Beneath the Surface: Derm Clues to Underlying Disorders
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Understanding and Managing Scleroderma
Understanding and Managing Scleroderma A publication of Scleroderma Foundation 300 Rosewood Drive, Suite 105 Danvers, MA 01923 Maureen D. Mayes, M.D., M.P.H. Understanding and Understanding My notes and Managing Scleroderma Managing Scleroderma This booklet is intended to help people with scleroderma, their families and others interested ________________________ in learning more about the disease to better understand what scleroderma is, what effects ________________________ it may have, and what those with scleroderma can do to help themselves and their physicians ________________________ manage the disease. It answers some of the most frequently asked questions about ________________________ A publication of Maureen D. Mayes, M.D., M.P.H. Scleroderma Foundation 300 Rosewood Drive, Suite 105 scleroderma. Danvers, MA 01923 800-722-HOPE (4673) www.scleroderma.org www.facebook.com/sclerodermaUS www.twitter.com/scleroderma ________________________ Disclaimer The Scleroderma Foundation does not provide medical advice nor does it ________________________ endorse any drug or treatment mentioned herein. ________________________ The material contained in this booklet is presented for general information only. It is not intended to provide medical advice, to answer questions specific to the condition or problems of particular individuals, nor in ________________________ any way to substitute for the professional advice and care of qualified physicians. Mention of particular drugs and/or treatments is for ________________________ information purposes only and does not constitute an endorsement of said drugs and/or treatments. ________________________ Thanks! ________________________ The Scleroderma Foundation expresses its deep appreciation to the many ________________________ physicians whose efforts have led to this booklet. Special thanks are owed to Maureen D. Mayes, M.D., M.P.H., of the ________________________ University of Texas McGovern Medical School, Houston. -
Dermatological Findings in Common Rheumatologic Diseases in Children
Available online at www.medicinescience.org Medicine Science ORIGINAL RESEARCH International Medical Journal Medicine Science 2019; ( ): Dermatological findings in common rheumatologic diseases in children 1Melike Kibar Ozturk ORCID:0000-0002-5757-8247 1Ilkin Zindanci ORCID:0000-0003-4354-9899 2Betul Sozeri ORCID:0000-0003-0358-6409 1Umraniye Training and Research Hospital, Department of Dermatology, Istanbul, Turkey. 2Umraniye Training and Research Hospital, Department of Child Rheumatology, Istanbul, Turkey Received 01 November 2018; Accepted 19 November 2018 Available online 21.01.2019 with doi:10.5455/medscience.2018.07.8966 Copyright © 2019 by authors and Medicine Science Publishing Inc. Abstract The aim of this study is to outline the common dermatological findings in pediatric rheumatologic diseases. A total of 45 patients, nineteen with juvenile idiopathic arthritis (JIA), eight with Familial Mediterranean Fever (FMF), six with scleroderma (SSc), seven with systemic lupus erythematosus (SLE), and five with dermatomyositis (DM) were included. Control group for JIA consisted of randomly chosen 19 healthy subjects of the same age and gender. The age, sex, duration of disease, site and type of lesions on skin, nails and scalp and systemic drug use were recorded. χ2 test was used. The most common skin findings in patients with psoriatic JIA were flexural psoriatic lesions, the most common nail findings were periungual desquamation and distal onycholysis, while the most common scalp findings were erythema and scaling. The most common skin finding in patients with oligoarthritis was photosensitivity, while the most common nail finding was periungual erythema, and the most common scalp findings were erythema and scaling. We saw urticarial rash, dermatographism, nail pitting and telogen effluvium in one patient with systemic arthritis; and photosensitivity, livedo reticularis and periungual erythema in another patient with RF-negative polyarthritis. -
Eye Findings in Patients with Juvenile Dermatomyositis JONATHAN D
