Mottled Raynaud's phenomenon and hand-arm vibration syndrome: followed up for 10 years.

Vibration white finger is a form of secondary Raynaud's phenomenon (RP) caused by the use of handheld vibrating tools. RP usually appears on the extremities of the fingers, and its borders are well recognised. No reports have been published on 'mottled' RP in continuous observation from the onset to the disappearance of RP. A man in his 60s who had been using vibrating tools such as jackhammers and tampers for 30 years presented with sensations of coldness, burning and numbness. Whole-body cold exposure was performed outdoors in winter, and RP was photographed continuously. 'Mottled' RP can be defined as triphasic colour changes: white, blue and red. The patient was taken off work, kept warm and medicated. His symptoms improved slightly after 10 years of follow-up, but the RP did not disappear. 'Mottled' RP is rare and refractory and should be recognised as a form of RP.

[Paroxysmal vascular acrosyndromes: Practical approach to diagnosis and management]. / Acrosyndromes vasculaires paroxystiques : démarche pratique de diagnostic et de prise en charge.

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.

The effect of vitamin D3 and thyroid hormones on the capillaroscopy-confirmed microangiopathy in pediatric patients with a suspicion of systemic connective tissue disease-a single-center experience with Raynaud phenomenon.

Raynaud phenomenon (RP) may be the first manifestation of a systemic connective tissue disease (SCTD). Early detection of dysfunction of small vessels called microangiopathy is essential for the diagnostic process. The focus of this single-center, retrospective study was to investigate the potential dependencies between microvascular image and laboratory markers measured in children with RP. The study analyzed the nail-fold video-capillaroscopy (NVC) findings and laboratory results of 81 children between the ages 6 and 17 who were referred to pediatric rheumatologist with a suspicion of SCTD. Out of 52 patients presenting with RP at the time of evaluation, abnormalities in capillary microscopic imaging were found in 34. NVC findings were then compared to levels of specific biomarkers in serum. Vitamin D3 serum levels have been significantly decreased in patients with RP (23.4 ng/mL ± 8.76 vs. 30.0 ng/mL ± 12.66, P = 0.0148). There were positive significant correlations between levels of vitamin D3 and acute-phase reactants in serum, such as C-reactive protein (P = 0.0292). Furthermore, free thyroxine levels (fT4) in patients with both RP (P = 0.0126) and micro-angiopathy (P = 0.05496) persisted in the lower range of the normal limit (< 1.0 ng/dL). Regular oral supplementation of vitamin D3 should be always considered in children with RP if deficiency is found. Additionally, low fT4 level (< 1.0 ng/dL) should be considered as an indication to perform NVC in patients suspected of SCTD even when they do not present RP.

Raynaud Phenomenon of the Nipple: An Under-Recognized Condition.

BACKGROUND: Obstetricians often feel ill-equipped to address the symptom of breast pain in pregnant and postpartum patients. CASES: In the first case, a 40-year-old woman in the second trimester of pregnancy reported nipple discoloration and severe pain. She was treated with nifedipine, and her symptoms decreased quickly and markedly. In the second case, a 32-year-old woman presented for a routine postpartum visit. She described breast pain and sporadic purple discoloration of the nipples, a finding confirmed on examination. Conservative measures of maintaining warmth were recommended. CONCLUSION: Raynaud phenomenon of the nipple is an underdiagnosed condition affecting women in both the prenatal and postpartum periods. A careful history and physical must be obtained in women presenting with breast pain, because diagnosis and treatment allows breastfeeding continuation and mitigation of symptoms.

Raynaud's phenomenon: Current concepts.

Raynaud's phenomenon (RP) is a transient, acral, vasospastic phenomenon that manifests with characteristic color changes. This vasospasm, classically triggered by cold temperatures, may also be driven by shifts in temperature, climate, or emotional state. Primary RP (PRP) is a common condition without severe sequelae. Secondary RP (SRP), which may be driven by vascular, autoimmune, hematologic, or endocrine etiologies, can result in digital ulceration, irreversible ischemia and necrosis, and secondary infection. This review delineates the clinical manifestations of both primary and secondary RP, as well as the current understanding of RP epidemiology and pathogenesis. Proper examination, including nailfold capillary microscopy, and laboratory workup for secondary causes of RP are also discussed. The traditional armamentarium of therapies used for RP, as well as newer medical and surgical options, is also summarized with particular regard to the clinical evidence for their efficacy.

