DOCSLIB.ORG

Scleroderma – Lupus Overlap Syndrome: Case Report

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Scleroderma – Lupus Overlap Syndrome: Case Report CASE REPORT Ref: Ro J Rheumatol. 2018;27(2) DOI: 10.37897/RJR.2018.2.6 SCLERODERMA – LUPUS OVERLAP SYNDROME: CASE REPORT Gabriela Ticu1, Marius Ioan Balea2, Camelia Badea1,3, Adelina Silvana Gheorghe1,4, Ruxandra Patrascu1,3 1Internal Medicine Department, Colentina Clinical Hospital, Bucharest 2Pneumology Department, Colentina Clinical Hospital, Bucharest 3Carol Davila University of Medicine and Pharmacy, Bucharest 4Prof. Dr. Alexandu Trestioreanu Institute of Oncology, Bucharest Abstract The overlap syndromes are a diverse group of conditions that usually have characteristic features of at least two well-defined rheumatic diseases occurring together. We present the case of a 32-year-old patient who sequen- tially develops manifestations of systemic lupus erythematosus and systemic sclerosis. The first manifestation of the disease was the pleural effusion and, over time, the patient associated various other complications which influenced the diagnostic course. Digital ulcerations associated to scleroderma have evolved into ischemic necro- sis lesions, requiring amputation. This case is a clinically suggestive example of the complexity of autoimmune diseases, of associations between pathologies and unexpected evolutions, requiring constant and long-term follow-up of the patients. Keywords: systemic lupus erythematosus, serositis, systemic sclerosis, digital ulcers, overlap syndrome INTRODUCTION CASE PRESENTATION Clinicians are often challenged by the clinical is- We are presenting the case of a 32 years old sues when confronting with patients who either pres- woman, ex-smoker (10 pack-years, quit smoking 3 ent an undifferentiated rheumatic disease or an over- years ago), with class III obesity (body mass index lap syndrome. In the medical literature, it is 58.2 kg/m2) and a history of abortion on demand one appreciated that approximately 25 percent of pa- month prior to the admission at the hospital. She de- tients with rheumatic diseases cannot be definitely scribes left posterior thoracic pain, night rigors, fe- diagnosed with a single well-characterized systemic ver (38.5ºC), followed by decreased effort tolerance rheumatic disease (1,2). Moreover, some of them re- and dyspnea on minimal exertion, installed two main undiagnosed for a long period of time, despite weeks before presentation. The patient also com- plains of morning joint stiffness, especially in the periodical reassessments and expectant management wrists, which had intensified in the last month. (1,2). It happens due to the fact that this category of From the pathological personal history, it is worth patients exhibits several patterns, manifesting multi- mentioning that the patient followed 6 months of tu- ple nonspecific serologic or clinical abnormalities, berculostatic treatment, 9 years ago, for positive sometimes of more than one defined disorder. Some Mantoux tuberculin skin test, but without suggestive examples include rheumatoid arthritis – lupus (rhu- chest radiography or confirmation of tuberculosis pus), scleroderma – polymyositis/dermatomyositis, through sputum smear microscopy. scleroderma – lupus, scleroderma – rheumatoid ar- Physical examination on admission to the hospi- thritis, Sjögren‘s syndrome overlaps and others (1). tal revealed that the patient was afebrile, tachypneic Correspondence address: Gabriela Ticu, Colentina Clinical Hospital, 19-21 Stefan cel Mare Road, Sector 2, Bucharest, Romania E-mail: [email protected] 84 ROMANIAN JOURNAL OF RHEUMATOLOGY – VOLUME XXVII, NO. 2, 2018 ROMANIAN JOURNAL OF RHEUMATOLOGY – VOLUME XXVII, NO. 2, 2018 85 with a respiratory rate of 24 breaths per minute and diaphragmatic recess. The rest of the investigations tachycardic with a ventricular rate of 104 rhythmic (electrocardiogram, echocardiogram, abdominal and beats per minute (regular heart rhythm). The vesicu- pelvic ultrasound, lower extremity veins Doppler ul- lar murmur was abolished with dullness to percus- trasound) did not show any relevant particularities. sion over left lung base and decreased breath sounds The patient underwent thoracentesis of the left bilaterally. A bilateral hand edema could also be ob- pleural effusion. The fluid obtained had the charac- served. The blood pressure was normal, without pul- teristics of an exudate, with 2.400 cells/mm3, 46% monary crackles, cardiac murmurs or extra sounds neutrophils and 27% lymphocytes, but