The Place of Nailfold Capillaroscopy Among Instrumental Methods for Assessment of Some Peripheral Ischaemic Syndromes in Rheumatology Sevdalina N

Total Page:16

File Type:pdf, Size:1020Kb

The Place of Nailfold Capillaroscopy Among Instrumental Methods for Assessment of Some Peripheral Ischaemic Syndromes in Rheumatology Sevdalina N DOI: 10.1515/folmed-2016-0011 REVIEW The Place of Nailfold Capillaroscopy Among Instrumental Methods for Assessment of Some Peripheral Ischaemic Syndromes in Rheumatology Sevdalina N. Lambova Department of Propedeutics of Internal Medicine, Faculty of Medicine, Medical University of Plovdiv, Plovdiv, Bulgaria Correspondence: Sevdalina Lam- Micro- and macrovascular pathology is a frequent fi nding in a number of common bova, Department of Propedeutics rheumatic diseases. Secondary Raynaud’s phenomenon (RP) is among the most in Internal Medicine, Faculty of common symptoms in systemic sclerosis and several other systemic autoimmune Medicine, Medical University of diseases including a broad diff erential diagnosis. It should be also diff erentiated Plovdiv, Plovdiv, 15A Vassil Aprilov from other peripheral vascular syndromes such as embolism, thrombosis, etc., Blvd., 4002 Plovdiv, Bulgaria E-mail: [email protected] some of which lead to clinical manifestation of the blue toe syndrome. Tel: +359 889 560 104 The current review discusses the instrumental methods for vascular assessments. Received: 9 March 2016 Nailfold capillaroscopy is the only method among the imaging techniques that Accepted: 4 May 2016 can be used for morphological assessment of the nutritive capillaries in the nail- Published: 30 June 2016 fold area. Laser-Doppler fl owmetry and laser-Doppler imaging are methods for Key words: Raynaud’s phenome- functional assessment of microcirculation, while thermography and plethysmog- non, blue toe syndrome, rheu- raphy refl ect both blood fl ow in peripheral arteries and microcirculation. Doppler matic diseases, microcirculation; ultrasound and angiography visualize peripheral arteries. The choice of the appro- nailfold capillaroscopy priate instrumental method is guided by the clinical presentation. The main role of Citation: Lambova SN. The capillaroscopy is to provide diff erential diagnosis between primary and secondary Place of Nailfold Capillaroscopy RP. In rheumatology, capillaroscopic changes in systemic sclerosis have been re- Among Instrumental Methods for cently defi ned as diagnostic. The appearance of abnormal capillaroscopic pattern Assessment of Some Peripheral inherits high positive predictive value for the development of a connective tis- Ischaemic Syndromes in Rheuma- sue disease that is higher than the predictive value of antinuclear antibodies. In tology. cases of abrupt onset of peripheral ischaemia, clinical signs of critical ischaemia, Folia Medica 2016;58(2);77-88, unilateral or lower limb involvement, Doppler ultrasound and angiography are doi: 10.1515/folmed-2016-0011 indicated. The most common causes for such clinical picture that may be referred to rheumatologic consultation are the antiphospholipid syndrome, mimickers of vasculitides such as atherosclerosis with cholesterol emboli, and neoplasms. INTRODUCTION RESULTS Micro- and macrovascular pathology is a common RAYNAUD’S PHENOMENON feature in a number of common rheumatic diseases. Raynaud’s phenomenon (RP) is among the most Reliable diagnostic tools should be used in order to common symptom in systemic autoimmune diseases. guide physicians in the differential diagnosis and It manifests as reversible vasospasm of the small administration of well-timed treatment that would peripheral arteries and arterioles at cold exposure or improve prognosis, slow up or prevent future dam- emotional stress. It presents in three or two phases age or provide full recovery in cases of medical e. g., ischaemia, asphyxia and reactive hyperemia emergency. with skin discolouration from pallor to cyanosis and redness with clearly visible demarcation line. It AIM affects acral body parts such as fi ngers, toes, nose, The present review discusses the instrumental ears, lips. RP could be idiopathic or primary, when methods for vascular assessment. Two case reports there is no underlying cause for its development and are presented to illustrate the possibilities of the the clinical course is benign without development of imaging modalities in clinical context. trophic changes because of the lack of endothelial 77 Folia Medica I 2016 I Vol. 58 I No. 2 I Article 1 S. Lambova damage. Secondary RP is observed in a number of to the higher use of different vascular interventions connective tissue diseases and includes a broad dif- such as vascular surgery or invasive percutaneous ferential diagnosis