Severe digital necrosis in a 4-year-old boy: primary Raynaud's or jellyfish sting.

Raynaud's phenomena is a common disorder which may be primary or secondary to some connective tissue disorders such as systemic sclerosis and systemic lupus erythematosus. Jellyfish sting is a rare but life-threatening cause of Raynaud's phenomena. Digital gangrene is reported in 3% of children with secondary Raynaud's phenomena but does not occur in children with primary Raynaud's phenomena. We report a case of a 4-year-old boy who initially presented with episodes of pain and bluish to blackish discolouration and necrosis affecting the fingers on both hands after a jellyfish sting without any sign of connective tissue disorder.

Raynaud's phenomenon, cytokines and acupuncture: a case report.

A 30-year-old African-American woman diagnosed in 2006 with primary Raynaud's phenomenon (RP) was seen in the clinic in 2010 and the diagnosis confirmed excluding underlying disorders. Acupuncture was administered bilaterally at the LI4 Hegu acupuncture points for 5 min twice weekly for 2 months, which resulted in improvement in pain severity, joint stiffness and the colour of her fingers and toes. The literature reveals that acupuncture is effective in improving pain severity and joint stiffness in RP. The patient's serum proinflammatory cytokines were compared with those from an ongoing study in our institution and the results indicated that acupuncture therapy might be anti-inflammatory. Acupuncture is relatively safe and should be considered as an alternative treatment or non-pharmacological therapy for pain associated with RP.

Fenómeno de Raynaud y el amamantamiento doloroso / Raynaud's phenomenon and pain during breastfeeding

El fenómeno de Raynaud es una patología provocada por la exposición al frío, que conduce al vasoespasmo intermitente de arteriolas, produciendo un cuadro clínico por la falta de aporte de oxígeno, consistente en palidez, parestesias y dolor de la zona afectada1. Es una causa tratable de amamantamiento doloroso que llega a afectar a 1 de cada 5 mujeres en edad fértil y que en ocasiones lleva al abandono de la lactancia si es erróneamente diagnosticado por su incomoda sintomatología (AU)

Raynaud's phenomenon of the nipple is a pathology precipitated by exposure to cold and leads to intermittent vasospasm of arterioles, producing clinical symptoms of pain, burning and paresthesias. This condition is a treatable cause of painful breastfeeding that can affect up to 1 in 5 women of childbearing age. If misdiagnosed, it can occasionally lead to cessation of breastfeeding due to its uncomfortable symptomatology (AU)

Effect of biofeedback and deep oscillation on Raynaud's phenomenon secondary to systemic sclerosis: results of a controlled prospective randomized clinical trial.

Our aim was to evaluate the effect of deep oscillation and biofeedback on Raynaud's phenomenon (RP) secondary to systemic sclerosis (SSc). A prospective randomized study was performed in SSc patients receiving either deep oscillation (n = 10) or biofeedback (n = 8) thrice a week for 4 weeks, or patients were randomized into the waiting group untreated for vasculopathy (n = 10) in time of running the study interventions. Biofeedback resulted in an improvement of RP as determined by score reduction of visual analogue scale compared with patients of the control group (P < 0.05), whereas deep oscillation revealed a tendency for improvement (P = 0.055). The study underlines the beneficial role of physiotherapy for the treatment of SSc-related RP.

Random effects models for assessing diagnostic accuracy of traditional Chinese doctors in absence of a gold standard.

Two common problems in assessing the accuracy of traditional Chinese medicine (TCM) doctors in detecting a particular symptom are the unknown true symptom status and the ordinal-scale of the symptom status. Wang et al. (Biostatistics 2011; DOI: 10.1093/biostatistics/kxq075) proposed a nonparametric maximum likelihood method for estimating the accuracy of different TCM doctors without a gold standard when the true symptom status is measured on an ordinal-scale. A key assumption of their work is that the diagnosis results are independent conditional on the gold standard. This assumption can be violated in many practical situations.In this paper, we propose a random effects modeling approach that extends their method to incorporate dependence structure among different tests or doctors. The proposed method is illustrated on a real data set from TCM, which contains the diagnostic results from five doctors for the same patients regarding symptoms related to Chills disease. The same data set was analyzed by Wang et al. under the conditional independence assumption. In addition, we also discuss an ad hoc test for the model fitting and a likelihood ratio test on the random effects.