Eye Findings in Patients with Juvenile Dermatomyositis JONATHAN D. AKIKUSA, DHENUKA K. TENNANKORE, ALEX V. LEVIN, and BRIAN M. FELDMAN ABSTRACT. Objective. Reports of eye involvement in juvenile dermatomyositis (JDM), including significant retinopathy with visual loss, have led some to recommend routine formal ophthalmologic assess- ments for all patients with JDM at diagnosis. Our objective was to document the frequency and spec- trum of eye involvement in patients followed in a single clinic caring for children with JDM. Methods. A chart review was conducted of formal ophthalmologic consultation notes for patients with JDM followed at the Hospital for Sick Children between 1981 and 2002. Results. Ophthalmologic assessments were found for 82 of 108 patients with JDM. The mean age at diagnosis of JDM was 7.0 years and 68.3% were female. Forty-five patients (55.6%) had abnormal eye examinations. Lid manifestations, found in 37 patients (45.7%), were the most common abnor- mality. Fourteen patients (17.1%) had corticosteroid-induced cataracts. Two patients had retinal abnormalities; one had a small retinal hemorrhage, the other an incidental chorioretinal scar. Neither had impairment of vision. No patient had uveitis. Conclusion. Eyelid and lens abnormalities are common in patients with JDM, while retinopathy is rare. As lid lesions and cataracts are easily detected by non-ophthalmologists, and retinal lesions are rare, we feel that JDM patients without visual symptoms do not require routine formal ophthalmo- logic assessment for disease manifestations. (J Rheumatol 2005;32:1986–91) Key Indexing Terms: JUVENILE DERMATOMYOSITIS RETINOPATHY CATARACTS HELIOTROPE Juvenile dermatomyositis (JDM) is a systemic inflammato- described in adult patients with DM11-13, in some cases pro- ry vasculopathy in which the predominant clinical manifes- ducing permanent visual impairment11,13. -
Visual Recognition of Autoimmune Connective Tissue Diseases
Seeing the Signs: Visual Recognition of Autoimmune Connective Tissue Diseases Utah Association of Family Practitioners CME Meeting at Snowbird, UT 1:00-1:30 pm, Saturday, February 13, 2016 Snowbird/Alta Rick Sontheimer, M.D. Professor of Dermatology Univ. of Utah School of Medicine Potential Conflicts of Interest 2016 • Consultant • Paid speaker – Centocor (Remicade- – Winthrop (Sanofi) infliximab) • Plaquenil – Genentech (Raptiva- (hydroxychloroquine) efalizumab) – Amgen (etanercept-Enbrel) – Alexion (eculizumab) – Connetics/Stiefel – MediQuest • Royalties Therapeutics – Lippincott, – P&G (ChelaDerm) Williams – Celgene* & Wilkins* – Sanofi/Biogen* – Clearview Health* Partners • 3Gen – Research partner *Active within past 5 years Learning Objectives • Compare and contrast the presenting and Hallmark cutaneous manifestations of lupus erythematosus and dermatomyositis • Compare and contrast the presenting and Hallmark cutaneous manifestations of morphea and systemic sclerosis Distinguishing the Cutaneous Manifestations of LE and DM Skin involvement is 2nd most prevalent clinical manifestation of SLE and 2nd most common presenting clinical manifestation Comprehensive List of Skin Lesions Associated with LE LE-SPECIFIC LE-NONSPECIFIC Cutaneous vascular disease Acute Cutaneous LE Vasculitis Leukocytoclastic Localized ACLE Palpable purpura Urticarial vasculitis Generalized ACLE Periarteritis nodosa-like Ten-like ACLE Vasculopathy Dego's disease-like Subacute Cutaneous LE Atrophy blanche-like Periungual telangiectasia Annular Livedo reticularis -
Juvenile Dermatomyositis - a Case Report with Review on Oral Manifestations and Oral Health Considerations
Volume 44 Number 1 pp. 52-61 2018 Case Report Juvenile dermatomyositis - A case report with review on oral manifestations and oral health considerations Pritesh Ruparelia ([email protected]) Oshin Verma ([email protected]) Vrutti Shah ([email protected]) Krishna Shah ([email protected]) Follow this and additional works at: https://ijom.iaom.com/journal The journal in which this article appears is hosted on Digital Commons, an Elsevier platform. Suggested Citation Ruparelia, P., et al. (2018). Juvenile dermatomyositis - A case report with review on oral manifestations and oral health considerations. International Journal of Orofacial Myology, 44(1), 52-61. DOI: https://doi.org/10.52010/ijom.2018.44.1.4 This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. The views expressed in this article are those of the authors and do not necessarily reflect the policies or positions of the International Association of Orofacial Myology (IAOM). Identification of