with negative and no evidence of arthritis or rashes. bacterial cultures. Examination by Ziehl-Neelsen Since the onset of symptoms, prior to the admis- staining did not reveal acid-alcohol-resistant bacilli, sion in the hospital, the patient presented to the gen- the level of adenosine deaminase in the effusion liq- eral practitioner, who recommended chest radiogra- uid was normal and Mycobacterium tuberculosis phy, computed tomography (CT) scan of the thorax DNA was not detected by polymerase chain reac- and pleural ultrasound. At these investigations, a tion. Also, the histopathological examination of the pleural biopsy was normal. small pleural effusion was discovered in the left Despite having received antibiotic (ceftriaxone), lung, along with atelectasis aspect or pulmonary pa- anti-inflammatory and antialgic (celecoxib) treat- renchyma condensation (Fig. 1). No direct or indi- ment, subfebrility persisted, only with a minimal im- rect signs of pulmonary embolism have been detect- provement of the thoracic pain. ed. After 10 days, the patient was admitted again in The episode was interpreted as the an intercurrent the hospital for paroxysmal nocturnal dyspnea, per- respiratory infection, with secondary pleural reac- sistence of the thoracic pain and dyspnea on minimal tion and the patient received broad-spectrum empiric exertion. Clinical examination and several investiga- antibiotic therapy: 6 days of levofloxacin, followed tions (thoracic ultrasound, echocardiography and CT by 3 days of ciprofloxacin and 5 days of drug com- scan) led to the discovery of pericardial and bilateral bination (gentamicin, amoxicillin/clavulanic acid pleural effusions. During thoracic ultrasound, an im- and metronidazole). Under this treatment, she con- portant right diaphragmatic elevation with low mo- tinued to develop fever (38.2-38.5ºC). bility of the diaphragm was also noticed. We identified an important inflammatory pro- The serological tests revealed positive anti-nu- cess, based on the elevation of serum biomarkers: clear antibodies, C3 hypocomplementemia, pres- C-reactive protein (146.55 mg/L), erythrocyte sedi- ence of lupus anticoagulant and high-titer anti-cardi- mentation rate (61 mm/1h) and fibrinogen (915 mg/ olipin IgM antibodies (antiphospholipid antibody dL). The complete blood count showed discrete ane- positivity). Anti-double stranded DNA (dsDNA) an- mia, thrombocytosis, without leukocytosis or leuko- tibodies, anti-Ro antibodies, anti-beta2-glycoprotein penia. Serum creatinine and microscopic urinalysis I IgG antibodies, anti-cardiolipin IgG antibodies were normal. were negative, as well as the rheumatoid factor and We also performed chest radiography after ad- direct Coombs test. mission, which revealed the same aspect of pleural These three immunological criteria, added to the effusion in small amounts, located in the left costo- forth clinical criterion (serositis, consisting in pleuri- FIGURE 1. Chest radiography and computed tomography scan of the thorax showing small pleural effusion in the left lung (with a maximum of 2 centimeters in transversal diameter at the ultrasound examination) 86 ROMANIAN JOURNAL OF RHEUMATOLOGY – VOLUME XXVII, NO. 2, 2018 sy and pericarditis), led to the classification and then The switch in the patient’s dominant signs and diagnosis of systemic lupus erythematosus (SLE) symptoms guided us to discover a second autoim- with minimal articular involvement (based on the mune illness: systemic sclerosis (SSc) or scleroder- aforementioned morning joint stiffness) – according ma. Meeting the classification criteria for two sepa- to SLICC (Systemic Lupus International Collaborat- rate well-characterized rheumatic diseases, SLE and ing Clinics) classification criteria 2012. Due to the SSc, we came to the final diagnosis of overlap syn- presence of progressive dyspnea, accompanied by drome between the two diseases. pleuritic chest pain, diaphragmatic elevation and The patient received treatment with corticoster- lung volume reduction, we suspected a rare compli- oid, anti-malaric, sulodexide and pentoxifylline, as cation associated with lupus, the shrinking lung syn- well as topical dermatological preparations for the drome, but this has not been confirmed. ischemic lesions. She was advised to avoid cold ex- Under treatment with systemic corticosteroids posure and to quit smoking. No further actions were (Dexamethasone, followed by Methyl-prednisolone) taken for the next 5 months, as the patient did not and anti-malarial drug (Hydroxychloroquine), the present to the hospital for immunosuppressive thera- patient had a favorable evolution, both clinically and py and association of anticoagulation. During this biologically. time, she underwent gynecological assessments in Over the following two months, the patient did the private healthcare system. The cervical biopsy not experience fever, dyspnea or thoracic pain, so we showed the presence of an intraepithelial squamous advised her to continue the treatment with corticos- lesion, positive for Human papillomavirus DNA teroid and hydroxychloroquine as well as to lose (high-risk genotype). weight. At the following
