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Raynaud's Phenomenon (raynaud's + phenomenon)
Selected AbstractsAre Migraine and Coronary Heart Disease Associated?HEADACHE, Issue 2004An Epidemiologic Review In evaluating the cardiovascular risks of triptans (5-HT1B/1D agonists) for the treatment of migraine, the possible relationship between migraine and cardiovascular disease warrants careful assessment. The vascular nature of migraine is compatible with the possibility that migraine is a manifestation of cardiovascular disease or is linked to cardiovascular disease via a common mechanism. If so, then migraine itself,independent of the use of triptans,may be associated with an increased risk of cardiac events. This article considers the epidemiologic literature pertinent to evaluating the association of migraine with coronary heart disease. The research reviewed herein fails to support an association between migraine and coronary heart disease. First, data from several large cohort studies show that the presence of migraine does not increase risk of coronary heart disease. Furthermore, although migraineurs are generally more likely than nonmigraineurs to report chest pain, the presence of chest pain in most studies did not predict serious cardiac events such as myocardial infarction. That the gender- and age-specific prevalence of migraine does not overlap with that of coronary heart disease is also consistent with a lack of association between migraine and atherosclerotic cardiovascular disease. While migraine appears not to be associated with coronary heart disease, preliminary evidence suggests a possible link of migraine with vasospastic disorders such as variant angina and Raynaud's phenomenon. These results warrant further investigation in large prospective studies. [source] Anorexia nervosa and Raynaud's phenomenon: A case reportINTERNATIONAL JOURNAL OF EATING DISORDERS, Issue 8 2007Basak Yucel MD Abstract Objective: To describe and discuss potential relationships between anorexia nervosa (AN) and Raynaud's phenomenon, the course and concurrent treatment of these two disorders as they appeared simultaneously, and a potential treatment modification entailed in such concurrent therapies. Background: Although Raynaud's phenomenon has been described during the course of AN, the associations and interactions between these two conditions are not clear. Method: We report the medical workup, treatment, and outcomes in a 19-year old female patient who developed Raynaud's phenomenon following the onset of AN. Results: After treatment with nutritional rehabilitation, counseling, and individual and group therapy, the patient's weight, eating disorder-related behaviors, and attitudes improved significantly. Raynaud's related symptoms improved, following treatment with a calcium channel blocker and antiaggregant therapy. In conjunction with nutritional efforts to treat the patient's long-standing amenorrhea and osteopenia, the treatment team elected to also administer estrogen hormone in addition to oral calcium and vitamin D supplementation. Since oral contraceptives are to be avoided in patients with Raynaud's phenomenon who show clinical findings suggesting connective tissue disorder, the treatment team elected to treat this patient with transdermal hormone replacement therapy. Conclusion: The co-occurrence of AN and Raynaud's phenomenon merits close and persistent follow-up by a multidisciplinary team and may lead to alterations of usual therapeutic approaches. © 2007 by Wiley Periodicals, Inc. [source] The cutaneous pathology of lupus erythematosus: a reviewJOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2001A. Neil Crowson The presentation of lupus erythematosus (LE) ranges from a skin rash unaccompanied by extracutaneous stigmata to a rapidly progressive lethal multiorgan disease. The diagnosis and subclassification is traditionally based on the correlation of serological and clinical findings. The latter include a photoinduced skin rash, arthralgia, arthritis, fever, Raynaud's phenomenon, anemia, leukopenia, serositis, nephritis and central nervous sysdtem disease. The conventional classification scheme includes systemic, subacute cutaneous and discoid LE. Recent advances in our understanding of the cutaneous histopathology which correlates with the traditional forms of LE, along with certain novel LE subtypes, are the focus of this review. In addition to the main subtypes of LE, we will discuss associated vasculopathic