including drug-induced RP and procedures i.e., angiography and angioplasty. In paraneoplastic conditions. The profound endothelial addition, anticoagulant therapy could also induce damage may lead to appearance of digital ulcers this iatrogenic complications.6 in a proportion of cases with secondary RP.1-4 It A number of instrumental techniques are used should be also differentiated from other peripheral to assess microcirculation in patients with symp- vascular syndromes such as embolism, thrombosis, toms of RP in order to prove the presence of etc., some of which lead to clinical manifestation vascular pathological changes and to differentiate of the blue toe syndrome. A number of laboratory the primary from secondary forms of the disease and instrumental methods are essential for the dif- that is crucial for the therapeutic approach such ferential diagnosis. Interpretation of the fi ndings is as nailfold capillaroscopy, laser Doppler fl owmetry, in the concrete clinical context. laser Doppler imaging, thermography at rest and in RP is diagnosed clinically. The diagnosis is combination with cold provocation. Colour Doppler based on the direct observation of 2 among the 3 and angiography are additional instrumental tech- possible phases of the condition. To be diagnosed niques that visualize larger vessels and facilitate with RP, a patient should have a history of sensi- the differential diagnosis in more complex cases tivity to the cold and episodic pallor, cyanosis or of peripheral vascular ischaemic pathology. redness of the distal portions of the digits after Nailfold capillaroscopy is the only method for exposure to cold. Photographs of the hands may morphological assessment of nutritive capillaries. be obtained during an attack and used to confi rm Laser Doppler and thermography assess cutaneous the history. In routine clinical practice it is not blood vessel function.7 necessary to perform a cold provocation test to RAYNAUD’S PHENOMENON IN RHEUMATIC DISEASES make a defi nitive diagnosis of RP.5 RP is a characteristic feature in a number of rheu- BLUE TOE SYNDROME matic diseases with substantially high frequency in The term ‘blue toe syndrome’ describes the devel- systemic sclerosis (SSc) - approximately 90-95% opment of blue or violaceous discoloration of one (Table 1).8,9 RP in SSc is a clinical manifesta- or more toes in the absence of obvious trauma, tion of vasospasm of the peripheral digital arteries serious cold-induced injury, or disorders producing and arterioles as well as of permanent structural generalized cyanosis. The condition may develop in endothelial damage of the microcirculation that is cases of micro-, macrovascular pathology, impaired associated with the high rate of development of venous outfl ow and hyperviscosity syndromes. Three digital ulcers in these patients in over half of the major categories are recognized: cases.10 In 100 SSc patients, La Montagna et al. 1. disorders with decreased arterial fl ow - arterial (2002) found that symptoms of RP of the feet obstruction due to: are present in 90% of the cases vs 100% fre- • embolism (atheroemboli due to spontaneous plaque quency of RP at the hands. In 43% of the cases haemorrhage or induced by different provoking from this group, RP of the feet was presented at factors, cardiac or aortic tumor, infective endo- initial evaluation, while 47% developed it in the carditis); course of the follow-up. In contrast, RP of the • thrombosis (antiphospholipid syndrome, malig- hands was registered in 100% of patients at the nancy (paraneoplastic acral vascular syndrome, initial evaluation. The onset of clinically evident disseminated intravascular coagulation, warfarin involvement of the feet was found to occur later skin necrosis); in limited SSc than in the diffuse form of the • vasoconstrictive disorders (acrocyanosis, chil- disease. Lower rate of necrotizing RP was found blains, drug induced vasoconstriction, infectious and non-infectious vessel infl ammation; in the feet of patients with SSc as compared with 11 2. impaired venous outfl ow (venous thrombosis); the hands. 3. abnormal circulating blood (paraproteinemia Among other common rheumatic diseases, the with hyperviscosity, myeloproliferative disorders, prevalence of RP in systemic lupus erythematosus cryofi brinogenemia, cryoglobulinemia). varies between 10 and 45% and it usually indicates 8,12 Prevalence of atheroembolism is increased due a more benign course without tissue necrosis. 78 Folia Medica I 2016 I Vol. 58 I No. 2 I Article 1 Instrumental Methods for Assessment of Peripheral Ischaemic Syndromes in Rheumatology Table 1. Common differential diagnosis of Raynaud’s phenomenon in rheumatologic practice. Systemic sclerosis Mixed connective tissue disease Undifferentiated connective tissue disease Systemic lupus erythematosus Dermatomyositis, polymyositis Sjögren syndrome Rheumatoid arthritis Systemic vasculitides - Buerger disease, Takayasu arteritis, polyarteritis nodosa, granulomatosis with poly- angiitis, etc. Other common conditions that are associated with Raynaud’s phenomenon and are included in the differential diagnosis in rheumatologic