specific oducts,pr programs, or equipment does not constitute or imply endorsement by the authors or the IAOM. International Journal of Orofacial Myology 2018, V44 JUVENILE DERMATOMYOSITIS - A CASE REPORT WITH REVIEW ON ORAL MANIFESTATIONS AND ORAL HEALTH CONSIDERATIONS. PRITESH RUPARELIA, MDS, OSHIN VERMA, BDS, VRUTTI SHAH, BDS, KRISHNA SHAH, BDS ABSTRACT Juvenile Dermatomyositis is the most common inflammatory myositis in children, distinguished by proximal muscle weakness, a characteristic rash and Gottron’s papules. The oral lesions most commonly manifest as diffuse stomatitis and pharyngitis with halitosis. We report a case of an 8 year old male with proximal muscle weakness of all four limbs, rash, Gottron’s papules and oral manifestations. -
A Acanthosis Nigricans, 139 Acquired Ichthyosis, 53, 126, 127, 159 Acute
Index A Anti-EJ, 213, 214, 216 Acanthosis nigricans, 139 Anti-Ferc, 217 Acquired ichthyosis, 53, 126, 127, 159 Antigliadin antibodies, 336 Acute interstitial pneumonia (AIP), 79, 81 Antihistamines, 324 Adenocarcinoma, 115, 116, 151, 173 Anti-histidyl-tRNA-synthetase antibody Adenosine triphosphate (ATP), 229 (Anti-Jo-1), 6, 14, 140, 166, 183, Adhesion molecules, 225–226 213–216 Adrenal gland carcinoma, 115 Anti-histone antibodies (AHA), 174, 217 Age, 30–32, 157–159 Anti-Jo-1 antibody syndrome, 34, 129 Alanine aminotransferase (ALT, ALAT), 16, Anti-Ki-67 antibody, 247 128, 205, 207, 255 Anti-KJ antibodies, 216–217 Alanyl-tRNA synthetase, 216 Anti-KS, 82 Aldolase, 14, 16, 128, 129, 205, 207, 255, 257 Anti-Ku antibodies, 163, 165, 217 Aledronate, 325 Anti-Mas, 217 Algorithm, 256, 259 Anti-Mi-2 Allergic contact dermatitis, 261 antibody syndrome, 11, 129, 215 Alopecia, 62, 199, 290 antibodies, 6, 15, 129, 142, 212 Aluminum hydroxide, 325, 326 Anti-Myo 22/25 antibodies, 217 Alzheimer’s disease-related proteins, 190 Anti-Myosin scintigraphy, 230 Aminoacyl-tRNA synthetases, 151, 166, 182, Antineoplastic agents, 172 212, 215 Antineoplastic medicines, 169 Aminoquinolone antimalarials, 309–310, 323 Antinuclear antibody (ANA), 1, 141, 152, 171, Amyloid, 188–190 172, 174, 213, 217 Amyopathic DM, 6, 9, 29–30, 32–33, 36, 104, Anti-OJ, 213–214, 216 116, 117, 147–153 Anti-p155, 214–215 Amyotrophic lateral sclerosis, 263 Antiphospholipid syndrome (APS), 127, Antisynthetase syndrome, 11, 33–34, 81 130, 219 Anaphylaxi, 316 Anti-PL-7 antibody, 82, 214 Anasarca, -
Adverse Drug Reactions Associated with Treatment in Patients With
Said et al. Advances in Rheumatology (2020) 60:53 Advances in Rheumatology https://doi.org/10.1186/s42358-020-00154-4 RESEARCH Open Access Adverse drug reactions associated with treatment in patients with chronic rheumatic diseases in childhood: a retrospective real life review of a single center cohort Manar Amanouil Said* , Liana Soido Teixeira e Silva, Aline Maria de Oliveira Rocha, Gustavo Guimarães Barreto Alves, Daniela Gerent Petry Piotto, Claudio Arnaldo Len and Maria Teresa Terreri Abstract Background: Adverse drug reactions (ADRs) are the sixth leading causes of death worldwide; monitoring them is fundamental, especially in patients with disorders like chronic rheumatic diseases (CRDs). The study aimed to describe the ADRs investigating their severity and associated factors and resulting interventions in pediatric patients with CRDs. Methods: A retrospective, descriptive and analytical study was conducted on a cohort of children and adolescents with juvenile idiopathic arthritis (JIA), juvenile systemic lupus erythematosus (JSLE) and juvenile dermatomyositis (JDM). The study evaluated medical records of the patients to determine the causality and the management of ADRs. In order to investigate the risk factors that would increase the risk of ADRs, a logistic regression model was carried out on a group of patients treated with the main used drug. Results: We observed 949 ADRs in 547 patients studied. Methotrexate (MTX) was the most frequently used medication and also the cause of the most ADRs, which occurred in 63.3% of patients, followed by glucocorticoids (GCs). Comparing synthetic disease-modifying anti-rheumatic drugs (sDMARDs) vs biologic disease-modifying anti- rheumatic drugs (bDMARDs), the ADRs attributed to the former were by far higher than the latter. -
Dermatomyositis: Observations on the Use of Cairo-Glasgow Study Group