Load more

Recommended publications
  • A Review of the Evidence for and Against a Role for Mast Cells in Cutaneous Scarring and Fibrosis
    International Journal of Molecular Sciences Review A Review of the Evidence for and against a Role for Mast Cells in Cutaneous Scarring and Fibrosis Traci A. Wilgus 1,*, Sara Ud-Din 2 and Ardeshir Bayat 2,3 1 Department of Pathology, Ohio State University, Columbus, OH 43210, USA 2 Centre for Dermatology Research, NIHR Manchester Biomedical Research Centre, Plastic and Reconstructive Surgery Research, University of Manchester, Manchester M13 9PT, UK; [email protected] (S.U.-D.); [email protected] (A.B.) 3 MRC-SA Wound Healing Unit, Division of Dermatology, University of Cape Town, Observatory, Cape Town 7945, South Africa * Correspondence: [email protected]; Tel.: +1-614-366-8526 Received: 1 October 2020; Accepted: 12 December 2020; Published: 18 December 2020 Abstract: Scars are generated in mature skin as a result of the normal repair process, but the replacement of normal tissue with scar tissue can lead to biomechanical and functional deficiencies in the skin as well as psychological and social issues for patients that negatively affect quality of life. Abnormal scars, such as hypertrophic scars and keloids, and cutaneous fibrosis that develops in diseases such as systemic sclerosis and graft-versus-host disease can be even more challenging for patients. There is a large body of literature suggesting that inflammation promotes the deposition of scar tissue by fibroblasts. Mast cells represent one inflammatory cell type in particular that has been implicated in skin scarring and fibrosis. Most published studies in this area support a pro-fibrotic role for mast cells in the skin, as many mast cell-derived mediators stimulate fibroblast activity and studies generally indicate higher numbers of mast cells and/or mast cell activation in scars and fibrotic skin.
    [Show full text]
  • Foot Pain in Scleroderma
    Foot Pain in Scleroderma Dr Begonya Alcacer-Pitarch LMBRU Postdoctoral Research Fellow 20th Anniversary Scleroderma Family Day 16th May 2015 Leeds Institute of Rheumatic and Musculoskeletal Medicine Presentation Content n Introduction n Different types of foot pain n Factors contributing to foot pain n Impact of foot pain on Quality of Life (QoL) Leeds Institute of Rheumatic and Musculoskeletal Medicine Scleroderma n Clinical features of scleroderma – Microvascular (small vessel) and macrovascular (large vessel) damage – Fibrosis of the skin and internal organs – Dysfunction of the immune system n Unknown aetiology n Female to male ratio 4.6 : 1 n The prevalence of SSc in the UK is 8.21 per 100 000 Leeds Institute of Rheumatic and Musculoskeletal Medicine Foot Involvement in SSc n Clinically 90% of SSc patients have foot involvement n It typically has a later involvement than hands n Foot involvement is less frequent than hand involvement, but is potentially disabling Leeds Institute of Rheumatic and Musculoskeletal Medicine Different Types of Foot Pain Leeds Institute of Rheumatic and Musculoskeletal Medicine Ischaemic Pain (vascular) Microvascular disease (small vessel) n Intermittent pain – Raynaud’s (spasm) • Cold • Throb • Numb • Tingle • Pain n Constant pain – Vessel center narrows • Distal pain (toes) • Gradually increasing pain • Intolerable pain when necrosis is present Leeds Institute of Rheumatic and Musculoskeletal Medicine Ischaemic Pain (vascular) Macrovascular disease (large vessels) n Intermittent and constant pain – Peripheral Arterial Disease • Intermittent claudication – Muscle pain (ache, cramp) during walking • Aching or burning pain • Night and rest pain • Cramps Leeds Institute of Rheumatic and Musculoskeletal Medicine Ulcer Pain n Ulcer development – Constant pain n Infected ulcer – Unexpected/ excess pain or tenderness Leeds Institute of Rheumatic and Musculoskeletal Medicine Neuropathic Pain n Nerve damage is not always obvious.