Recommended publications
  • Biology and Management of Unusual Plasma Cell Dyscrasias
    Todd M. Zimmerman Shaji K. Kumar Editors Biology and Management of Unusual Plasma Cell Dyscrasias 123 Biology and Management of Unusual Plasma Cell Dyscrasias Todd M. Zimmerman • Shaji K. Kumar Editors Biology and Management of Unusual Plasma Cell Dyscrasias 123 Editors Todd M. Zimmerman, MD Shaji K. Kumar, MD Section of Hematology/Oncology Division of Hematology, The University of Chicago Department of Medicine Chicago, IL, USA Mayo Clinic Rochester, MN, USA ISBN 978-1-4419-6847-0 ISBN 978-1-4419-6848-7 (eBook) DOI 10.1007/978-1-4419-6848-7 Library of Congress Control Number: 2016940068 © Springer Science+Business Media New York 2017 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made.
    [Show full text]
  • Vasculitis in Systemic Sclerosis
    Hindawi Publishing Corporation International Journal of Rheumatology Volume 2010, Article ID 385938, 9 pages doi:10.1155/2010/385938 Review Article Vasculitis in Systemic Sclerosis Lily Kao and Cornelia Weyand Division of Immunology and Rheumatology, School of Medicine, Stanford University, Stanford, 1000 Welch Road, Suite #203, Palo Alto, CA 94304, USA Correspondence should be addressed to Lily Kao, [email protected] Received 14 May 2010; Accepted 17 July 2010 Academic Editor: Laura K. Hummers Copyright © 2010 L. Kao and C. Weyand. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Systemic sclerosis (SSc) is a multiorgan connective tissue disease characterized by autoantibody production and fibroproliferative stenosis of the microvasculature. The vascoluopathy associated with SSc is considered to be noninflammatory, yet frank vasculitis can complicate SSc, posing diagnostic and therapeutic challenges. Here, we have reviewed the literature for reports of small-, medium-, and large-vessel vasculitis occurring in SSc. Amongst 88 reported cases of vasculitis in SSc, patients with ANCA-associated vasculitis appear to present a unique subclass in that they combined typical features of SSc with the renal manifestation of ANCA-associated glomerulonephritis. Other vasculitic syndromes, including large-vessel vasculitis, Behcet’s disease, cryoglobulinemia, and polyarteritis nodosa, are rarely encountered in SSc patients. ANCA-associated vasculitis needs to be considered as a differential diagnosis in SSc patients presenting with renal insufficiency, as renal manifestations may result from distinct disease processes and require appropriate diagnostic testing and treatment. 1. Introduction 2.
    [Show full text]
  • Review Article Vasculitis in Systemic Sclerosis
    Hindawi Publishing Corporation International Journal of Rheumatology Volume 2010, Article ID 385938, 9 pages doi:10.1155/2010/385938 Review Article Vasculitis in Systemic Sclerosis Lily Kao and Cornelia Weyand Division of Immunology and Rheumatology, School of Medicine, Stanford University, Stanford, 1000 Welch Road, Suite #203, Palo Alto, CA 94304, USA Correspondence should be addressed to Lily Kao, [email protected] Received 14 May 2010; Accepted 17 July 2010 Academic Editor: Laura K. Hummers Copyright © 2010 L. Kao and C. Weyand. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Systemic sclerosis (SSc) is a multiorgan connective tissue disease characterized by autoantibody production and fibroproliferative stenosis of the microvasculature. The vascoluopathy associated with SSc is considered to be noninflammatory, yet frank vasculitis can complicate SSc, posing diagnostic and therapeutic challenges. Here, we have reviewed the literature for reports of small-, medium-, and large-vessel vasculitis occurring in SSc. Amongst 88 reported cases of vasculitis in SSc, patients with ANCA-associated vasculitis appear to present a unique subclass in that they combined typical features of SSc with the renal manifestation of ANCA-associated glomerulonephritis. Other vasculitic syndromes, including large-vessel vasculitis, Behcet’s disease, cryoglobulinemia, and polyarteritis nodosa, are rarely encountered in SSc patients. ANCA-associated vasculitis needs to be considered as a differential diagnosis in SSc patients presenting with renal insufficiency, as renal manifestations may result from distinct disease processes and require appropriate diagnostic testing and treatment. 1. Introduction 2.