lesions and the contribution of immunofluorescence microscopy to the diagnosis of LE and related connective tissue disease syndromes. Consideration will be given to unusual variants of LE such as anti-Ro/SSA-positive systemic lupus erythematosus (SLE), bullous SLE, lymphomatoid LE, lupus erythematosus profundus, drug induced LE, linear cutaneous LE, chiblains LE and parvovirus B19-associated LE. [source] Digital gangrene: a rare skin symptom in systemic lupus erythematosusJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 5 2000E Vocks Abstract A case of digital gangrene in a patient with systemic lupus erythematosus without secondary antiphospholipid syndrome is reported. The acute onset of the necrotizing acrovasculitis occurred without a history of Raynaud's phenomenon. Preceding symptoms of the systemic lupus erythematosus were arthritis, photosensitivity, alopecia and anorexia. Despite the seriousness of the acute episode the patient achieved an excellent outcome. [source] Raynaud's phenomenon in a child presenting as oxygen desaturation during transfusion with cold bloodPEDIATRIC ANESTHESIA, Issue 12 2008XIAOPENG ZHANG MD Summary We report a case of Raynaud's phenomenon (RP) triggered by transfusion of cold blood to a pediatric burn patient under general anesthesia. The child was febrile so a decision was made to not use a blood warmer. When the blood was rapidly administered the child suddenly developed ,desaturation'. The child was placed on 100% oxygen, adequate ventilation assured, and the color of his oral mucosa assessed as ,pink'. Placement of the oximeter on the opposite hand revealed 100% saturation. To our knowledge, this is the first case of apparent RP reported in a pediatric patient triggered by transfusion of cold blood. [source] Major colorectal surgery in a patient with cold agglutinin diseaseANAESTHESIA, Issue 6 2006S. Young Summary We present the case of a 62-year-old man with severe cold agglutinin disease who underwent major colorectal surgery. Cold agglutinin disease is a condition in which auto-antibodies, usually immunoglobulin M, cause red blood cell agglutination at decreased body temperature. Haemolysis may result. Agglutination results in impaired perfusion, resulting in symptomatic Raynaud's phenomenon and acrocyanosis. Haemolysis can result in anaemia and thrombotic events caused by microvascular occlusion, in addition to haemoglobinuria and renal failure. Peri-operative hypothermia is common in all patients and may be associated with significant morbidity, but is potentially catastrophic in a patient suffering from cold agglutinin disease. [source] Clinical implications from capillaroscopic analysis in patients with Raynaud's phenomenon and systemic sclerosisARTHRITIS & RHEUMATISM, Issue 9 2010Ariane L. Herrick First page of article [source] Heritability of vasculopathy, autoimmune disease, and fibrosis in systemic sclerosis: A population-based studyARTHRITIS & RHEUMATISM, Issue 7 2010Tracy Frech Objective To investigate the familiality of systemic sclerosis (SSc) in relation to Raynaud's phenomenon (RP) (a marker of vasculopathy), other autoimmune inflammatory disease, and fibrotic interstitial lung disease (ILD). Methods A genealogic resource, the Utah Population Database (UPDB), was used to test heritability of RP, other autoimmune disease, and ILD. Diseases were defined by International Classification of Diseases, Ninth Revision codes and identified from statewide discharge data, the University of Utah Health Science Center Enterprise Data Warehouse, and death certificates and were linked to the UPDB for analysis. Familial standardized incidence ratio (FSIR), relative risks (RRs) to first-, second-, third-, and fourth-degree relatives for SSc, RP, other autoimmune disease, and ILD (with 95% confidence intervals [95% CIs]), and population attributable risk (PAR) were calculated. Results A software kinship analysis tool was used to analyze 1,037 unique SSc patients. Fifty SSc families had significant FSIRs, ranging from 2.07 to 17.60. The adjusted PAR was ,8%. The RRs were significant for other autoimmune disease in the first-degree relatives (2.49 [95% CI 1.99,3.41], P = 2.42 × 10,15) and second-degree relatives (1.48 [95% CI 1.34,2.39], P = 0.002), for RP in first-degree relatives (6.38 [95% CI 3.44,11.83], P = 4.04 × 10,9) and second-degree relatives (2.39 [95% CI 1.21,4.74], P = 0.012), and for ILD in first-degree relatives (1.53 [95% CI 1.04,2.26], P = 0.03), third-degree relatives (1.47 [95% CI 