Juvenile Dermatomyositis


Article Author:
Soumaya Gara
Radia Jamil


Article Editor:
Noureddine Litaiem


Editors In Chief:
Amanda Oakley
Jules Lipoff
Shyam Verma


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Frank Smeeks
Kristina Soman-Faulkner
Trevor Nezwek
Radia Jamil
Patrick Le
Sobhan Daneshfar
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Pritesh Sheth
Hassam Zulfiqar
Navid Mahabadi
Steve Bhimji
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Mark Pellegrini
James Hughes
Beata Beatty
Nazia Sadiq
Hajira Basit
Phillip Hynes
Tehmina Warsi


Updated:
6/6/2019 5:18:47 PM

Introduction

Dermatomyositis is a rare autoimmune inflammatory myositis of unknown etiology affecting both children and adults. It involves striated muscles and skin. The juvenile form is associated with a multisystemic vasculitis and a high frequency of calcinosis. However, unlike the adult form, it does not have an increased risk of malignancy.[1]

Etiology

Dermatomyositis is an inflammatory myopathy distinguished by a mononuclear inflammatory infiltrate of the striated muscle. Genetic susceptibility is no longer in doubt since predisposing HLA systems have been identified. There is an activation of the complement leading to the deposition of the membrane attack complex in the wall of blood vessels, which causes microangiopathy and an inflammatory reaction.

Epidemiology

The mean age at onset of the disease is around 7 years of age, with earlier onset in girls. The mean time between the onset of the first symptoms and confirming the diagnosis is 6 months, ranging from 5 weeks to 2 years.

History and Physical

In juvenile dermatomyositis (JDM), the clinical features are usually insidious. Muscle weakness is the main reason for consultation, usually accompanied by systemic signs like asthenia, anorexia, irritability, pain, fever, and deterioration of one's general condition. The dermatological manifestations are like those of adults. They are highly characteristic. Their recognition is essential because they can precede muscular signs. The periorbital violaceous patches like frames of glasses are almost pathognomonic as well as Gottron's papules.[2][3] These latter are found in 30% to 70% of cases and are easy to recognize. Violaceous papules are usually seen over the metacarpophalangeal, the proximal and distal interphalangeal joints of hands, less commonly, in the extensor surfaces of elbows and knees. We can also observe painful periungual erythema with telangiectasia, cuticular hypertrophy, facial edema, erythematous patches involving the "V-neck," shoulders, and anterior chest wall and Raynaud's phenomenon. More rarely, there are skin lesions of cutaneous vasculitis and photosensitivity. The muscular deficit is usually progressive, symmetric, bilateral, proximal and non-selective, involving striated muscles of pelvic and shoulders. The intensity of the muscle weakness varies from one patient to another, ranging from simple myalgia to a severe muscle deficit. Children may report difficulties in dressing, combing hair, and climbing stairs. During evolution, calcinosis may occur more commonly in children than in adults. These calcifications arise from subcutaneous tissues or deeper locations such as musculoaponeurotic structures. The commonest sites of calcinosis include elbows, knees, buttocks and trauma areas. Its evolution is unpredictable. It can regress spontaneously, ulcerate with a risk of secondary infections causing major aesthetic and functional sequelae. It can mimic cold abscesses.[4] No standardized approach to the management of calcinosis associated with JDM has been established. Its incidence may decrease with early treatment by systemic corticosteroid therapy at high doses. Experienced physicians are more likely to use bisphosphonate as a first-line treatment.[5][6] The involvement of the pharyngeal and esophageal muscles is responsible for dysphonia, dysphagia, and dyspnea. The vital prognosis is affected. Transferring the patient for admission to the intensive care unit is necessary to initiate appropriate treatment.[7] Amyopathic forms of JDM are rare.[8] They are characterized by typical skin manifestations without muscle involvement. Serum muscle enzymes, electromyographic examination, and muscle biopsy are normal. There is usually no calcinosis or vasculopathy. The prognosis is excellent.