    [Show full text]
  • A Patient with Plaque Type Morphea Mimicking Systemic Lupus Erythematosus
    CASE REPORT A Patient With Plaque Type Morphea Mimicking Systemic Lupus Erythematosus Wardhana1, EA Datau2 1 Department of Internal Medicine, Siloam International Hospitals. Karawaci, Indonesia. 2 Department of Internal Medicine, Prof. Dr. RD Kandou General Hospital & Sitti Maryam Islamic Hospital, Manado, North Sulawesi, Indonesia. Correspondence mail: Siloam Hospitals Group’s CEO Office, Siloam Hospital Lippo Village. 5th floor. Jl. Siloam No.6, Karawaci, Indonesia. email: [email protected] ABSTRAK Morfea merupakan penyakit jaringan penyambung yang jarang dengan gambaran utama berupa penebalan dermis tanpa disertai keterlibatan organ dalam. Penyakit ini juga dikenal sebagai bagian dari skleroderma terlokalisir. Berdasarkan gambaran klinis dan kedalaman jaringan yang terlibat, morfea dikelompokkan ke dalam beberapa bentuk dan sekitar dua pertiga orang dewasa dengan morfea mempunyai tipe plak. Produksi kolagen yang berlebihan oleh fibroblast merupakan penyebab kelainan pada morfea dan mekanisme terjadinya aktivitas fibroblast yang berlebihan ini masih belum diketahui, meskipun beberapa mekanisme pernah diajukan. Morfe tipe plak biasanya bersifat ringan dan dapat sembuh dengan sendirinya. Morfea tipe plak yang penampilan klinisnya menyerupai lupus eritematosus sistemik, misalnya meliputi alopesia dan ulkus mukosa di mulut, jarang dijumpai. Sebuah kasus morfea tipe plak pada wanita berusia 20 tahun dibahas. Pasien ini diobati dengan imunosupresan dan antioksidan local maupun sistemik. Kondisi paisen membaik tanpa disertai efek samping yang berarti. Kata kunci: morfea, tipe plak. ABSTRACT Morphea is an uncommon connective tissue disease with the most prominent feature being thickening or fibrosis of the dermal without internal organ involvement. It is also known as a part of localized scleroderma. Based on clinical presentation and depth of tissue involvement, morphea is classified into several forms, and about two thirds of adults with morphea have plaque type.