1.18,1.82], P = 0.0004), and fourth-degree relatives (1.2 [95% CI 1.06,1.35], P = 0.004). Conclusion These data suggest that SSc pedigrees include more RP, autoimmune inflammatory disease, and ILD than would be expected by chance. In SSc pedigrees, genetic predisposition to vasculopathy is the most frequent risk among first-degree relatives. [source] Course of the modified Rodnan skin thickness score in systemic sclerosis clinical trials: Analysis of three large multicenter, double-blind, randomized controlled trialsARTHRITIS & RHEUMATISM, Issue 8 2009Sogol Amjadi Objective To assess the course of the modified Rodnan skin thickness score (MRSS) in 3 large, multicenter, double-blind, randomized controlled trials (RCTs) of patients with diffuse cutaneous systemic sclerosis (dcSSc) with different baseline disease durations, as defined from the date of onset of the first dcSSc symptom (excluding Raynaud's phenomenon) or from the date of onset of the first dcSSc-related symptom (including Raynaud's phenomenon). Methods Data from 3 RCTs examining high-dose versus low-dose D-penicillamine (D-Pen Trial), recombinant human relaxin versus placebo (Relaxin Trial), and oral bovine type I collagen versus placebo (Collagen Trial) treatment in patients with dcSSc were pooled and analyzed. Patients were divided into 5 groups according to their disease duration at baseline. The linear mixed model for correlated data was used to model the 2 predictors of MRSS: time in study (expressed in months after baseline) and baseline disease duration (expressed in months, calculated from the date of onset of the first symptom characteristic of dcSSc with and without Raynaud's phenomenon). Results At study entry, the mean MRSS value was 21.0 in the D-Pen Trial cohort, 27.3 in the Relaxin Trial cohort, and 26.1 in the Collagen Trial cohort. Time in study was a significant predictor of improvement in MRSS regardless of the disease duration at baseline (P < 0.0001). Patients with a disease duration of ,24 months showed a greater rate of decline as compared with patients with a disease duration of <24 months (P < 0.05). Similar results were obtained when disease duration was reclassified by including the time of the first Raynaud's phenomenon symptom in the definition. Conclusion Our study confirms recent findings that in patients entered into these 3 RCTs, skin thickening did not follow the same trend in natural history as that seen in the dcSSc populations entered into early, open longitudinal studies previously reported. These findings have important implications for study design, in which "prevention of worsening" is the main objective. [source] MQX-503, a novel formulation of nitroglycerin, improves the severity of Raynaud's phenomenon: A randomized, controlled trial,ARTHRITIS & RHEUMATISM, Issue 3 2009Lorinda Chung Objective Raynaud's phenomenon (RP) affects 3,9% of the general population and >90% of patients with systemic sclerosis. Nitrates are often prescribed for the treatment of RP, but currently available formulations are limited by side effects, particularly headaches, dizziness, and skin irritation. The purpose of this study was to evaluate the tolerability and efficacy of a novel formulation of topical nitroglycerin, MQX-503, in the treatment of RP in an ambulatory setting. Methods We conducted a multicenter, randomized, placebo-controlled study with a 2-week single-blind run-in period to determine baseline severity, followed by a 4-week double-blind treatment phase. Two hundred nineteen adult patients with a clinical diagnosis of primary or secondary RP received 0.9% MQX-503 gel or matching placebo during the treatment period. Gel was applied immediately before or within 5 minutes of the beginning of an episode of RP (maximum of 4 applications daily). End points included the change in the mean Raynaud's Condition Score (RCS; scale 0,10), the frequency and duration of episodes, and subjective assessments at the target week (the week during the treatment phase that most closely matched the run-in period in terms of ambient temperature) compared with baseline. Results The mean (%) change in the RCS at the target week compared with baseline was significantly greater in the MQX-503 group (0.48 [14.3%]) than that in the placebo group (0.04 [1.3%]; P = 0.04). Changes in the frequency and duration of RP episodes and subjective assessments were not statistically different between the groups. MQX-503 had a side effect profile similar to that of placebo. Conclusion