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Background: Raynaud's phenomenon is a microvascular disorder that results in exaggerated vasoconstriction over vasodilatation secondary to an alteration in autonomic control. Though benign, it can result in severe ulceration and ultimately gangrene associated with disfiguration and permanent deformity. We present a case of severe secondary Raynaud's phenomenon in a black-African patient from a resource-limited setting, with focus on the difficulties encountered in the diagnosis and treatment., Case Presentation: A 43-year-old female Cameroonian farmer with a 7-year history of episodic paresthesia in her fingers and toes (when exposed to cold) presented to our emergency department with severe pain, ulceration, and "darkening" of her fingertips over a period of 2 days. An examination revealed bilateral ulceration and dry gangrene of her fingers and toes, based on which a diagnosis of secondary Raynaud's phenomenon due to a connective tissue disease was proposed. Results of paraclinical investigations were normal. Lifestyle modification along with a calcium channel blocker and phosphodiesterase type 5 inhibitor provided significant relief., Conclusions: An early diagnosis and knowledge on appropriate treatment of Raynaud's phenomenon is of vital importance to prevent permanent tissue damage and disability. Relying on biphasic color change for the diagnosis of Raynaud's phenomenon in black Africans can be potentially misleading.

Systemic sclerosis (SSc) is a connective tissue disease frequently associated with Raynaud's Phenomenon (RP). Among possible pharmacological treatments, phosphodiesterase 5 inhibitors are considered in cases of severe non -responsive RP. We present the case of a male SSc patient wh presented with critical finger ischemia and concomitant appearance of myocardial fibrosis after sudden interruption of sildenafil treatment.

In patients with Raynaud's phenomenon (RP), the role of medical history, capillaroscopy, and autoantibodies in order to provide an early diagnosis of connective tissue disease (CTD) were examined. 115 consecutive adults with uni-, bi-, or triphasic colour changes of the fingers were studied. RP was bilateral in 92.7% of patients. The middle finger was significantly more affected. A lack of association between fingers affected by RP and fingers with capillary abnormalities was observed OR = 0.75 (0.34-1.66). RP with the cyanotic phase had a higher risk at capillaroscopy to have hemorrhages OR = 4.46 (1.50-13.30) and giant capillaries OR = 24.85 (1.48-417.44). The thumb and triphasic involvement have an OR of 1.477 and 1.845, respectively. RP secondary to systemic sclerosis (SSc) had greater value of VAS pain (p = 0.011). The presence of anti-centromere antibodies was significantly associated with a higher risk of SSc (p < 0.001). 44.3% of subjects had uniphasic blanching of the fingers, and among these, 27% was diagnosed as having an overt or suspected CTD. Markers of a potential development of CTDs include severe RP symptoms, positive autoantibodies, and capillary abnormalities. These data support the proposal to not discharge patients with uniphasic blanching of the fingers to avoid missing the opportunity of an early diagnosis.

Nail-fold capillaroscopy is a non-invasive tool to study the microcirculation and is increasingly being used in dermatology, angiology and rheumatology. More recently, the use of video-capillaroscopy has allowed computer storage of capillaroscopic images (video-capillaroscopy), enabling evaluation of changes in capillaroscopic abnormalities during the follow-up of patients with systemic sclerosis or mixed connective tissue disease. Qualitative and quantitative assessment of the nail-fold dermal capillaries and of their organization can readily distinguish between a normal capillaroscopic pattern in primary Raynaud phenomenon and a specific sclerodermic pattern in secondary Raynaud phenomenon carrying a very high risk of systemic sclerosis. Apart from its important role as a diagnostic tool for distinguishing between primary and secondary Raynaud phenomenon, capillaroscopy is now used to predict the risk of development of digital ulcers and of future visceral complications in patients with systemic sclerosis. Moreover, nail-fold capillaroscopy is essential for differential diagnosis between connective tissue diseases, for the etiologic diagnosis of digital necrosis and diffuse interstitial lung disease, and in sclerodermiform syndromes., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Objective: To evaluate the prevalence of digital necrosis requiring surgical amputation in a single-center group of patients with systemic sclerosis (SSc) and to compare the characteristics of patients with and those without this severe complication., Methods: We reviewed the medical records of 188 patients with SSc [162 women, 26 men, mean age 59.2 yrs, mean disease duration 8.0 yrs, mean time from onset of Raynaud's phenomenon (RP) 11.7 yrs, median followup duration 92 mo] enrolled in the Rheumatology Unit since 2004. Demographic and clinical features were collected, as well as the presence of the typical risk factors for atherosclerosis., Results: Nine patients (4.8%) underwent partial or total surgical digital amputation because of necrotic process; all these patients except 1 had a long history of multiple and persisting digital ulcers. All 9 patients had concomitant large-vessel involvement. Comparison of cases with and without digital amputation showed that this complication was associated with older age, long history of RP, long disease duration, presence of anticentromere antibody, and coexistence of peripheral artery disease and hypercholesterolemia. Discussion. We noted that 4.8% of patients with SSc underwent digital amputation. Our retrospective analysis suggests that peripheral artery disease is strongly associated with digital amputation. The preventive strategy for digital ulcers and amputation associated with SSc should include an extensive diagnostic and preventive investigation for peripheral atherosclerosis.

