enlargement appeared, leading to the diagnosis of IgG4-RD without a link being able to be established with vascular involvement. This is the second observation of this association. A French translation of the new classification criteria for IgG4-RD published in 2019 by the American College of Rheumatology and European Ligue Against Rhumatism (ACR/EULAR) is offered with direct application to the clinical case.
CONCLUSIONS: A Raynaud phenomenon with distal arterial thrombosis is rarely observed in the IgG4-RD.

Hypothenar hammer syndrome is a rare entity secondary to ulnar artery damage in the wrist, affecting mainly those exposed to repeated hand-palm trauma. Surgery is discussed in case of severe symptoms, resistant to medical treatment, and/or when anatomical lesions with emboligenic potential are demonstrated in the radiological exams. In this case, resection of the pathological zone with revascularization by autologous vein graft is the best option. We report the case of a 60-year-old patient who had a recurrence of symptoms more than 10 years after the completion of a surgical treatment. There was an aneurysmal thrombosed vein graft with extensive thrombus extending from the ulnar artery upstream of the Guyon\'s canal to the superficial palmar arch. The digital revascularization was provided by the radial superficial palmar arch and the presence of a collateral vascular supply. This late complication was responsible for compression of the ulnar nerve in Guyon\'s canal. A new surgery was performed consisting of the resection of the thrombosed zone, including the vein graft, without vascular reconstruction given the good vascularization of all the fingers, and release of the ulnar nerve to the wrist. The operative follow-up was uneventful with the disappearance of pain and sensory-motor deficits. Good digital vascularization was confirmed by imaging at 3 months postoperatively; nerve recovery by electromyogram at 6 months with normal conduction.

Hypothenar hammer syndrome is a rare condition secondary to ulnar artery damage in Guyon\'s canal, affecting mainly those exposed to repeated palm trauma. Surgery is discussed in cases of severe symptoms that are resistant to conservative treatment, and/or when anatomical lesions with high embolism potential are discovered during imaging exams. Resection of the pathological zone with revascularization by autologous vein graft is the best option. We report the case of a 60-year-old patient who had a recurrence of symptoms more than 10 years after this type of surgical treatment was performed. There was an aneurysmal thrombosed vein graft with extensive thrombus from the ulnar artery upstream to Guyon\'s canal to the superficial palmar arch. Finger revascularization was provided by the superficial branch of the radial artery and the presence of a collateral vascular supply. This late complication was responsible for compression of the ulnar nerve in Guyon\'s canal. A new surgery was performed to resect the thrombosed zone, including the vein graft, without vascular reconstruction due to the good vascularization of all the fingers, and to release the ulnar nerve at the wrist. The postoperative course was uneventful with the disappearance of pain and sensory-motor deficits. Good finger vascularization was confirmed by imaging at 3 months postoperative; nerve conduction was normal at 6 months on electroneuromyography.

BACKGROUND: Raynaud\'s phenomenon is a reversible episodic vasospastic disorder triggered by cold or emotion. Two types of Raynaud\'s phenomenon were distinguished: Raynaud\'s disease and secondary Raynaud\'s phenomenon. The purpose of this study was to determine the etiologic profile of secondary Raynaud\'s phenomenon in an internal medicine department.
METHODS: A descriptive retrospective study including patients with secondary Raynaud\'s phenomenon followed in a tertiary internal medicine department between 2000 and 2013.
RESULTS: We included 121 patients. The sex ratio M/F was 0.16. The mean age at the onset of Raynaud\'s phenomenon was 41.7 years. The average age of patients at the time of the etiologic diagnosis was 47.3 years. The mean delay between Raynaud\'s phenomenon onset and the first consultation was 41.33 months. Raynaud\'s phenomenon involved hands in all cases and feet in 16.10% of cases with a typical form in most cases (41.4%). Complications (digital ulcers and scars) were noted in 32.23% of cases. Nail fold capillaroscopy showed scleroderma pattern in 49.52% of patients. Antinuclear antibodies were positive in 88.49% of patients. Interstitial lung disease was reported in 54.04% of cases. Connective tissue diseases were diagnosed in 86.77% of patients. Other secondary Raynaud\'s phenomenon causes were vasculitis (6.61%), atherosclerosis (1.65%) and medical or professional causes (1.65%). The most frequent one cause systemic sclerosis (n=61, 98%) followed by systemic lupus erythematosus (11.57%) and primary Sjögren syndrome (6.61%).
CONCLUSIONS: In our study, the Raynaud\'s phenomenon was most frequently secondary to connective tissue diseases. This may be a selection bias because our department is a third-line unit where patients are often referred for systemic disease suspicion.