Postgrad Med J: first published as 10.1136/pgmj.54.634.516 on 1 August 1978. Downloaded from Postgraduate Medical Journal (August 1978) 54, 516-527. Dermatomyositis: observations on the use of immunosuppressive therapy and review of literature Cairo-Glasgow Study Group AHMED EL- GHOBAREY GEZA BALINT M.R.C.P. M.D. (Budapest) KAREL DE CEULAER W. CARSON DICK M.D. (Leuven) M.D., M.R.C.P. W. W. BUCHANAN M.D., F.R.C.P. TAHSIN HADIDI* T. A. HASSAN* F.R.C.P. M.R.C.P. The Centre for Rheumatic Diseases, University Department ofMedicine, Royal Infirmary, Protected by copyright. Glasgow, Scotland, and *Maadi Armed Forces Hospital, and Azhar University, Cairo, Egypt Summary The response to therapy and the subsequent Seven young adults, six of whom were male, all clinical course are briefly described as follows. suffering from dermatomyositis unassociated with Patient N.D. was treated with corticosteroids. The malignancy are described. These patients were not course of therapy, clinical response and changes in adequately controlled with high doses of corti- serum muscle enzymes are summarized in Fig. 1. costeroids but all responded when immunosuppressive Intravenous dexamethasone was discontinued at the therapy was also given. nineteenth week and by the end of the twenty-first week the dose of prednisolone was reduced to 10 mg/ day. Introduction http://pmj.bmj.com/ Dermatomyositis (as the name suggests) is a The patient was discharged at the end of the syndrome consisting of polymyositis associ- twenty-fourth week. clinical One year later, the patient had a relapse, the ated with skin lesions (Pearson, 1966a; Currie and subsequent course and response to treatment are Walton, 1971). -
Collagen Vascular Diseases: SLE, Dermatomyositis, Scleroderma, and MCTD
Collagen Vascular Diseases: SLE, Dermatomyositis, Scleroderma, and MCTD Richard K. Vehe, MD,* Mona M. Riskalla, MD* *Division of Pediatric Rheumatology, Department of Pediatrics, University of Minnesota and the University of Minnesota Masonic Children’s Hospital, Minneapolis, MN Practice Gap Timely and accurate recognition of collagen vascular disorders (CVDs), and implementation of effective screening and referral processes for patients suspected of having a CVD, remain a challenge for many physicians. The result, too often, is unnecessary testing and referrals, and in some cases unnecessary anxiety for physicians, patients, and parents. Objectives After completing this article, readers should be able to: 1. Recognize the common clinical symptoms and signs of systemic lupus erythematosus, dermatomyositis, and scleroderma, and their distinction from common infectious mimics. 2. Recognize the testing that can clarify the likelihood of whether a child has a rheumatic disease, including the limited utility of early serologic testing for autoantibodies. 3. Recognize the prognosis and management objectives for these often- chronic disorders, and practical steps to ensure excellent outcomes. AUTHOR DISCLOSURE Drs Vehe and Riskalla have disclosed no financial relationships relevant to this article. This commentary does not contain a discussion of an unapproved/ INTRODUCTION investigative use of a commercial product/ device. Timely and accurate recognition of collagen vascular diseases (CVDs), and ABBREVIATIONS implementation of effective screening and referral processes for patients sus- ANA antinuclear antibody pected of having a CVD, remain challenging for many physicians. The result, too CTD connective tissue disease often, is unnecessary testing with questionable results, leading in some cases to CVD collagen vascular disease unnecessary referrals and anxiety for physicians, patients, and parents. -
Autoantibodies in Myositis
Autoantibodies in Myositis Neil McHugh University of Bath and Royal National Hospital for Rheumatic Diseases Bath UK Idiopathic Inflammatory Myositis Spectrum Disease Muscle inflammation Skin disorder Interstitial lung disease Myositis spectrum disease autoantibodies (MSDA)! Myositis Spectrum Disease • Polymyositis • Anti-synthetase syndrome • Immune-mediated necrotising myopathy • Dermatomyositis • Clinically amyopathic dermatomyositis (CADM) • Cancer associated myositis (CAM) • Inclusion Body Myositis • Juvenile Dermatomyositis • Myositis associated with connective tissue disease • Otherj • Granulomatous, eosinophilic, focal, orbital, macrophagic, myofasciitis Autoantibodies in Myositis Spectrum Disease • MSDA (myositis spectrum • MAA (myositis associated disorder