25. Ischemic pain in the extremities and Raynaud's phenomenon.

Two important groups of disorders result from an insufficient blood supply to the extremities: critical vascular disease and the Raynaud's phenomenon. The latter can be subdivided into a primary and a secondary type. Critical ischemic disease is often caused by arteriosclerosis due to hypertension or diabetes. Primary Raynaud's is idiopathic and will be diagnosed as such if underlying systemic pathology has been excluded. Secondary Raynaud's is often a manifestation of a systemic disease. It is essential to try to establish a diagnosis as soon as possible in order to influence the evolution of the disease. A sympathetic nerve block can be considered in patients with critical ischemic vascular disease after extensive conservative treatment, preferably in the context of a study (2B±). If this has insufficient effect, spinal cord stimulation can be considered in a selected patient group (2B±). In view of the degree of invasiveness and the costs involved, this treatment should preferably be applied in the context of a study and with the use of transcutaneous pO(2) measurements. In case of primary Raynaud's, life style changes are the first step. Sympathectomy can be considered as a treatment of Raynaud's phenomenon (2C+), but only after multidisciplinary evaluation of the patient and in close consultation with the patient's rheumatologist, vascular surgeon or internist.

[The experience with thoracoscopic sympathectomy for the treatment of distal lesions of limb arteries].

The study included 107 patients with distal lesions of limb arteries treated with the use of thoracoscopic sympathectomy. The best results in the early postoperative period were obtained in patients with Raynaud's disease. Modifications introduced into the methods of its treatment permitted to retain effectiveness of sympathectomy till the late postoperative period in 90% of the patients. Surgery for obliterative endarteritis and atherosclerosis was viewed as a possibility to preserve the supporting function of the limb. This purpose was attained in 73.2 and 62.5% of the patients respectively in the early and in 62 and 25% in the late postoperative periods.

Auricular electroacupuncture reduces frequency and severity of Raynaud attacks.

BACKGROUND: Acupuncture has been shown to influence skin perfusion and the subjective cold perception threshold. Therefore, we hypothesized that auricular electroacupuncture (EA) might reduce symptoms in primary Raynaud's phenomenon (PRP). METHODS: Twenty-six patients with PRP received 6 cycles of auricular EA. After 3, 6 and 24 weeks attack frequency and severity were reevaluated using standardized questionnaires and a visual analogue scale (VAS). Skin temperature was assessed by infrared thermography and laser Doppler perfusion imaging was used to determine skin perfusion. RESULTS: Compared to baseline we found a significant reduction of attack frequency after 3 (p = 0.001) and 6 weeks (p < 0.001) of auricular EA. This improvement sustained following cessation of EA, after 24 weeks (p < 0.001). Furthermore, attack associated pain was reduced after 3 (p = 0.003), 6 (p = 0.003) and 24 weeks (p = 0.001) of treatment, while skin temperature and skin perfusion did not change significantly throughout the study period. CONCLUSIONS: Auricular EA reduces symptoms by means of frequency and severity of attacks in PRP but has no influence on skin perfusion and skin temperature.

[The usefulness of capillaroscopy for the evaluation of pulsed magnetic field therapy in patients with primary and secondary Raynaud's phenomenon]. / Przydatnoc kapilaroskopii do oceny terapii impulsowym polem magnetycznym pacjentów z pierwotnym i wtórnym objawem Raynauda.