Myocardial involvement may cause ventricular arrhythmias. Lipodystrophy usually occurs after a long evolution. The fat loss is slow and progressive, typically affecting the upper part of the trunk. It can be associated with hirsutism, hepatomegaly and acanthosis nigricans lesions. JDM is associated with a high risk of cardiovascular and cerebrovascular comorbidities.[9] Pulmonary manifestations include interstitial lung disease and respiratory muscles involvement.[10]

Evaluation

Abnormal elevation of serum activities of muscle enzymes is the most common biological finding in JDM.  Elevation of creatine kinase is expected in 75% to 85% of cases with average values around 2000 U/L plus or minus 1000. A normal rate can be observed at the beginning of the disease and in amyopathic forms. Regular creatine phosphokinase (CPK) monitoring is recommended during the evolution under treatment to evaluate its effectiveness. The electromyogram shows a myogenic pattern. Muscle biopsy confirms the diagnosis. Some histological abnormalities are common to other myositides: myofiber degeneration and an inflammatory mononuclear infiltrate. Other findings are more specific, for example, a highly characteristic perifascicular atrophy, perivascular inflammatory infiltrate, and sometimes intravascular microthrombi. Magnetic resonance imaging (MRI) is a non-invasive tool used to guide the site of the muscle biopsy to optimize its sensitivity. Muscle edema is the major abnormality observed on MRI, particularly in T2 sequence with fat suppression or STIR sequence. This edema is usually proximal and symmetrical but may be focal and distal. The signal intensity seems to be correlated with an aggressive chronic disease course.[11]

Treatment / Management

Systemic corticosteroids are the established primary treatment for the management of JDM. As yet, there is no consensus regarding the dose regimen and the therapeutic modalities. It is recommended to initiate the treatment with 1 milligram per kilogram of daily equivalent prednisone. Intravenous (IV) treatment is preferable in the case of gastrointestinal (GI) involvement. This late is known to be associated with vasculitis causing malabsorption. Long-term steroid treatment is usually required to achieve complete remission. It should be gradually decreased. Adjunctive hygienic-dietary measures, as well as daily supplementation with calcium and vitamin D,  must be systematic. However, these children are at high risk of osteoporosis.

In cases of therapeutic failure or significant side effects under steroid therapy, the use of subcutaneous methotrexate should be considered. Combining corticosteroids and methotrexate is a good alternative to control the disease activity without significant adverse reaction.[12] Using hydroxychloroquine may be a new therapeutic approach. Targeted biotherapies (infliximab, rituximab) may be indicated in refractory forms of JDM. Only controlled and randomized clinical trials will be able to validate their safety in children.[13] The therapeutic abstention in amyopathic JDM is the rule. In this case, patients generally remain asymptomatic even after several years of evolution. Physical therapy is an important part of the treatment. It should be started as soon as symptoms are under control. It aims to rebuild muscle strength and to prevent joint contraction.[14]

Prognosis

The evolution is unpredictable. The disease may have a monocyclic, polycyclic or chronic course. The prognosis has significantly improved over recent years with the emergence of new therapeutic options. Early treatment and close monitoring may lead to better control of the disease. However, developing countries still have a high mortality rate.[15] The major causes of death are usually severe muscle weakness, super-added infection, gastrointestinal vasculitis with a risk of bowel perforation, myocardial failure, and respiratory distress. JDM is usually not associated with the development of malignancies, unlike adult form.[16]

Deterrence and Patient Education

Having a child with JDM may impact the family quality of life negatively.[17] Psychological assistance is necessary for the child and his or her parents.