MQX-503 is well tolerated and more effective than placebo for the treatment of RP. [source] Identification of transition from primary Raynaud's phenomenon to secondary Raynaud's phenomenon by nailfold videocapillaroscopy: Comment on the article by Hirschl et alARTHRITIS & RHEUMATISM, Issue 6 2007Maurizio Cutolo MD No abstract is available for this article. [source] Increase in circulating endothelial precursors by atorvastatin in patients with systemic sclerosisARTHRITIS & RHEUMATISM, Issue 6 2006Masataka Kuwana Objective To evaluate whether atorvastatin can increase bone marrow,derived circulating endothelial precursors (CEPs) and improve the vascular symptoms in patients with systemic sclerosis (SSc; scleroderma). Methods The study was designed as an open-label, prospective study involving 14 patients with SSc who received 10 mg/day of atorvastatin for 12 weeks and were followed up for the subsequent 4 weeks. CEPs were quantified at weeks 0 (pretreatment), 4, 8, 12 (during treatment), and 16 (posttreatment) by cell sorting followed by 3-color flow cytometry. Raynaud's phenomenon variables, global measures, and psychological scales as well as circulating angiogenic factors and endothelial activation/injury markers were serially assessed. The potential of CEPs to differentiate into mature endothelial cells was examined in cultures with angiogenic stimuli. Results None of the patients experienced an adverse event, but 1 dropped out because of an excessive decrease in serum total cholesterol. Atorvastatin treatment resulted in a 1.7- to 8.0-fold increase in CEPs from baseline levels (P < 0.0001), but the numbers returned to within baseline levels at posttreatment. However, 8 patients (62%) experienced a gradual decrease in the number of CEPs, even while taking atorvastatin. Variables indicating the extent of Raynaud's phenomenon improved significantly, and up-regulated levels of angiogenic factors and vascular endothelial activation/injury markers decreased significantly during atorvastatin treatment. These variables returned to within baseline levels after discontinuation of the drug. In contrast, atorvastatin failed to improve the in vitro maturation potential of CEPs. Conclusion The results of this pilot study suggest that atorvastatin treatment can increase CEPs and may be effective in improving Raynaud's phenomenon, even in SSc patients who have CEP dysfunction intrinsically. [source] Clinical study of hereditary disorders of connective tissues in a Chilean populationARTHRITIS & RHEUMATISM, Issue 2 2006Joint hypermobility syndrome, vascular Ehlers-Danlos syndrome Objective To demonstrate the high frequency and lack of diagnosis of joint hypermobility syndrome (JHS) and the seriousness of vascular Ehlers-Danlos syndrome (VEDS). Methods Two hundred forty-nine Chilean patients with hereditary disorders of the connective tissues (CTDs) and 64 control subjects were evaluated for the diagnoses of JHS and VEDS using the validated Brighton criteria, as compared with the traditional Beighton score. In addition, the presence of blue sclera was determined, with the degree of intensity graded as mild, moderate, or marked. Results The frequency of hereditary CTDs was 35%, with diagnoses of JHS in 92.4% of subjects, VEDS in 7.2%, and osteogenesis imperfecta in 0.4%. The Beighton score proved to be insufficient for the diagnosis of JHS (35% of subjects had a negative score), whereas the Brighton criteria yielded positive findings (a diagnosis of JHS) in 39% of control subjects. Blue sclera was frequent, being identified in 97% of JHS patients and 94% of VEDS patients. Moderate osteopenia/osteoporosis was observed in 50% of patients with VEDS and 26% of those with JHS. Dysautonomia, dyslipidemia, and scoliosis were more frequent in VEDS patients than in JHS patients. The typical JHS facial appearance and the "hand holding the head sign" were identified. Raynaud's phenomenon was extremely rare in JHS patients (2%). Ruptured uterus and cerebral aneurysm occurred in 12% and 6% of VEDS patients, respectively. Spontaneous pneumothorax was more frequent in VEDS patients (11%) than in JHS patients (0.9%). Conclusion JHS is very frequent but usually undiagnosed. The Beighton score is an insufficient method for JHS diagnosis. We recommend that physicians learn to recognize the typical facial features of JHS and be able to identify blue sclera. We also propose that validated hypermobility criteria be routinely used. Further research is needed to determine why