INTRODUCTION: Raynaud's phenomenon (RP) as a vasospastic response to cold and emotions has an impact on the quality of life. This also applies to patients with primary (idiopathic) RP. Moreover, RP may also be a factor of irreversible tissue injury manifested by ulceration or necrosis, especially in patients with connective tissue disease. Nailfold videocapillaroscopy (NVC) is a recognized method for diagnosing and monitoring of microvascular abnormalities in rheumatic diseases. The purpose of this study was to assess the usefulness of capillaroscopy for monitoring of the therapeutic effect of low-frequency pulsed magnetic field in 44 patients with RP. MATERIAL AND METHODS: Clinical examination and NVC were performed before and after 2 weeks of treatment. Low-frequency pulsed magnetic field was administered 5 days per week for 2 weeks with the Magnetronic MF-10 generator operating at 40 Hz frequency and 1.0-5.0 mT induction values. Each session lasted 10-20 minutes. RESULTS: Patients with primary and secondary RP experienced a significant decrease in the number and duration of RP episodes and reported a reduction in pain on the Visual Analog Scale. Moreover, an improvement in vascular flow and reduction in interstitial edema was seen with NVC. A correlation between capillaroscopic findings and severity of RP was also observed. CONCLUSIONS: Capillaroscopy is an effective method for evaluation of the effects of low-frequency pulsed magnetic field therapy on the microcirculation in patients with primary and secondary RP. Further prospective studies on the effect of this therapy on capillaroscopic abnormalities in RP patients are needed.

Fenómeno de Raynaud / Raynaud’s Phenomenon

El fenómeno de Raynaud se caracteriza por una disminución de la circulación sanguínea, fundamentalmenteen los dedos de las manos y de los pies, con dolor y cambios de coloración en la piel deuna forma secuencial: blanco, azul y rojo. Es más frecuente en las mujeres, en la 2ª y 3ª décadasde la vida. Se desconoce cuál es su causa, pero guarda relación con el consumo de tabaco, alcohol,y fundamentalmente con los cambios de temperatura y alteraciones emocionales. Se asocia aenfermedades del tejido conectivo, entre las que Esclerodermia y Lupus son las más frecuentes. ElNifedipino es el fármaco de primera elección(AU)

Raynaud’s phenomenon is characterized by a decrease in blood circulation mainly in the fi ngers andtoes, with pain and changes in skin colour which follow a characteristic pattern white, blue and red. Itis more common in women, in the 2nd and 3rd decade of life. The cause is unknown, but is related tosmoking and alcohol consumption, and particularly body temperature changes and emotional stress.It is associated with connective tissue diseases amongst which scleroderma and lupus are the mostfrequent. Nifedipine is the treatment of choice(AU)

Cold stimulus fingertip lacticemy test--an effective method to monitor acute therapeutic intervention on primary Raynaud's phenomenon and systemic sclerosis.

OBJECTIVES: The recently developed cold stimulus fingertip lacticemy test (CS-FTL) provides biochemical assessment of peripheral perfusion in patients with Raynaud's phenomenon (RP). We evaluated how the CS-FTL test can assess the acute effect of nifedipine in microvascular dynamics on primary RP and RP secondary to SSc. METHODS: A double-blinded controlled trial with crossover design was performed in 20 primary RP and 20 SSc patients. Patients received one single sublingual placebo or 10 mg nifedipine capsule, with crossover after a 15-day washout period. FTL was determined in resting conditions (pre-CS-FTL) and 10 min after CS (post-CS-FTL), before and 1 h after drug administration. Percent variation in post- vs pre-CS-FTL was expressed as deltaCS-FTL. RESULTS: Before intervention, CS induced FTL decrease in primary RP (deltaCS-FTL = -21.3 +/- 13.0%) and FTL increase in SSc patients (deltaCS-FTL = +24.5 +/- 21.2%). Placebo had no effect on pre-CS-FTL, post-CS-FTL and deltaCS-FTL in primary RP and SSc. Nifedipine induced a significant decrease in pre-CS-FTL (1.94 +/- 0.45 vs 1.57 +/- 0.41 mg/dl; P = 0.005) and post-CS-FTL (1.53 +/- 0.35 vs 1.32 +/- 0.37 mg/dl; P = 0.004) in primary RP and a significant decrease in post-CS-FTL (3.18 +/- 1.43 vs 2.56 +/- 1.30 mg/dl; P = 0.028) and deltaCS-FTL (+15.9 +/- 24.7% vs -12.9 +/- 16.6%; P = 0.001) in SSc. CONCLUSIONS: The CS-FTL test was able to demonstrate and quantify a dual effect of nifedipine on the biochemical dimension of peripheral perfusion in primary RP and in SSc patients in which there was a significant improvement in tissue perfusion in resting conditions and after exposure to a CS. The CS-FTL test will enrich the armamentarium for investigation and clinical evaluation of conditions associated with RP.