Enhancing Healthcare Team Outcomes

Dermatomyositis is a rare autoimmune inflammatory myositis of unknown etiology affecting both children and adults. It involves striated muscles and skin. The juvenile form is associated with a multisystemic vasculitis and a high frequency of calcinosis. However, unlike the adult form, it does not have an increased risk of malignancy.[1]  The disorder is best managed by a multidisciplinary team that includes a rheumatologist, dermatologist, neurologist, a gastroenterologist and an internist. Once the disorder is diagnosed, it progression is unpredictable but with treatment, the symptoms can be managed. However, delay in diagnosis can lead to premature death. The major causes of death are usually severe muscle weakness, super-added infection, gastrointestinal vasculitis with a risk of bowel perforation, myocardial failure, and respiratory distress. JDM is usually not associated with the development of malignancies, unlike adult form.[16]


Interested in Participating?

We are looking for contributors to author, edit, and peer review our vast library of review articles and multiple choice questions. In as little as 2-3 hours you can make a significant contribution to your specialty. In return for a small amount of your time, you will receive free access to all content and you will be published as an author or editor in eBooks, apps, online CME/CE activities, and an online Learning Management System for students, teachers, and program directors that allows access to review materials in over 500 specialties.

Improve Content - Become an Author or Editor

This is an academic project designed to provide inexpensive peer-reviewed Apps, eBooks, and very soon an online CME/CE system to help students identify weaknesses and improve knowledge. We would like you to consider being an author or editor. Please click here to learn more. Thank you for you for your interest, the StatPearls Publishing Editorial Team.

Juvenile Dermatomyositis - Questions

Take a quiz of the questions on this article.

Take Quiz
Which of the following infections is not in the differential diagnosis of juvenile dermatomyositis and include prominent muscular symptoms?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Which autoantibody is not associated with juvenile dermatomyositis?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
How is the spontaneous course of juvenile dermatomyositis characterized prior to the advent of corticosteroids?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A child presents with a malar rash, Raynaud phenomenon, interstitial lung disease, arthralgia, and polymyositis. Screening for autoantibodies was positive for anti-U1-RNP. What is the most likely diagnosis?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Which is not a treatment of juvenile dermatomyositis?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up