the prevalence of JHS is so high in Chile. [source] Increased asymmetric dimethylarginine and endothelin 1 levels in secondary Raynaud's phenomenon: Implications for vascular dysfunction and progression of diseaseARTHRITIS & RHEUMATISM, Issue 7 2003Sanjay Rajagopalan Objective To compare microvascular and macrovascular functions in a cohort of patients with primary and secondary Raynaud's phenomenon (RP) who were matched for demographic, risk factor, and severity profiles. Methods Forty patients with primary or secondary RP matched for vascular risk factors and severity scores underwent testing of endothelial function and cold pressor responsiveness of the brachial artery. Microvascular perfusion of the digital vasculature was assessed using laser Doppler fluxmetry in response to reactive hyperemia. Plasma was assayed for endothelin 1 (ET-1), asymmetric dimethylarginine (ADMA), intercellular adhesion molecule 1, vascular cell adhesion molecule 1 (VCAM-1), and monocyte chemoattractant protein 1 (MCP-1). Results Patients with RP had abnormal vasoconstrictor responses to cold pressor tests (CPT) that were similar in primary and secondary RP. There were no differences in median flow-mediated and nitroglycerin-mediated dilation or CPT of the brachial artery in the 2 populations. Patients with secondary RP were characterized by abnormalities in microvascular responses to reactive hyperemia, with a reduction in area under the curve adjusted for baseline perfusion, but not in time to peak response or peak perfusion ratio. Plasma ET-1, ADMA, VCAM-1, and MCP-1 levels were significantly elevated in secondary RP compared with primary RP. There was a significant negative correlation between ET-1 and ADMA values and measures of microvascular perfusion but not macrovascular endothelial function. Conclusion Secondary RP is characterized by elevations in plasma ET-1 and ADMA levels that may contribute to alterations in cutaneous microvascular function. [source] Measuring disease activity and functional status in patients with scleroderma and Raynaud's phenomenonARTHRITIS & RHEUMATISM, Issue 9 2002Peter A. Merkel Objective To document disease activity and functional status in patients with scleroderma (systemic sclerosis [SSc]) and Raynaud's phenomenon (RP) and to determine the sensitivity to change, reliability, ease of use, and validity of various outcome measures in these patients. Methods Patients with SSc and moderate-to-severe RP participating in a multicenter RP treatment trial completed daily diaries documenting the frequency and duration of RP attacks and recorded a daily Raynaud's Condition Score (RCS). Mean scores for the 2-week periods prior to baseline (week 0), end of trial (week 6), and posttrial followup (week 12) were calculated. At weeks 0, 6, and 12, physicians completed 3 global assessment scales and performed clinical assessments of digital ulcers and infarcts; patients completed the Health Assessment Questionnaire (HAQ), the Arthritis Impact Measurement Scales 2 (AIMS2) mood and tension subscales, 5 specific SSc/RP-related visual analog scales (VAS), and 3 other VAS global assessments. We used these measures to document baseline disease activity and to assess their construct validity, sensitivity to change, and reliability in trial data. Results Two hundred eighty-one patients (248 women, 33 men; mean age 50.4 years [range 18,82 years]) from 14 centers participated. Forty-eight percent had limited cutaneous SSc; 52% had diffuse cutaneous SSc. Fifty-nine patients (21%) had digital ulcers at baseline. Patients had 3.89 ± 2.33 (mean ± SD) daily RP attacks (range 0.8,14.6), with a duration of 82.1 ± 91.6 minutes/attack. RCS for RP activity (possible range 0,10) was 4.30 ± 1.92. HAQ scores (0,3 scale) indicated substantial disability at baseline (total disability 0.86, pain 1.19), especially among the subscales pertaining to hand function (grip, eating, dressing). AIMS2 mood and tension scores were fairly high, as were many of the VAS scores. Patients with digital ulcers had worse RCS, pain, HAQ disability (overall, grip, eating, and dressing), physician's global assessment, and tension, but no significant difference in the frequency of RP, duration of RP, patient's global assessment, or mood, compared with patients without digital ulcers. VAS scores for digital ulcers as rated by the patients were not consistent with the physician's ratings. Factor analysis of the 18 measures showed strong associations among variables in 4 distinct domains: disease activity, RP measures, digital ulcer measures, and mood/tension. Reliability of the RCS, HAQ pain and disability scales, and AIMS2 mood and tension subscales was high. The RP measures demonstrated good sensitivity to change (effect sizes 0.33,0.76). Conclusion Our findings demonstrate that the significant activity, disability, pain, and psychological impact of RP and digital ulcers in SSc can be measured by a small set of valid and reliable outcome measures. These outcome measures provide information beyond the quantitative metrics of RP attacks. We propose a core set of measures for use in clinical trials of RP in SSc patients that includes the RCS, patient and physician VAS ratings of RP activity, a digital ulcer/infarct measure, measures of disability and pain (HAQ), and measures of psychological function (AIMS2). [source] The diagnostic quandary of hereditary haemorrhagic telangiectasia vs.BRITISH JOURNAL OF DERMATOLOGY, Issue 4 2001CREST syndrome The distribution and clinical appearance of the telangiectasia in the CREST syndrome (calcinosis, Raynaud's phenomenon, oesophageal involvement, sclerodactyly, telangiectasia) and hereditary haemorrhagic telangiectasia (HHT) are very similar. Several previously reported cases of the CREST syndrome simulating HHT illustrate this diagnostic quandary. We report a patient who met the diagnostic criteria for both the CREST syndrome and HHT, and discuss the distinguishing features of the two diseases, including the distinctive histopathological findings of telangiectasia in HHT. [source] A case of porphyria cutanea tarda in association with idiopathic myelofibrosis and CREST syndromeBRITISH JOURNAL OF DERMATOLOGY, Issue 1 2001S.C. Lee We report a 56-year-old Korean woman with porphyria cutanea tarda (PCT), showing multiple scarring bullae and hypertrichosis on sun-exposed areas of skin with postinflammatory hyperpigmentation. Sclerodermoid changes were also found on both hands, the face and neck. The patient had suffered from CREST syndrome, manifesting with Raynaud's phenomenon and sclerodactyly, for more than 15 years. Anticentromere antibody was positive. She had presented with splenomegaly 3 years before the development of PCT, and was diagnosed as having idiopathic myelofibrosis, based on bone marrow biopsy. In summary, she had had CREST syndrome for 15 years and later developed idiopathic myelofibrosis and PCT. This is the first reported case of PCT in association with idiopathic myelofibrosis and CREST syndrome. [source] Hypereosinophilic syndrome presenting as cutaneous necrotizing eosinophilic vasculitis and Raynaud's phenomenon complicated by digital gangreneBRITISH JOURNAL OF DERMATOLOGY, Issue 3 2000K-A. Jang Cutaneous necrotizing eosinophilic vasculitis is a recently identified type of vasculitis that is characterized by an eosinophil-predominant necrotizing vasculitis affecting small dermal vessels. Clinically, it presents with pruritic erythematous and purpuric papules and plaques, peripheral eosinophilia and a good response to systemic steroid therapy. This vasculitis can be idiopathic or associated with connective tissue diseases. Although the pathogenic roles of eosinophil-derived granule proteins and interleukins have been documented in diseases associated with eosinophilia, a role of CD40 (a glycoprotein of the tumour necrosis factor receptor superfamily) has rarely been described. We describe two patients with idiopathic hypereosinophilic syndrome (HES) presenting with multiple erythematous patches and plaques on the lower extremities and Raynaud's phenomenon. They satisfied the criteria for the diagnosis of HES by clinical and laboratory investigations. Histopathology of the cutaneous lesions revealed prominent eosinophilic infiltration with local fibrinoid change in vessel walls in the dermis and subcutis. Immunohistochemical detection of CD3, CD4, CD8 and CD40 was performed. Infiltrating eosinophils were strongly stained by anti-CD40 monoclonal antibody. One patient improved with prednisolone, pentoxifylline and nifedipine, without recurrence. The other patient initially improved with steroids, but after self-withdrawal of steroid developed digital ischaemia that evolved to severe necrosis and required amputation. Cutaneous necrotizing eosinophilic vasculitis, Raynaud's phenomenon and digital gangrene may develop as cutaneous manifestations of HES. CD40 may play a part in the pathogenesis of eosinophilic vasculitis in HES. [source] Systemic disease associations