OBJECTIVE: Raynaud\'s phenomenon (RP) is a common cause for consultation. Capillaroscopy is a well-established technique to detect capillary abnormalities suggestive of a connective tissue disease, but it is sometimes unavailable. The aim of this study was to compare dermoscopy and capillaroscopy in the assessment of RP.
METHODS: This was a prospective single-centre observational study in adult patients consulting for RP at the Hôpital Nord Franche-Comté between January 2014 and June 2015. Dermoscopy was performed at dermatological consultations and capillaroscopy was prescribed. For each capillaroscopy and dermoscopy, the following parameters were examined: normal appearance, giant capillaries, avascular areas, dystrophic capillaries or tortuosity and haemorrhages. Kappa coefficients were calculated.
RESULTS: Twenty-six patients participated in this study. The kappa coefficient was 0.76 for \"normal\" status, 0.78 for tortuosity, 0.70 for giant capillaries, 0.48 for haemorrhage and 0.62 for avascular areas. The global kappa coefficient was 0.33. Detection of these abnormalities with capillaroscopy was significantly associated with abnormal dermoscopic status (P<0.05). The sensitivity of dermoscopy for the detection of \"abnormal\" capillaroscopic status was 0.87.
CONCLUSIONS: The correlation coefficients were good. Despite poor global concordance, 80% of patients had the same status, normal or abnormal, for both capillaroscopy and dermoscopy, which resulted in the same clinical management. Dermoscopy is thus a valuable tool screening for periungual anomalies and provides support for clinical examination by the dermatologist, although the reference method continues to be capillaroscopy.

OBJECTIVE: To estimate the association between exposure to medicinal products and Raynaud phenomenon.
METHODS: The study used the data of all adverse drug reactions notified to the French national pharmacovigilance database. All cases reported between 1st January 1995 and 10th December 2012 were selected. A case/non-case method was used to measure disproportionality of the association between drug exposure and Raynaud phenomenon. The cases concerned all observations involving Raynaud phenomenon. Non-cases comprised all other reports of adverse drug reactions over the same period.
CONCLUSIONS: Among the 307,128 adverse drug reaction reports selected from the French national pharmacovigilance database, 175 involved Raynaud phenomenon, most of them affecting women (61.1%). The mean age was 50.1 years, and 8% had a past medical history of Raynaud phenomenon. According to the summaries of product characteristics, 42.3% of these cases were exposed to drugs known to induce Raynaud phenomenon. Unexpected Raynaud phenomenons (unlisted in the summaries of product characteristics) were associated with exposure to drugs for which Raynaud phenomenons are published (interferons, ribavirin, gemcitabine) or for which Raynaud phenomenons are not published (hepatitis B vaccine, isotretinoin, leflunomide, hydroxycarbamide, rofecoxib, telmisartan, zolmitriptan).
CONCLUSIONS: The case/non-case method is usually used to generate signals. Further epidemiological studies are now necessary to confirm these findings.

Vascular acrosyndromes are associated with vasomotor disorders. They may be paroxysmal, like Raynaud\'s phenomenon, whitening of the fingers on exposure to cold, or erythromelalgia, a painful form of erythema induced by exposure to heat. Others are permanent or semi-permanent, such as acrocyanosis, chilblains, spontaneous haematoma of the fingers, acrocholose and digital ischaemia or necrosis. Diagnosis of the type of acrosyndrome at issue is based primarily on clinical examination and history-taking. Capillaroscopy and antinuclear antibody assay are key examinations essential for distinguishing between primary and secondary Raynaud\'s phenomenon and connective tissue disorders. Complete blood counts, screening for thyroid dysthyroidism, and antinuclear antibody assay can help distinguish between primary erythromelalgia and erythromelalgia secondary to a systemic disease, principally myeloproliferative syndrome. In the case of acrocyanosis, spontaneous digital haematomas and typical bilateral chilblains, examinations are of no value. For the other permanent and semi-permanent acrosyndromes such as digital ischaemia and purpuric or livedoid lesions, screening for arterial or thrombotic disease is necessary.

Periungueal capillaroscopy is a simple and reliable non-invasive technique allowing evaluation of cutaneous microcirculation. It was promoted for decades in patients with Raynaud\'s phenomenon in order to differentiate between the benign primary Raynaud\'s phenomenon and the secondary form in connective tissue diseases, especially systemic sclerosis. Nevertheless, the value of this procedure has also been shown in numerous pathologies such as diabetes or cardiovascular diseases. This literature review points to the versatility of this useful exam and its results in a large spectrum of diseases with microvascular involvement.

We report a case of a 76-year-old woman with isolated unilateral Raynaud phenomenon revealing giant-cell arteritis with diffuse arterial lesions and bilateral renal artery stenosis. Doppler ultrasonography showed bilateral stenosis of the subclavian and axillary arteries. Angio-CT PET enlightened diffuse arterial lesions, mainly involving the aorta and the brachial and femoral arteries as well as bilateral renal ostial stenosis with right kidney ischemia. Diagnosis of giant-cell arteritis was made on the temporal artery biopsy. Corticosteroid therapy led to rapid clinical and radiological improvement. Clinical manifestations of giant-cell arteritis may be atypical. Diffuse arterial disease may exist in the absence of cephalic symptoms or significant inflammatory biological features. Ostial renal artery stenosis may induce potentially threatening renal ischemia.