    [Show full text]
  • ESVM Guidelines – the Diagnosis and Management of Raynaud's Phenomenon
    413 Review ESVM guidelines – the diagnosis and management of Raynaud’s phenomenon Writing group Jill Belch1, Anita Carlizza2, Patrick H. Carpentier3, Joel Constans4, Faisel Khan1, and Jean-Claude Wautrecht5 1 University of Dundee School of Medicine, Dundee, United Kingdom 2 Azienda Ospedaliera S.Giovanni-Addolorata, Rome, Italy 3 Grenoble University Hospital, Grenoble, France 4 Hopital St Andre, Bordeaux, France 5 Cliniques universitaires de Bruxelles, Brussels, Belgium ESVM board authors Adriana Visona6, Christian Heiss7, Marianne Brodeman8, Zsolt Pécsvárady9, Karel Roztocil10, Mary-Paula Colgan11, Dragan Vasic12, Anders Gottsäter13, Beatrice Amann-Vesti14, Ali Chraim15, Pavel Poredoš16, Dan-Mircea Olinic17, Juraj Madaric18, and Sigrid Nikol19 6 Angiology Unit, Azienda ULSS 2, Marca Trevigiana, Treviso, Italy 7 Department of Cardiology, Pulmonology and Vascular Medicine, Düsseldorf, Germany 8 Division of Angiology, Medical University, Graz, Austria 9 Head of 2nd Dept. of Internal Medicine, Vascular Center, Flor Ferenc Teaching Hospital, Kistarcsa, Hungary 10 Institute of Clinical and Experimental Medicine, Prague, Czech Republic 11 St. James’s Hospital and Trinity College, Dublin, Ireland 12 Clinical Centre of Serbia, Belgrade, Serbia 13 Department of Vascular Diseases, Skåne University Hospital, Sweden 14 Clinic for Angiology, University Hospital Zurich, Switzerland 15 Department of Vascular Surgery, Cedrus Vein and Vascular Clinic, Lviv Hospital, Lviv, Ukraine 16 University Medical Centre Ljubljana, Slovenia 17 Medical Clinic no. 1,
    [Show full text]
  • Characteristic Electron-Microscopic Features of Cryofibrinogen
    Ibuki et al. BMC Nephrology (2020) 21:27 https://doi.org/10.1186/s12882-020-1696-0 CASE REPORT Open Access Characteristic electron-microscopic features of cryofibrinogen-associated glomerulonephritis: a case report Emi Ibuki1*† , Aiko Shiraishi2†, Tadashi Sofue2, Yoshio Kushida1, Kyuichi Kadota1, Kazuho Honda3, Dedong Kang3, Kensuke Joh4, Tetsuo Minamino2 and Reiji Haba1 Abstract Background: Cryofibrinogenemia is a rare disorder that mainly affects the skin and occasionally the kidney. However, there are few published reports of cryofibrinogenemia-associated renal pathology. We therefore report a patient with cryofibrinogen-associated glomerulonephritis. Samples from this patient were examined by electron microscopy, laser microdissection, and liquid chromatography-tandem mass spectrometry (LC-MS/MS). Case presentation: A 78-year-old Japanese man presented with declining renal function, proteinuria, and gross hematuria. Kidney biopsy showed a membranoproliferative pattern with crescent formation and dominant C3c deposition in which subendothelial deposits with uniquely organized electron-microscopic features were observed. Additional ultrastructural analysis of cryoprecipitates extracted from plasma revealed similar structures of the glomerular subendothelial deposits. LC-MS/MS identified an increase in fibrinogen α, β, and γ chains, fibronectin, filamin-A, and C3. The glomerular lesions were diagnosed as cryofibrinogen-associated glomerulonephritis on the basis of these findings. Conclusions: Although there are few reports of cryofibrinogen-associated
    [Show full text]
  • Vascular Disease in Systemic Sclerosis