of familial and sporadic glaucoma: the Glaucoma Inheritance Study in TasmaniaACTA OPHTHALMOLOGICA, Issue 1 2010Alex W. Hewitt Abstract. Background:, This aim of this study was to compare the prevalence of various disease-associated and potentially modifiable risk factors between people with familial and sporadic forms of primary open angle glaucoma (OAG). Methods:, A cross-sectional, retrospective study design was utilized. A detailed questionnaire enquiring about knowledge of family history, demographic data, current medications, and medical history of systemic disorders was administered. Where possible, living relatives were examined for signs of OAG. Results:, A total of 3,800 potential patients with OAG were identified, of whom 2062 were examined. One thousand twelve (59.5%) subjects were found to have familial OAG, and 688 (40.5%) subjects had no known or identified relative with OAG (sporadic glaucoma). One thousand forty-two unaffected family members examined. A past history of migraine was found more often with familial OAG (OR: 1.67 95% CI: 1.15,2.42). This effect was primarily driven by patients who had a first-degree relative also affected by OAG. Following adjustment for male gender and the age at review, the presence of atherosclerosis was also found to be more common in patients with familial glaucoma than in people with sporadic disease (OR: 1.42 95% CI: 1.05,1.92). No significant difference in the prevalence of hypertension, Raynaud's phenomenon, diabetes mellitus or thyroid disease was identified. Conclusions:, Patients with a known relative affected by OAG were statistically significantly more likely to have a past history for migraine or presence of atherosclerosis compared to people with no known affected relative. An understanding of such differences and systemic comorbidities will be useful for further work investigating the underlying molecular mechanisms of this disease. [source] Fluorescein-induced Raynaud's phenomenonACTA OPHTHALMOLOGICA, Issue 8 2007Pierre Blaise No abstract is available for this article. [source] Keratotic vascular papules over the feet: a case of Waldenström's macroglobulinaemia-associated cutaneous macroglobulinosisCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 3 2010M. Harnalikar Summary Waldenström's macroglobulinaemia (WM) is a plasma-cell dyscrasia characterized by the monoclonal proliferation of lymphoplasmacytes. A 48-year-old man presented with a 4-year history of multiple painful, hyperkeratotic deep-seated papules over the pressure areas of both soles. He had a 1-year history of Raynaud's phenomenon, intermittent epistaxis, recurrent vomiting, tingling and numbness, and visual disturbances. Histological examination of a skin biopsy found amyloid-like deposits in the upper and mid dermis involving dermal blood vessels, but apart from periodic-acid,Schiff, various stains gave negative results for amyloid. Direct immunofluorescence was positive for IgM antibody. Hence, a diagnosis of WM with cutaneous macroglobulinosis was made. Immunoelectrophoresis found monoclonal IgM kappa antibody, and bone-marrow examination revealed a lymphoplasmacytoid malignancy. The patient's systemic systems were attributed to hyperviscosity syndrome associated with WM and the cutaneous papules were identified as deposits of excess IgM antibodies. The patient received five cycles of chemotherapy, resulting in nearly complete resolution of the skin lesions and systemic symptoms. [source] Hypereosinophilic syndrome with various skin lesions and juvenile temporal arteritisCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 5 2009K. Ito Summary Hypereosinophilic syndrome (HES) is a multisystem disease with a high mortality rate. It is characterized by peripheral blood eosinophilia and eosinophilic infiltration of the skin and many other organs. The commonest cutaneous features include erythematous pruritic maculopapules and nodules, angio-oedema or urticarial plaques. However, some case reports have indicated that eosinophilic cellulitis, cutaneous necrotizing eosinophilic vasculitis, Raynaud's phenomenon and digital gangrene may also occur as cutaneous features of HES. Juvenile temporal arteritis (JTA) of unknown cause is characterized by an asymptomatic nodule in the temporal artery area in young adults. Histologically, the lesion is characterized by a significant intimal thickening with moderate eosinophilic infiltrates, constriction or occlusion of the vascular lumen and absence of giant cells. We