autoantibodies) autoantibodies) • Anti-tRNA synthetases (e.g. • Anti-PM-Scl anti-Jo-1) • • Anti-Mi-2 Anti-U1RNP • Anti-signal recognition • Anti-Ku particle • Anti-U3RNP • Anti-SAE • Anti-Ro (SSA) • Anti-TIF1-g • Anti-NXP2 • Anti-MDA5 • Anti-HMGCR • Anti-cN-1A MSDAs and target autoantigens I Autoantibodies Target autoantigen Autoantigen function Clinical phenotype Anti-ARS tRNA synthetase Intracytoplasmic protein ASS Anti-Jo-1 Histidyl synthesis Myositis Anti-PL-7 Threonyl Binding between an amino Interstitial pneumonia Anti-PL-12 Alanyl acid and its cognate tRNA Mechanics hands Anti-EJ Glycyl Arthritis Anti-OJ Isoleucyl Anti-KS Asparaginyl Fever Anti-Zo Phenylalanyl Raynauds Anti-YRS Tyrosyl Anti-Mi-2 Helicase protein part of the Nuclear transcription Adult and juvenile DM -
Childhood-Onset Systemic Lupus Erythematosus: a Review and Update
MEDICAL www.jpeds.com • THE JOURNAL OF PEDIATRICS PROGRESS Childhood-Onset Systemic Lupus Erythematosus: A Review and Update Onengiya Harry, MD, MPH†, Shima Yasin, MD, MSc†, and Hermine Brunner, MD, MSc, MBA, FAAP, FACR upus is a chronic, autoimmune multisystem inflam- Genetic Factors matory disease that is associated with sizable morbid- There is a 10-fold increase in SLE risk among monozygotic as L ity and mortality.1 When lupus commences in an compared to dizygotic twins.15,16 Further, siblings of a patient individual less than 18 years of age,2 it is commonly referred with SLE carry an 8- to 20-fold higher risk of developing SLE to as childhood-onset systemic lupus erythematosus (cSLE). as compared with a healthy general population.16,17 With a reported incidence of 0.3-0.9 per 100 000 children per SLE is considered a polygenic disease, although rare mono- year, and a prevalence of 3.3-24 per 100 000 children,3 cSLE genic causes have been described recently.18 Genetic variants is rare. About 10%-20% of all patients with SLE are diag- that are well-established include very rare mutations in genes nosed during childhood. Typically, cSLE has a more severe clini- coding for select complement factors. Indeed, a single gene mu- cal course than that seen in adults, with a higher prevalence tation that results in a complete deficiency of C1q increases of lupus nephritis, hematologic anomalies, photosensitivity, neu- the risk of SLE, or lupus-like symptoms, to more than 90%. ropsychiatric, and mucocutaneous involvement.3-5 C4 deficiency is also -
Juvenile Dermatomyositis: Novel Treatment Approaches and Outcomes
REVIEW CURRENT OPINION Juvenile dermatomyositis: novel treatment approaches and outcomes Giulia C. Varniera, Clarissa A. Pilkingtona, and Lucy R. Wedderburna,b,c Purpose of review The aim of this article is to provide a summary of the recent therapeutic advances and the latest research on outcome measures for juvenile dermatomyositis (JDM). 04/06/2021 on BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3i3D0OdRyi7TvSFl4Cf3VC4/OAVpDDa8KKGKV0Ymy+78= by http://journals.lww.com/co-rheumatology from Downloaded Downloaded Recent findings Several new international studies have developed consensus-based guidelines on diagnosis, outcome from measures and treatment of JDM to standardize and improve patient care. Myositis-specific antibodies http://journals.lww.com/co-rheumatology together with muscle biopsy histopathology may help the clinician to predict disease outcome. A newly developed MRI-based scoring system has been developed to standardize the use of MRI in assessing disease activity in JDM. New data regarding the efficacy and safety of rituximab, especially for skin disease, and cyclophosphamide in JDM support the use of these medications for severe refractory cases. Summary International network studies, new biomarkers and outcome measures have led to significant progress in by BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3i3D0OdRyi7TvSFl4Cf3VC4/OAVpDDa8KKGKV0Ymy+78= understanding and managing the rare inflammatory myositis conditions such as JDM. Keywords advanced treatment, juvenile dermatomyositis, outcome measures INTRODUCTION To overcome these issues, these two international Juvenile dermatomyositis (JDM) is a rare systemic organizations joined forces and developed a new set autoimmune disease characterized by a vasculopathy of consensus-driven response criteria for adult that primarily affects muscle and skin, but may dermatomyositis/polymyositis and children with involve the lung, bowel, heart and other organs JDM.