    International Journal of Rheumatology Vascular Disease in Systemic Sclerosis Guest Editors: Lorinda Chung, Oliver Distler, Laura Hummers, Eswar Krishnan, and Virginia Steen Vascular Disease in Systemic Sclerosis International Journal of Rheumatology Vascular Disease in Systemic Sclerosis Guest Editors: Lorinda Chung, Oliver Distler, Laura Hummers, Eswar Krishnan, and Virginia Steen Copyright © 2010 Hindawi Publishing Corporation. All rights reserved. This is a special issue published in volume 2010 of “International Journal of Rheumatology.” All articles are open access articles dis- tributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. International Journal of Rheumatology Editorial Board Salvatore M. F. Albani, USA Shinichi Kawai, Japan Lisa G. Rider, USA Ernest Brahn, USA Charles J. Malemud, USA Vicente Rodriguez-Valverde, Spain Ruben Burgos-Vargas, Mexico Johanne Martel-Pelletier, Canada Bruce M. Rothschild, USA Dirk Elewaut, Belgium Terr y L. Moore, USA J. R. Seibold, USA Luis R. Espinoza, USA Ewa Paleolog, UK Leonard H. Sigal, USA Barri J. Fessler, USA Thomas Pap, Germany Malcolm Smith, Australia Piet Geusens, Belgium Karel Pavelka, Czech Republic G. C. Tsokos, USA Hiroshi Hashimoto, Japan Jean-Pierre Pelletier, Canada Ronald van Vollenhoven, Sweden Sergio Jimenez, USA Proton Rahman, Canada Muhammad B. Yunus, USA Kenneth C. Kalunian, USA Morris Reichlin, USA Contents Vascular Disease in Systemic Sclerosis, Lorinda Chung, Oliver Distler, Laura Hummers, Eswar Krishnan, and Virginia Steen Volume 2010, Article ID 714172, 2 pages The Vascular Microenvironment and Systemic Sclerosis, Tracy Frech, Nathan Hatton, Boaz Markewitz, Mary Beth Scholand, Richard Cawthon, Amit Patel, and Allen Sawitzke Volume 2010, Article ID 362868, 6 pages Digital Ischemia in Scleroderma Spectrum of Diseases, Elena Schiopu, Ann J.
    [Show full text]
  • Thrombophilia: the Dermatological Clinical Spectrum
    Global Dermatology Review Article ISSN: 2056-7863 Thrombophilia: the dermatological clinical spectrum Paulo Ricardo Criado1*, Gleison Vieira Duarte2, Lidia Salles Magalhães2 and Jozélio Freire de Carvalho2 1Department of Dermatology, Universidade de São Paulo, Brazil 2Rheumatology, Universidade Federal da Bahia, Brazil Abstract The aim of this article is to review the hypercoagulable states (thrombophilia) most commonly encountered by dermatologists, as well as their cutaneous signs, including livedo racemosa, cutaneous necrosis, digital ischemia and ulcerations, reticulated purpura, leg ulcers, and other skin conditions. Recognizing these cutaneous signs is the first step to proper treatment. Our aim is to describe which tests are indicated, and when, in these clinical settings. Introduction Hereditary thrombophilia can be classified into three large groups according to the pathogenic mechanism [1]: The possible causes of vascular occlusion can be divided into three large groups: (i) abnormalities of the vascular wall, (ii) changes in blood (i) Reduction of anticoagulant capacity: There are two systems flow, and (iii) hypercoagulation of the blood [1]. The term thrombophilia capable of blocking the clotting cascade and preventing the was introduced in 1965 by Egeberg [2] and is currently used to describe development of a massive and uncontrollable thrombosis: conditions resulting from hereditary (or primary) and/or acquired natural anticoagulants (antithrombin III, protein C, and hyper-coagulation of the blood that increase the predisposition to protein S) and fibrinolysis (plasminogen-plasmin system). A thromboembolic events [3]. Various risk factors, whether genetic or congenital deficit in any one of these components constitutes acquired, make up the pathogenesis of thrombosis in both arteries a possible cause of primary thrombophilia.