report a patient with HES presenting with eosinophilic cellulitis, Raynaud's phenomenon, digital gangrene and JTA. JTA may also be one of the features of HES. [source] A review of the cutaneous paraneoplastic associations and metastatic presentations of ovarian carcinomaCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 1 2008N. Scheinfeld Summary Ovarian carcinoma possesses cutaneous and paraneoplastic associations. The aim of this study was to review the paraneoplastic associations and metastatic presentations of ovarian carcinoma. PubMed was searched through December 2006 for references to cutaneous metastatic ovarian carcinoma (CMOC). CMOC occurs in 2,7% of cases, manifests in advanced disease and indicates a poor prognosis. The paraneoplastic associations of ovarian carcinoma include acanthosis nigricans, Raynaud's phenomenon, scleroderma, dermatomyositis and palmar fasciitis with polyarthritis. Dermatomyositis, in particular, can precede the diagnosis of ovarian carcinoma. Ovarian carcinoma has many cutaneous paraneoplastic effects and metastatic presentations, all of which portend a poor prognosis. Dermatomyositis is sometimes the initial manifestation of ovarian cancer, thus women > 40 years of age with dermatomyositis should be checked for ovarian carcinoma. It is possible that paraneoplastic dermtomyosititis can be distinguished from nonparaneoplastic dermatomyostitis by the former's lack of (i) associated Raynaud's phenomenon, (ii) response to treatment, (iii) autoantibodies, (iv) overlap and association with other collagen vascular diseases and (v) the presence of the prodromal symptoms of ovarian carcinoma such as gastrointestinal symptoms, urinary symptoms and/or fatigue or malaise. [source] Infrared-monitored cold response in the assessment of Raynaud's phenomenonCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 1 2006J. Foerster Summary Background., Evaluation of treatments for Raynaud's phenomenon (RP) requires objective response parameters in addition to clinical activity scores. Thermographic monitoring of fingertip re-warming after cold challenge has been widely used but usually requires sophisticated equipment. We have previously shown that fingertip re-warming after cold challenge follows a first-order transient response curve that can be described by a single variable, designated ,. Objectives., Here, we describe a novel device termed a duosensor, which records the , value upon cold challenge in an automated manner. Methods., We determined , values in healthy probands, patients with primary or secondary RP associated with autoimmune disease and patients with scleroderma-associated RP following cold challenge, to determine assay variability, sensitivity and specificity. Results., Duosensor-based thermography exhibited low intraindividual variability in healthy probands. As expected, , values in RP patients were significantly increased compared with controls (8.08 ± 3.65 min vs. 3.23 ± 1.65 min). The duosensor-determined , value yielded a specificity of 94.6% and predictive value of 95.3% for the presence of RP in a retrospective analysis of 139 patients. Furthermore, in a cohort of scleroderma patients with RP, patient self-assessment of RP severity correlated with , values. Conclusions., Taken together, the present data suggest that , value determination provides a suitable outcome measure for clinical studies of novel RP treatments. As the duosensor is a simple stand-alone device requiring no supporting equipment and minimal personnel attention, it should allow RP activity monitoring even in clinical settings with minimal technical infrastructure. [source] Systemic lupus erythematosus (SLE) presenting with nephrotic syndrome and membranous glomerulopathy in a 10-year-old girlACTA PAEDIATRICA, Issue 10 2005Vânia Machado Abstract The authors report the case of a 10-y-old girl with clinical diagnosis of systemic lupus erythematosus (SLE), made at the age of 6 y, based upon arthritis, serositis, haematological disorder and positive antinuclear antibody. The first manifestation of disease,Raynaud's phenomenon,appeared at the age of 4 y. Seven months after the diagnosis, she developed nephrotic proteinuria with haematuria. Percutaneous renal biopsy showed membranous glomerulonephritis, the least common form of lupus nephritis. Conclusion: Intravenous cyclophosphamide therapy associated with oral prednisolone proved effective in inducing complete remission of nephrotic syndrome. [source] | ||||||||||||||||||||||