    [Show full text]
  • Case Report an Exophytic and Symptomatic Lesion of the Labial Mucosa Diagnosed As Labial Seborrheic Keratosis
    Int J Clin Exp Pathol 2019;12(7):2749-2752 www.ijcep.com /ISSN:1936-2625/IJCEP0093949 Case Report An exophytic and symptomatic lesion of the labial mucosa diagnosed as labial seborrheic keratosis Hui Feng1,2, Binjie Liu1, Zhigang Yao1, Xin Zeng2, Qianming Chen2 1XiangYa Stomatological Hospital, Central South University, Changsha 410000, Hunan, P. R. China; 2State Key Laboratory of Oral Diseases, West China Hospital of Stomatology, Sichuan University, Chengdu 610041, Sichuan, P. R. China Received March 16, 2019; Accepted April 23, 2019; Epub July 1, 2019; Published July 15, 2019 Abstract: Seborrheic keratosis is a common benign epidermal tumor that occurs mainly in the skin of the face and neck, trunk. The tumors are not, however, seen on the oral mucous membrane. Herein, we describe a case of labial seborrheic keratosis confirmed by histopathology. A healthy 63-year-old man was referred to our hospital for evalu- ation and treatment of a 2-month history of a labial mass with mild pain. Clinically, the initial impressions were ma- lignant transformation of chronic discoid lupus erythematosus, syphilitic chancre, or keratoacanthoma. Surprisingly, our laboratory results and histopathologic evaluations established a novel diagnosis of a hyperkeratotic type of labial seborrheic keratosis (SK). This reminds us that atypical or varying features of seborrheic keratosis make it difficult to provide an accurate diagnosis. Clinical manifestations of some benign lesions may be misdiagnosed as malignancy. Consequently, dentists should consider this as a differential diagnosis in labial or other oral lesions. Keywords: Seborrheic keratosis, exophytic lesion, symptomatic lesion, labial mucosa, oral mucous membrane Introduction findings were revealed in his medical history, and he had no known allergies to foods or me- Seborrheic keratosis is a common benign epi- dications.
    [Show full text]
  • Systemic Lupus Erythematosus and Granulomatous Lymphadenopathy
    Shrestha et al. BMC Pediatrics 2013, 13:179 http://www.biomedcentral.com/1471-2431/13/179 CASE REPORT Open Access Systemic lupus erythematosus and granulomatous lymphadenopathy Devendra Shrestha1*, Ajaya Kumar Dhakal1, Shiva Raj KC2, Arati Shakya1, Subhash Chandra Shah1 and Henish Shakya1 Abstract Background: Systemic lupus erythematosus (SLE) is known to present with a wide variety of clinical manifestations. Lymphadenopathy is frequently observed in children with SLE and may occasionally be the presenting feature. SLE presenting with granulomatous changes in lymph node biopsy is rare. These features may also cause diagnostic confusion with other causes of granulomatous lymphadenopathy. Case presentation: We report 12 year-old female who presented with generalized lymphadenopathy associated with intermittent fever as well as weight loss for three years. She also had developed anasarca two years prior to presentation. On presentation, she had growth failure and delayed puberty. Lymph node biopsy revealed granulomatous features. She developed a malar rash, arthritis and positive ANA antibodies over the course of next two months and showed WHO class II lupus nephritis on renal biopsy, which confirmed the final diagnosis of SLE. She was started on oral prednisolone and hydroxychloroquine with which her clinical condition improved, and she is currently much better under regular follow up. Conclusion: Generalized lymphadenopathy may be the presenting feature of SLE and it may preceed the other symptoms of SLE by many years as illustrated by this patient. Granulomatous changes may rarely be seen in lupus lymphadenitis. Although uncommon, in children who present with generalized lymphadenopathy along with prolonged fever and constitutional symptoms, non-infectious causes like SLE should also be considered as a diagnostic possibility.
    [Show full text]
  • Nodular Morphea
    Case Report Dermatology 2009;218:63–66 Received: July 13, 2008 DOI: 10.1159/000173976 Accepted: July 23, 2008 Published online: November 13, 2008 Nodular Morphea a b c F. Kauer J.C. Simon M. Sticherling a b Department of Dermatology and Venerology, Vivantes Klinikum Neukölln, Berlin , Department of Dermatology, c Venerology and Allergology, University of Leipzig, Leipzig , and Department of Dermatology, Venerology and Allergology, University of Erlangen, Erlangen , Germany Key Words can range in size from 2 mm to 4–5 cm, flamed skin that is already involved in an -Scleroderma ؒ Keloid ؒ Hypertrophic scar ؒ usually appear spontaneously and tend to active fibrotic process inherent to the dis Morphea involve the trunk and upper extremities. ease in those patients who are genetically A linear presentation has also been de- predisposed to keloid development, or at scribed. The literature on this topic is con- sites of the skin that show a high predilec- Abstract fusing because the terms ‘nodular sclero- tion for keloid formation, such as the trunk Scleroderma may present as being strictly derma’ and ‘keloidal scleroderma’ are used [6, 7] . limited to the skin, as in morphea, or within interchangeably even though there is a a multiorgan disease, as in systemic sclero- great degree of variability in the histologi- sis. Accordingly, cutaneous manifestations cal findings of these nodules [4] . In con- C a s e R e p o r t vary clinically. In nodular or keloidal sclero- trast, other authors stress that the cutane- derma, patients develop lesions that are ous manifestations may vary clinically, but Medical History clinically indistinguishable from a keloid; all share the same histopathological pat- A 16-year-old girl presented with mul- however, the histopathological findings are tern of both morphea/scleroderma and ke- tiple progressive morpheic skin lesions more variable.