Background: Thoracic sympathectomy is used in the management of a variety of upper limb disorders. We have analyzed the evidence for thoracic sympathectomy in the management of digital ischemia., Methods: We reviewed the English literature between 1980 and 2010. Our analysis included reports with the clinical end points of relief, recurrence of symptoms or healing of ulcers, or both. Primary Raynaud disease (PRD) and secondary Raynaud phenomenon (SRP) were analyzed separately., Results: An initial postoperative positive effect was reported in 92% of PRD patients and in 89% of SRP patients. Long-term beneficial effect was 58% for PRD and 89% for SRP. Ulcer healing or improvement was achieved in 95%., Conclusions: The available evidence suggests that thoracic sympathectomy has a role in the treatment of severe PRD and SRP, albeit with better results in SRP patients than in PRD patients. In case of digital ulceration, thoracic sympathectomy may maximize tissue preservation or prevent amputation., (Copyright © 2011 Society for Vascular Surgery. Published by Mosby, Inc. All rights reserved.)

Objectives: To demonstrate that unilateral digital sympathectomy, in patients with Raynaud's phenomenon (RP) and systemic sclerosis (SSc), may result in bilateral resolution of RP and digital ulcerations., Methods: We report a case of SSc and RP that had bilateral benefits from unilateral digital sympathectomy. A computer-assisted Medline/PubMed search of the medical literature was performed for 1960 through June 2009 using the keywords sympathectomy, Raynaud's phenomenon, systemic sclerosis, CREST, and digital ulcers. These searches were also combined with text words unilateral, ipsilateral, bilateral, digital sympathectomy, selective sympathectomy, autonomic nervous system, hyperhidrosis, etiology, pathogenesis, hypothesis, and treatment. Only pertinent literature, primarily in the English language, was included., Results: The majority of patients with SSc have RP and many suffer from digital ulcerations. Medical and behavioral management may have limited benefit and surgical intervention can be considered in recalcitrant cases, although efficacy data are sparse. We describe a man with limited SSc who underwent unilateral digital sympathectomy but manifested bilateral benefit. To our knowledge, this is the first published report of contralateral response with this procedure. The patient ultimately demonstrated these digital benefits when stressed with extreme cold and hypoxia while mountaineering. Despite the onset of high-altitude sickness and cerebral edema, his fingers remained unaffected while other mountaineers sustained severe frostbite or died of hypothermia., Conclusions: Selective unilateral sympathectomy in SSc, for RP with digital ulcerations, can result in bilateral benefits despite intense challenge with cold and hypoxia., (© 2010 Elsevier Inc. All rights reserved.)

The aim of the study is to assess the clinical characteristics, risk factors and outcome of patients with systemic lupus erythematosus (SLE) complicated with digital gangrene. In all, 2684 consecutive SLE inpatients admitted to Peking Union Medical College Hospital from December 1997 to August 2007 were studied. Demographic data, clinical features, laboratory findings as well as therapeutic regimens were systematically reviewed and a database was established. Cases with digital gangrene were identified and followed up. 1) Eighteen patients with SLE were complicated with digital gangrene, the average age at event was 33.1 +/- 11.8 years and the average disease duration was 99.1 +/- 60.1 months. 2) Patients with SLE, with long disease duration (> or =4 years), Raynaud's phenomenon and elevated serum C-reactive protein (CRP) were more likely to develop digital gangrene, P = 0.006, 0.001, and 0.031, respectively, OR = 1.03 (95% CI 1.01, 1.04), 35.76 (95% CI 4.67, 273.83) and 9.93 (95% CI 1.23, 80.30), respectively. 3) Fifteen gangrene patients started prednisone > or =1 mg/kg/d, and 18 were treated with cyclophosphamide, although 8 cases failed and ultimately received digital amputation. Prompt corticosteroid treatment (prednisone > or = mg/kg/d started within 3 weeks) decreased the hazard of amputation, P = 0.073, HR = 0.13 (95% CI 0.01, 1.21). Long disease duration, Raynaud's phenomenon and elevated serum CRP were independent predictive factors for SLE to develop digital gangrene. Early and aggressive corticosteroid treatment prevented gangrene from progression and improved prognosis.