    [Show full text]
  • An Approach to Vasculitis and Vasculopathy
    Resident CoRneR An Approach to Vasculitis and Vasculopathy Amanda Pickert, MD asculitis and vasculopathy may arise from (ie, hospitalization if not already addressed). Ideally a primary or secondary cause, which often the workup should be tailored to the clinical situation Vmakes the workup and diagnosis challenging. and the diagnosis. Generally I order several specific Vasculitis occurs when inflammation in the blood tests at the initial visit to look for diagnoses such vessel wall leads to its destruction and vasculopathy as connective-tissue disease or infection. Frequently when a thrombus forms in the arterial lumen and taking a good patient history will guide you on what compromises blood flow. The difference is subtle but to order. However, when I am uncertain, gauging the important to distinguish since there are divergent severity and level of care needed should be the first diagnoses and treatments for vasculitis and vascu- step. Organ involvement usually results in morbidity lopathy. The hallmark clinical feature of vasculitis is and mortality and requires aggressive intervention palpable purpura, which also can be a manifestation and treatment. After assessing the patient’s level of of vasculopathy. Similarly theCUTIS emergence of livedo illness, determining whether the outbreak is vasculitis reticularis is more consistent with vasculopathy but or vasculopathy is crucial. Unless there is compelling also may be seen with either entity. This overlap is evidence for vasculopathy, I tend to assume patients important to keep in mind. Herein I will give a brief have a vasculitis and consider adding in the workup overview of how I approach vasculitis and vasculopa- for vasculopathy as appropriate.
    [Show full text]
  • Cutaneous Vasculitis
    Cutaneous Vasculitis Authors: Lorinda Chung, M.D. and David Fiorentino1, M.D., Ph.D. Creation date: March 2005 Scientific editor: Prof Loïc Guillevin 1Department of Dermatology, Division of Rheumatology and Immunology, Stanford University School of Medicine, 900 Blake Wilbur Drive W0074, Stanford, CA. USA. [email protected] Abstract Keywords Definition Classification / Etiology Approach to the Patient Treatment Future Directions References Abstract Cutaneous vasculitis is a histopathologic entity characterized by neutrophilic transmural inflammation of the blood vessel wall associated with fibrinoid necrosis, termed leukocytoclastic vasculitis (LCV). Clinical manifestations of cutaneous vasculitis occur when small and/or medium vessels are involved. Small vessel vasculitis can present as palpable purpura, urticaria, pustules, vesicles, petechiae, or erythema multiforme-like lesions. Signs of medium vessel vasculitis include livedo reticularis, ulcers, subcutaneous nodules, and digital necrosis. The frequency of vasculitis with skin involvement is unknown. Vasculitis can involve any organ system in the body, ranging from skin-limited to systemic disease. Although vasculitis is idiopathic in 50% of cases, common associations include infections, inflammatory diseases, drugs, and malignancy. The management of cutaneous vasculitis is based on four sequential steps: confirming the diagnosis with a skin biopsy, evaluating for systemic disease, determining the cause or association, and treating based on the severity of disease. Keywords Cutaneous vasculitis, leukocytoclastic vasculitis Definition Vasculitis is inflammation of the blood vessel Classification / Etiology wall that leads to various clinical manifestations The classification schemes for the vasculitides depending on which organ systems are involved. are based on several criteria, including the size Cutaneous vasculitis is a histopathologic entity of the vessel involved, clinical and characterized by neutrophilic transmural histopathologic features, and etiology.
    [Show full text]
  • Giant Cell Myositis and Myocarditis Revisited
    ACTA MYOLOGICA 2020; XXXIX: p. 302-306 doi:10.36185/2532-1900-033 Giant cell myositis and myocarditis revisited Piraye Oflazer Department of Neurology, Koç University Hospital Muscle Center, Koç University Medical Faculty, Topkapı, Istanbul, Turkey Giant cell myositis (GCMm) and giant cell myocarditis (GCMc) are two rare auto- immune conditions. Among these, GCMc is a life-threatening disease with a 1-year mortality rate of 70%. Lethal ventricular arrhythmias, rapid evolution to heart failure and sudden death risk makes GCMc an emergency condition. It is thought to be mediated by T-cells and characterized by the presence of myofiber necrosis and giant cells in biopsies. Most commonly co-manifesting conditions with GCMm and/or GCMc are thymoma, myasthenia gravis and orbital myositis, all of which are treatable. As suspicion is the key approach in diagnosis, the physician follow- ing patients with thymoma with or without myasthenia gravis and with orbital myositis should always be alert. The fatal nature of GCMc associated with these relatively benign diseases deserves a special emergency attention with prompt in- stitution of combined immunosuppressive treatment and very early inclusion of heart failure teams. Key words: giant cell myositis, giant cell myocarditis, thymoma/myasthenia gravis, orbital myositis Giant cell myositis/giant cell myocarditis Received: October 15, 2020 Giant cell myositis and myocarditis, diseases of skeletal and cardiac Accepted: October 15, 2020 muscles are both abbreviated as GCM. As both conditions are the subjects of this report abbreviations will be GCMmuscle (GCMm) for giant cell Correspondence Piraye Oflazer myositis and GCMcardiac (GCMc) for giant cell myocarditis. Department of Neurology, Koç University Hospital Muscle Myositis with giant cells is a rare but treatable acquired inflammatory Center, Koç University Medical Faculty, Davutpaşa Cad 4, disease of the skeletal muscle.