    [Show full text]
  • Topical Photodynamic Therapy for Localized Scleroderma
    Acta Derm Venereol 2000; 80: 26±27 Topical Photodynamic Therapy for Localized Scleroderma SIGRID KARRER, CHRISTOPH ABELS, MICHAEL LANDTHALER and ROLF-MARKUS SZEIMIES Department of Dermatology, University of Regensburg, Regensburg, Germany Therapy of localized scleroderma is unsatisfactory, with MATERIAL AND METHODS numerous treatments being used that have only limited success Patients or considerable side-effects. The aim of this trial was to determine whether topical photodynamic therapy would be Five patients aged between 21 and 63 years with biopsy-proven effective in patients with localized scleroderma. Five patients localized scleroderma (morphoea) were studied. All patients had evidence of progressive disease, i.e. lesions showed an in¯ammatory with progressive disease, in whom conventional therapies had reaction and increase in size. In each patient the condition had failed, were treated by application of a gel containing 3% previously failed to respond to potent topical corticosteroids, systemic 5-aminolevulinic acid followed by irradiation with an incoherent therapy with penicillin and/or PUVA bath photochemotherapy. lamp (40 mW/cm2, 10 J/cm2). The treatment was performed Patients were required to discontinue all therapy at least 4 weeks once or twice weekly for 3 ± 6 months. In all patients the before study initiation. The most affected sclerotic plaque of each therapy was highly effective for sclerotic plaques, as measured patient was judged at baseline and then every 2 weeks during by a quantitative durometer score and a clinical skin score. The treatment. Sclerotic plaques were assessed by a clinical skin score (10) only side-effect was a transient hyperpigmentation of the and the durometer score (11).
    [Show full text]
  • Tumid Lupus Erythematosus
    Tumid Lupus Erythematosus Sylvia Hsu, MD; Linda Y. Hwang, MD; Hiram Ruiz, MD Tumid lupus erythematosus (TLE) is a variant of cutaneous lupus erythematosus. Most patients who present with these skin lesions are young women. The condition clinically resembles polymorphous light eruption, systemic lupus ery- thematosus (SLE), reticulated erythematous mucinosis, or gyrate erythema. Histopathologi- cally, the lesions resemble classic lupus erythem- atosus because of their superficial and deep lymphohistiocytic inflammatory infiltrates and dermal mucin. However, unlike classic lupus ery- thematosus, there is little or no epidermal or dermo-epidermal involvement. Antinuclear anti- body test results are usually negative. We describe 4 cases of TLE and discuss the differ- ential diagnosis. here have been few reports of tumid lupus erythematosus (TLE) in the literature.1-4 T Most major textbooks of dermatology or Figure 1. Erythematous papules and plaques on the dermatopathology mention this entity only briefly, chest of patient 1. if at all. Ackerman et al5 consider TLE to be a manifestation of discoid lupus erythematosus (DLE). We describe 4 patients with TLE and review the her chest, upper extremities, and face (Figure 1). list of controversial entities that overlap clinically Test results for antinuclear antibodies (ANA), Ro, and histologically with TLE. La, and dsDNA were all negative. Histopathology results revealed superficial and deep perivascular Case Reports and periadnexal lymphohistiocytic infiltrates with Patient 1—A 34-year-old white woman presented dermal mucin. There was no involvement of the with an 8-year history of asymptomatic lesions on epidermis, the dermo-epidermal junction, or the her face, neck, chest, and upper extremities.