Objectives: To investigate the association between functional difficulties in using the upper limb and extent and frequency of finger blanching, and the merits of these markers in grading the severity of Raynaud's phenomenon (RP) and vibration-induced white finger (VWF)., Methods: A questionnaire was mailed to a randomly selected community sample of 22,194 working-aged adults. Information was collected on cold-induced finger blanching--including the extent and frequency of attacks in the past year, and on difficulty in using the upper limb in several everyday activities (e.g. doing up buttons, opening a tight screwtop jar, and pouring from a jug). Associations were examined by logistic regression with the resultant odds ratios converted into prevalence ratios (PRs) and 95% confidence intervals (95% CI)., Results: Among 12,907 respondents were 1,359 who reported finger blanching and provided details of its extent and frequency. Of these, 7.4% reported frequent attacks (50 or more over the year), and 12% reported extensive blanching (affecting nine or ten digits). After adjustment for potential confounders, subjects with finger blanching reported an excess of difficulties in using the limb. Thus, in men with blanching the PR for difficulty with buttons was 4.7 (95% CI 3.9-5.8), and that for pouring from a jug was 3.8 (3.0-4.9) in comparison with men who had never had blanching. Similar associations were found in women and in those men with exposure to hand-transmitted vibration. The risk of reporting difficulties increased markedly with frequency of blanching--up to four- or fivefold in those with 50 or more attacks in the past year compared with those who had none; but differences by extent were less marked, with PRs < or = 1.6 in those with nine to ten digits affected compared with one to two digits., Conclusions: RP and VWF are both associated with difficulties in using the upper limb in everyday tasks. Further investigation of potential reporting biases is warranted, but if the associations are causal, frequency of attacks influences impairment more than extent of disease. More account may need to be taken of frequency of blanching episodes in assessing and in compensating subjects with VWF.

In vivo nailfold capillary microscopy was performed on 10 men with vibration white finger (VWF) and 10 age-matched male controls. The observed nailfold capillaries required adaptation of Maricq's classifications and addition of new morphological scoring systems. These new classifications produced numerical scores for assessing capillary: dropout, tortuosity, elongation, visualization of subpapillary venular plexus, and the degree of disarrangement of nailfold capillary polarity. Application of these new scores showed, for the first time, a complex pattern of abnormal-nailfold capillaries in patients with VWF. Capillary dropout was evident in 7/10 patients, with an associated disarrangement in nailfold capillary polarity in five. All 10 controls had normal capillary morphology. Tortuosity of the capillary loops and elongation of their length was observed in 30% of patients. These significant morphological alterations seen in VWF suggest a local small-vessel vasculitis.

Temporary threshold shifts (TSSs) in vibrotactile perception produced by continuous vibration and repetitive shocks having one complete cycle of a 100 Hz sine wave and exponential decays. The repetition rate of the cycles was 5, 25, 50, or 100/s, while the root-mean-square (r.m.s.) acceleration measured over exposure of five minutes was held constant (weighted according to British Standard (BS) 6842 and International Standard (ISO) 5349). When exposed to five shocks per second at each of the three frequency-weighted acceleration magnitudes, the subjects developed a small TTS. Exposure to 100 shocks per second (continuous vibration) at each of the three frequency-weighted acceleration magnitudes caused a large TTS, although the total frequency-weighted energy was the same as when exposed to five shocks per second. The results suggest that the equal energy hypothesis underlying BS 6842 and ISO 5349 is inappropriate for the prediction of the TTS produced by repetitive shocks.