    [Show full text]
  • Uncorrected Author Proof
    Clinical Hemorheology and Microcirculation xx (20xx) x–xx 1 DOI 10.3233/CH-160218 IOS Press 1 Clinical conditions responsible for 2 hyperviscosity and skin ulcers 3 complications ∗ 4 Gregorio Caimi , Baldassare Canino, Rosalia Lo Presti, Caterina Urso and Eugenia Hopps 5 Dipartimento Biomedico di Medicina Interna e Specialistica, Universit`a di Palermo, Italy 6 Abstract. In this brief review, we have examined some clinical conditions that result to be associated to an altered hemorhe- 7 ological profile and at times accompanied by skin ulcers. This skin condition may be observed in patients with the following 8 condtions, such as primary polycythemic hyperviscosity (polycythemia, thrombocytemia) treated with hydroxyurea, primary 9 plasma hyperviscosity (multiple myeloma, cryoglobulinemia, cryofibrinogenemia, dysfibrinogenemia, and connective tissue 10 diseases), primary sclerocythemic hyperviscosity (hereditary spherocytosis, thalassemia, and sickle cell disease). In addition, 11 it may be present in patients with secondary hyperviscosity conditions such as diabetes mellitus, arterial hypertension, critical limb ischemia and chronic venous insufficiency. 12 13 Keywords: Hyperviscosity syndrome, blood viscosity, skin ulcers 13 1. Introduction 14 The blood flow differs from that running through microvessels and that through large vessels. These 15 differences refer to the blood composition, haemodynamics, and specifically blood viscosity. Rheolog- 16 ical alterations play a prominent role in microcirculation than in large vessels haemodynamics. When 17 a potentially ischemic condition emerges, some changes develop in microcirculation in relation to 18 the diameter and the wall permeability of microvessel, the cell metabolism and the haemorheological 19 profile. Physiologically, the blood flow is influenced by blood velocity, vessel diameter, structure and 20 blood viscosity.
    [Show full text]
  • Case Report Cryofibrinogenemia and Skin Necrosis in a Patient With
    Bone Marrow Transplantation (2000) 26, 1343–1345 2000 Macmillan Publishers Ltd All rights reserved 0268–3369/00 $15.00 www.nature.com/bmt Case report Cryofibrinogenemia and skin necrosis in a patient with diffuse large cell lymphoma after high-dose chemotherapy and autologous stem cell transplantation A Shimoni, M Ko¨rbling, R Champlin and J Molldrem The Department of Blood and Marrow Transplantation, The University of Texas MD Anderson Cancer Center, Houston, TX, USA Summary: day 11 (with sustained ANC Ͼ1 × 109/l for 3 consecutive days) and restaging tests on day 30 showed she was in A 34-year-old woman with diffuse mediastinal B cell complete remission. The patient completed the first mainte- large cell lymphoma presented 60 days after high-dose nance course of IL-2 that was given in reduced doses (2 × chemotherapy and autologous stem cell transplantation, 106 U/m2 daily by subcutaneous injection, 25% of planned and post-transplant immunotherapy with interleukin-2, dosing) due to fevers and pancytopenia. with skin necrosis in the ears and extremities. Extensive She presented 2 weeks later, on day 56 of the transplant, work-up revealed the presence of cryofibrinogenemia with symptoms of an upper respiratory tract infection and and associated thrombotic vasculopathy. The patient was given oral antibiotics. A few days later she developed was successfully treated with corticosteroids and thera- fever up to 38.2°C and then rapidly progressive large peutic plasma exchange. However, she had recurrence purpuric/necrotic lesions on her ears, and upper and lower of large cell lymphoma a few weeks later and died of extremities (see Figure 1).
    [Show full text]