    [Show full text]
  • Clinical Spectrum of Lyme Disease
    European Journal of Clinical Microbiology & Infectious Diseases (2019) 38:201–208 https://doi.org/10.1007/s10096-018-3417-1 REVIEW Clinical spectrum of Lyme disease Jesus Alberto Cardenas-de la Garza1 & Estephania De la Cruz-Valadez1 & Jorge Ocampo-Candiani 1 & Oliverio Welsh1 Received: 4 September 2018 /Accepted: 30 October 2018 /Published online: 19 November 2018 # Springer-Verlag GmbH Germany, part of Springer Nature 2018 Abstract Lyme disease (borreliosis) is one of the most common vector-borne diseases worldwide. Its incidence and geographic expansion has been steadily increasing in the last decades. Lyme disease is caused by Borrelia burgdorferi sensu lato, a heterogeneous group of which three genospecies have been systematically associated to Lyme disease: B. burgdorferi sensu stricto Borrelia afzelii and Borrelia garinii. Geographical distribution and clinical manifestations vary according to the species involved. Lyme disease clinical manifestations may be divided into three stages. Early localized stage is characterized by erythema migrans in the tick bite site. Early disseminated stage may present multiple erythema migrans lesions, borrelial lymphocytoma, lyme neuroborreliosis, carditis, or arthritis. The late disseminated stage manifests with acordermatitis chronica atrophicans, lyme arthritis, and neurological symptoms. Diagnosis is challenging due to the varied clinical manifestations it may present and usually involves a two-step serological approach. In the current review, we present a thorough revision of the clinical manifestations Lyme disease may present. Additionally, history, microbiology, diagnosis, post-treatment Lyme disease syndrome, treatment, and prognosis are discussed. Keywords Lyme disease . Borrelia burgdorferi . Tick-borne diseases . Ixodes . Erythema migrans . Lyme neuroborreliosis History posteriorly meningitis, establishing a link between both mani- festations.
    [Show full text]
  • Oral Health Care for Patients with Epidermolysis Bullosa
    Oral Health Care for Patients with Epidermolysis Bullosa Best Clinical Practice Guidelines October 2011 Oral Health Care for Patients with Epidermolysis Bullosa Best Clinical Practice Guidelines October 2011 Clinical Editor: Susanne Krämer S. Methodological Editor: Julio Villanueva M. Authors: Prof. Dr. Susanne Krämer Dr. María Concepción Serrano Prof. Dr. Gisela Zillmann Dr. Pablo Gálvez Prof. Dr. Julio Villanueva Dr. Ignacio Araya Dr. Romina Brignardello-Petersen Dr. Alonso Carrasco-Labra Prof. Dr. Marco Cornejo Mr. Patricio Oliva Dr. Nicolás Yanine Patient representatives: Mr. John Dart Mr. Scott O’Sullivan Pilot: Dr. Victoria Clark Dr. Gabriela Scagnet Dr. Mariana Armada Dr. Adela Stepanska Dr. Renata Gaillyova Dr. Sylvia Stepanska Review: Prof. Dr. Tim Wright Dr. Marie Callen Dr. Carol Mason Prof. Dr. Stephen Porter Dr. Nina Skogedal Dr. Kari Storhaug Dr. Reinhard Schilke Dr. Anne W Lucky Ms. Lesley Haynes Ms. Lynne Hubbard Mr. Christian Fingerhuth Graphic design: Ms. Isabel López Production: Gráfica Metropolitana Funding: DEBRA UK © DEBRA International This work is subject to copyright. ISBN-978-956-9108-00-6 Versión On line: ISBN 978-956-9108-01-3 Printed in Chile in October 2011 Editorial: DEBRA Chile Acknowledgement: We would like to thank Coni V., María Elena, María José, Daniela, Annays, Lisette, Victor, Coni S., Esteban, Coni A., Felipe, Nibaldo, María, Cristián, Deyanira and Victoria for sharing their smile to make these Guidelines more friendly. 4 Contents 1 Introduction 07 2 Oral care for patients with Inherited Epidermolysis Bullosa 11 3 Dental treatment 19 4 Anaesthetic management 29 5 Summary of recommendations 33 Development of the guideline 37 6 Appendix 43 7.1 List of abbreviations and glossary 7.2 Oral manifestations of Epidermolysis Bullosa 7 7.3 General information on Epidermolysis Bullosa 7.4 Exercises for mouth, jaw and tongue 8 References 61 5 A message from the patient representative: “Be guided by the professionals.