Image analysis of videographs of nail fold capillaries was performed in patients with systemic sclerosis (SS), and the capillary patterns were defined by canonical discriminant analysis. Forty-eight of 55 SS patients were significantly differentiated from patients with systemic lupus erythematosus and normal controls. Normal controls and patients with systemic lupus erythematosus showed identical nail fold capillary patterns; in patients with dermatomyositis the pattern was similar to that of SS. The canonical discriminant analysis revealed that only 5 of 55 (9%) SS patients showed a normal capillary pattern. The abnormal capillary pattern in SS patients correlated with microvascular disturbances such as Raynaud's phenomenon, digital pitting scars and low finger temperature. Our results indicate that quantitative analysis of the nail fold capillaries is useful in evaluating the microvascular disturbances in SS patients.

We describe a clinical skin sign in scleroderma termed round fingerpad sign. This term refers to disappearance of the peaked contour on fingerpads and replacement with a hemisphere-like fingertip contour; this change is especially apparent on the ring fingers. A positive round fingerpad sign was found in 72 of 72 ring fingers in 36 patients with progressive systemic sclerosis in 69 of 72 ring fingers in patients with mixed connective tissue disease, and in 24 of 24 ring fingers of patients with Raynaud's phenomenon and sclerodactyly. In contrast, a negative round fingerpad sign was seen in 240 of 240 fingers in normal women (controls). The high sensitivity of this sign is noteworthy. A positive round fingerpad was seen in sign not only typical scleroderma patients but also in groups with less severe skin sclerosis (i.e., in those patients with mixed connective tissue disease or those with Raynaud's phenomenon and sclerodactyly). The sign is a new and useful clinical marker for the early diagnosis of scleroderma.

Twenty young adult patients with hand and digital ischemia were found to have obstructive arterial disease. All patients were surgically explored, and the occluded vessels were resected and by-passed. Eighteen patients had obstruction at the level of the distal ulnar artery and palmar arch, and 12 had obstruction of the common digital and digital proper arteries. Occlusive arteriosclerotic lesions were found in all patients; these lesions were characterized by prominent fibromuscular intimal plaques with superimposed thrombosis. Six patients had also thromboembolism to distal digital vessels. Vasculitis, calcifications, cholesterol deposits, and atheromatous emboli were not observed. Five patients had transmural neovascularization of the lesions in a similar manner to that described in coronary artery lesions. Obstructive lesions due to fibromuscular intimal proliferation with associated thrombosis and/or distal thromboembolization affecting arteries of hands and digits appear to be an important lesion that can affect young patients.

Antinuclear antibodies (ANA) and nailfold capillary microscopy have been evaluated as prognostic markers in patients with apparently idiopathic Raynaud's phenomenon. Results have shown that either ANA and peculiar alterations in nailfold capillaries are important risk factors as regard to the evolution into a connective tissue disease; furthermore, when both these markers are present in a patient with Raynaud's phenomenon, the risk of evolution is even greater. When sensibility and specificity have been compared, ANA appeared to be a more sensible test but nailfold capillary microscopy seemed to be more specific. Our data suggest that ANA and capillary microscopy are important tests in patients with Raynaud's phenomenon; they should be performed in order to identify those patients who are at greater risk of evolving to overt connective tissue disease.

A report of a case of long-standing Scleroderma (Progressive Systemic Sclerosis) is presented to emphasize the severe consequences of vascular compromise in the hand. The disease process is reviewed and is illustrated by the arteriographic findings in this patient.

A method for performing capillary microscopy using an ophthalmoscope is described. Using this technic, we examined 130 patients with various connective tissue diseases. As in prior studies, four patterns could be discerned; scleroderma (SD) pattern, lupus erythematosus (LE) pattern, normal pattern, and a nonspecific pattern. Of the patients with systemic SD, 70% had SD pattern. Of LE patients, 53% had LE pattern. There was little crossover. It is hoped that the use of the ophthalmoscope will result in the widespread use of capillary microscopy.

The ultrastructural changes in the vessels of the fingertips of patients with Raynaud's phenomenon or disease, with or without scleroderma, are described for the first time. Fingertip specimens were taken by punch biopsy. The light microscopic changes, including segmental vasculitis, fibrinoid degeneration of capillaries, and involuntary regression of glomus bodies explain the reduced digital blood flow associated with Raynaud's disease and phenomenon. The moment-to-moment digital blood flow, recorded rheoplethysmographically, was low when the patient was in a comfortable environment and was increased in a hot environment. This result substantiates the therapeutic importance of reflex vasodilatation. These findings correlate well with the ultrastructural changes in capillaries, including multiple cytoplasmic folds in endothelial cells, abundant intracytoplasmic filaments, and unusual incorporation of collagen fibrils in the basement membrane. These changes must influence nutritional and thermoregulatory function of the digital blood vessels.