Juvenile Dermatomyositis - References

References

Lundberg IE,Tjärnlund A,Bottai M,Werth VP,Pilkington C,Visser M,Alfredsson L,Amato AA,Barohn RJ,Liang MH,Singh JA,Aggarwal R,Arnardottir S,Chinoy H,Cooper RG,Dankó K,Dimachkie MM,Feldman BM,Torre IG,Gordon P,Hayashi T,Katz JD,Kohsaka H,Lachenbruch PA,Lang BA,Li Y,Oddis CV,Olesinska M,Reed AM,Rutkowska-Sak L,Sanner H,Selva-O'Callaghan A,Song YW,Vencovsky J,Ytterberg SR,Miller FW,Rider LG, 2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Annals of the rheumatic diseases. 2017 Dec     [PubMed]
Sukumaran S,Palmer T,Vijayan V, Heliotrope Rash and Gottron Papules in a Child with Juvenile Dermatomyositis. The Journal of pediatrics. 2016 Apr     [PubMed]
Kumar A,Gupta A,Suri D,Gupta A,Singh S, The Expanding Spectrum of Gottron Papules in Juvenile Dermatomyositis. Indian journal of pediatrics. 2017 Mar     [PubMed]
Nagar RP,Bharati J,Sheriff A,Priyadarshini P,Chumber S,Kabra SK, Calcinosis in juvenile dermatomyositis mimicking cold abscess. The National medical journal of India. 2016 Mar-Apr     [PubMed]
Orandi AB,Baszis KW,Dharnidharka VR,Huber AM,Hoeltzel MF, Assessment, classification and treatment of calcinosis as a complication of juvenile dermatomyositis: a survey of pediatric rheumatologists by the childhood arthritis and rheumatology research alliance (CARRA). Pediatric rheumatology online journal. 2017 Sep 21     [PubMed]
Saini I,Kalaivani M,Kabra SK, Calcinosis in juvenile dermatomyositis: frequency, risk factors and outcome. Rheumatology international. 2016 Jul     [PubMed]
Besançon A,Brochard K,Dupic L,Gitiaux C,Delville M,Krid S,Quartier P,Saire E,Salomon R,Talbotec C,Bodemer C,Bader-Meunier B, Presentations and outcomes of juvenile dermatomyositis patients admitted to intensive care units. Rheumatology (Oxford, England). 2017 Oct 1     [PubMed]
Oberle EJ,Bayer ML,Chiu YE,Co DO, How Often are Pediatric Patients with Clinically Amyopathic Dermatomyositis Truly Amyopathic? Pediatric dermatology. 2017 Jan     [PubMed]
Ladd PE,Emery KH,Salisbury SR,Laor T,Lovell DJ,Bove KE, Juvenile dermatomyositis: correlation of MRI at presentation with clinical outcome. AJR. American journal of roentgenology. 2011 Jul     [PubMed]
Silverberg JI,Kwa L,Kwa MC,Laumann AE,Ardalan K, Cardiovascular and cerebrovascular comorbidities of juvenile dermatomyositis in US children: an analysis of the National Inpatient Sample. Rheumatology (Oxford, England). 2018 Apr 1     [PubMed]
Pouessel G,Deschildre A,Le Bourgeois M,Cuisset JM,Catteau B,Karila C,Nève V,Thumerelle C,Quartier P,Tillie-Leblond I, The lung is involved in juvenile dermatomyositis. Pediatric pulmonology. 2013 Oct     [PubMed]
McCann LJ,Pain CE, A Practical Approach to Juvenile Dermatomyositis and Juvenile Scleroderma. Indian journal of pediatrics. 2016 Feb     [PubMed]
Aggarwal R,Loganathan P,Koontz D,Qi Z,Reed AM,Oddis CV, Cutaneous improvement in refractory adult and juvenile dermatomyositis after treatment with rituximab. Rheumatology (Oxford, England). 2017 Feb     [PubMed]
Papadopoulou C,Wedderburn LR, Treatment of Juvenile Dermatomyositis: An Update. Paediatric drugs. 2017 Oct     [PubMed]
Singh S,Suri D,Aulakh R,Gupta A,Rawat A,Kumar RM, Mortality in children with juvenile dermatomyositis: two decades of experience from a single tertiary care centre in North India. Clinical rheumatology. 2014 Nov     [PubMed]
Morris P,Dare J, Juvenile dermatomyositis as a paraneoplastic phenomenon: an update. Journal of pediatric hematology/oncology. 2010 Apr     [PubMed]
Kountz-Edwards S,Aoki C,Gannon C,Gomez R,Cordova M,Packman W, The family impact of caring for a child with juvenile dermatomyositis. Chronic illness. 2017 Dec     [PubMed]

Disclaimer

The intent of StatPearls is to provide practice questions and explanations to assist you in identifying and resolving knowledge deficits. These questions and explanations are not intended to be a source of the knowledge base of all of medicine, nor is it intended to be a board or certification review of Dermatology. The authors or editors do not warrant the information is complete or accurate. The reader is encouraged to verify each answer and explanation in several references. All drug indications and dosages should be verified before administration.

StatPearls offers the most comprehensive database of free multiple-choice questions with explanations and short review chapters ever developed. This system helps physicians, medical students, dentists, nurses, pharmacists, and allied health professionals identify education deficits and learn new concepts. StatPearls is not a board or certification review system for Dermatology, it is a learning system that you can use to help improve your knowledge base of medicine for life-long learning. StatPearls will help you identify your weaknesses so that when you are ready to study for a board or certification exam in Dermatology, you will already be prepared.

Our content is updated continuously through a multi-step peer review process that will help you be prepared and review for a thorough knowledge of Dermatology. When it is time for the Dermatology board and certification exam, you will already be ready. Besides online study quizzes, we also publish our peer-reviewed content in eBooks and mobile Apps. We also offer inexpensive CME/CE, so our content can be used to attain education credits while you study Dermatology.