    [Show full text]
  • Immune Thrombocytopenic Purpura (ITP) — Adult Conditions for Which Ivig Has an Established Therapeutic Role
    Immune thrombocytopenic purpura (ITP) — adult Conditions for which IVIg has an established therapeutic role. Specific Conditions Newly Diagnosed Immune thrombocytopenic purpura (ITP) Persistent Immune thrombocytopenic purpura (ITP) Chronic Immune thrombocytopenic purpura (ITP) Evans syndrome ‐ with significant Immune thrombocytopenic purpura (ITP) ‐ adult Indication for IVIg Use Newly diagnosed ITP — initial Ig therapy ITP in pregnancy — initial Ig therapy ITP with life‐threatening haemorrhage or the potential for life‐threatening haemorrhage Newly diagnosed or persistent ITP — subsequent therapy (diagnosis <12 months) Refractory persistent or chronic ITP — splenectomy failed or contraindicated and second‐line agent unsuccessful Subsequent or ongoing treatment for ITP responders during pregnancy and the postpartum period ITP and inadequate platelet count for planned surgery HIV‐associated ITP Level of Evidence Evidence of probable benefit – more research needed (Category 2a) Description and Diagnostic Immune thrombocytopenic purpura (ITP) is a reduction in platelet count Criteria (thrombocytopenia) resulting from shortened platelet survival due to anti‐platelet antibodies, reduced platelet production due to immune induced reduced megakaryopoeisis and/or immune mediated direct platelet lysis. When counts are very low (less than 30x109/L), bleeding into the skin (purpura) and mucous membranes can occur. Bone marrow platelet production (megakaryopoiesis) is morphologically normal. In some cases, there is additional impairment of platelet function related to antibody binding to glycoproteins on the platelet surface. It is a common finding in patients with human immunodeficiency virus (HIV) disease, and while it may be found at any stage of the infection, its prevalence increases as HIV disease advances. Around 80 percent of adults with ITP have the chronic form of disease.
    [Show full text]
  • Systemic Sclerosis/Scleroderma: a Treatable Multisystem Disease
    Systemic Sclerosis/Scleroderma: A Treatable Multisystem Disease MONIQUE HINCHCLIFF, MD, and JOHN VARGA, MD Northwestern University, Feinberg School of Medicine, Chicago, Illinois Systemic sclerosis (systemic scleroderma) is a chronic connective tissue disease of unknown etiology that causes wide- spread microvascular damage and excessive deposition of collagen in the skin and internal organs. Raynaud phenome- non and scleroderma (hardening of the skin) are hallmarks of the disease. The typical patient is a young or middle-age woman with a history of Raynaud phenomenon who presents with skin induration and internal organ dysfunction. Clinical evaluation and laboratory testing, along with pulmonary function testing, Doppler echocardiography, and high-resolution computed tomography of the chest, establish the diagnosis and detect visceral involvement. Patients with systemic sclerosis can be classified into two distinct clinical subsets with different patterns of skin and internal organ involvement, autoantibody production, and survival. Prognosis is determined by the degree of internal organ involvement. Although no disease-modifying therapy has been proven effective, complications of systemic sclerosis are treatable, and interventions for organ-specific manifestations have improved substantially. Medications (e.g., cal- cium channel blockers and angiotensin-II receptor blockers for Raynaud phenomenon, appropriate treatments for gastroesophageal reflux disease) and lifestyle modifications can help prevent complications, such as digital ulcers and Barrett esophagus. Endothelin-1 receptor blockers and phosphodiesterase-5 inhibitors improve pulmonary arte- rial hypertension. The risk of renal damage from scleroderma renal crisis can be lessened by early detection, prompt initiation of angiotensin-converting enzyme inhibitor therapy, and avoidance of high-dose corticosteroids. Optimal patient care includes an integrated, multidisciplinary approach to promptly and effectively recognize, evaluate, and manage complications and limit end-organ dysfunction.
    [Show full text]