Finger pulp biopsies of 31 patients suffering from Raynaud's syndrome (RS) were investigated by light and electron microscopy. The patients suffered from various underlying diseases. Interest was focused on the vascular changes and nerve and connective tissue alterations, especially the elastic fiber changes. In the dermal capillaries, endothelial swelling, narrowing of the lumen, increased numbers of intracytoplasmic filaments and Weibel-Palade bodies, and various types of basement membrane alterations were observed. The basement membrane of the unmyelinated nerves showed similar alterations to the capillary basement membrane. Severe degenerative changes were found in the myelin sheath of the myelinated axons. In the dermal connective tissue, severe elastotic degeneration and alterations of the collagen fibers were observed. The data probably represent the ultrastructural picture of the end stage of this syndrome.

Lesion biopsies of 60 fingers from 30 patients with vibration-induced white finger (VWF) and control observations from seven fingers of five referents demonstrated three main characteristic pathological changes. First, in each case, the muscular layers of the arteries revealed intense thickening with strong hypertrophy of individual muscle cells without intimal fibrosis. Periarterial fibrosis was also noted. Arteriosclerosis with foamy cells, lipid deposition, and fibrous sclerosis was occasionally observed. The second main change was demyelinating neuropathy in the peripheral nerves in which a marked loss of nerve fibers had occurred. There was also an increase in the number of Schwann cells and fibroblasts with strong collagen formation. Severe loss of myelin sheath frequently occurred, and relatively smaller axons without myelin which appeared to have regenerated were observed. Perineural fibrosis was also noted. In the third main pathological change connective tissues with collagen were increased not only in the perivascular and perineural lesions, but also abundantly in the corium of the skin. The elastic fibers there had been destroyed. These three principal pathological changes are very useful for the histopathological diagnosis of VWF.

Digital blood flow was assessed by photoplethysmography, ultrasonic arterial flow tracing and measurement of systolic blood pressure, and compared to nailfold capillary microscopy in 20 normal controls, 16 patients with primary Raynaud's phenomenon, 40 with undifferentiated connective tissue disease and 30 with systemic sclerosis. All 4 measurements showed significant differences between controls, Raynaud's phenomenon and systemic sclerosis but only capillaroscopy (mean number of enlarged capillary loops and avascular score) was able to differentiate between primary Raynauds and undifferentiated connective tissue disease. Capillaroscopy (mean numbers of enlarged capillary loops) was the most sensitive (100%) and specific (81%) test with a positive predictive value of 90% for systemic sclerosis.

A simple questionnaire, combined with color charts consisting of a 12-point color scale and a series of photographs illustrating blanching and cyanosis, has been developed to assist in the diagnosis of Raynaud's phenomenon (RP). The value of the color charts alone was first tested on a group of 48 patients with RP in whom the RP attacks were witnessed by the investigators (gold standard) and on a group of 246 RP negative subjects; the results showed a sensitivity of 90% and a specificity of 100%. A short RP diagnostic test was then devised by combining the questionnaire and color chart responses, which increased the sensitivity to 100% in our gold standard group of RP positive subjects. Further evaluation of this RP diagnostic test on a sample of 325 subjects whose RP status was unknown demonstrated that the majority could be classified as clearly RP positive or negative with only 4 subjects in the intermediate category.

A finger biopsy was performed on three patients with vibration-induced white finger (VWF), and the specimens were examined by electron microscopy for peripheral nerve changes. A characteristic neuropathy with perineurial fibrosis was revealed which was often accompanied by a thickened perineurium with a lamellar structure resembling onion skin. This peculiar fibrosis consisted of elongated cytoplasmic projections of perineurial cells or fibroblasts and a greatly increased amount of collagen which occasionally contained fibrous long-spacing collagen. In the endoneurium, a decrease in the number of nerve fibers and a marked increase in collagen with fibroblasts were noted. Myelinated axons became smaller, and this occurrence suggested incomplete regeneration. The pathological changes were presumably the result of the long-term clinical course of VWF.