Dermatomyositis

doi:10.1016/S0022-3476(64)80325-5

The Journal of Pediatrics

Volume 64, Issue 1, January 1964, Pages 101-131

https://doi.org/10.1016/S0022-3476(64)80325-5 Get rights and content

Dermatomyositis, a syndrome closely related to the other connective tissue diseases has a wide clinical spectrum involving primarily muscle at the one end, but presenting a more generalized disorder with skin and visceral involvement at the other. Multiple etiologic agents probably exist, but the present series of 13 children implicated unknown agricultural factors. The diagnosis is essentially a clinical one, the laboratory findings being either nonspecific or unreliable. Renal biopsy revealed striking but nonspecific changes in all 6 patients studied, often without other evidence of renal disease. Current therapy does not contribute significantly to the prognosis, although steroids frequently yield dramatic results in the acute stage, and mechanical respiratory aids may be life-saving.

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Cited by (140)

The clinical features of juvenile dermatomyositis: A single-centre inception cohort
2022, Seminars in Arthritis and Rheumatism
Juvenile Dermatomyositis (JDM), a severe and rare autoimmune disease, is the most common idiopathic inflammatory myopathy in children. We describe the clinical features of a large single-centre cohort.
We studied an inception cohort (0-18 years old) referred for diagnosis to the JDM clinic at The Hospital for Sick Children (SickKids), between January 1989 and September 2017. Probable or definite diagnosis of JDM was done according to the 2017 ACR/EULAR Criteria. We excluded children who had treatment started at another hospital. The data were collected retrospectively from clinical charts and the SickKids JDM database.
172/230 (74.8%) patients were included. They were most often female (female:male = 1.8:1); the age at diagnosis was 8.5±4.3 years. There was a positive family history for autoimmune disease in 52%, mainly rheumatoid arthritis. No patient died. The most common signs at inception were muscle weakness (85.5%), nailfold capillary abnormalities (83.4%), Gottron papules (78.5%), heliotrope rash (66.3%), abnormal gait (55.8%), and malar/facial rash (54.7%). The prevalence of Gottron papules, heliotrope rash, facial/malar rash, nailfold capillary abnormalities, Raynaud phenomenon, dysphonia/dysphagia (a frequent cause of hospitalization), mouth ulcers, calcinosis, eye problems, joint involvement, acanthosis nigricans and lipodystrophy increased during follow-up. Muscle enzymes, namely CK, ALT, AST, were often normal or only slightly raised despite active muscle disease; conversely LD was often high. Anti-Nuclear Autoantibodies were positive in 49.7% of patients at diagnosis. The course of the disease was: 29.1% monocyclic, 5.3% polycyclic, 33.1% chronic. The course of 56 patients (32.5%) was not classifiable due to length of follow-up. Corticosteroids were used as treatment in almost all our patients and 30% required intravenous therapy due to the severity of the presentation; methotrexate was added in 64%, more often in recent years. Unresponsive patients were treated mostly with intravenous immunoglobulins (IVIG).
The information obtained from this relatively large number of patients adds to the growing knowledge base of this rare disease.
SickKids Research Ethics Board approved the study.
50 Years Ago in THE JOURNAL OF PEDIATRICS: What Changed the Prognosis of Juvenile Dermatomyositis?
2022, Journal of Pediatrics
Updates on Juvenile Dermatomyositis from the Last Decade: Classification to Outcomes
2021, Rheumatic Disease Clinics of North America
Citation Excerpt :
The high frequency of damage in these cohorts point to the urgent need for better JDM treatments. Although mortality rates for JDM have improved since the 1960s, when it was reported in one-third of patients,117 current rates remain unacceptably high. The JDM specific mortality rate from the National Institutes of Health natural history questionnaire–based protocols administered between 1989 and 2011 was estimated at 2.4%, most commonly attributed to ILD.118
Juvenile Idiopathic Inflammatory Myopathies
2018, Pediatric Clinics of North America
Advances in Juvenile Dermatomyositis: Myositis Specific Antibodies Aid in Understanding Disease Heterogeneity
2018, Journal of Pediatrics
Juvenile dermatomyositis: Latest advances
2017, Best Practice and Research: Clinical Rheumatology
Citation Excerpt :
No difference in the frequency of menstrual disturbances or the development of secondary sexual characteristics has been observed [65]. While overall mortality rate has significantly improved (falling from 30% [66] to less than 2% [41]) since the introduction of corticosteroids, DMARDS and novel biologics, the systemic inflammatory response remains profound in some children with JDM, and for a small subset, the resulting pulmonary, gastrointestinal or multisystem failure is fatal [67]. Risk factors include delay to treatment, older age, severe disease activity at onset and weight loss [67].
Registries and biobanks for juvenile dermatomyositis (JDM) have generated statistical power to help understand pathogenesis and determine treatment and long-term outcomes in this rare and heterogeneous disease. Genotype, autoantibodies, muscle histology and early clinical features may predict prognosis and guide personalised treatment. While corticosteroids and disease-modifying anti-rheumatic drugs improve outcomes, there remain children who experience refractory disease. Ongoing research into the aberrant immune response and novel biological targets is necessary. Best practice guidelines promote prompt stepwise treatment, and there is growing appreciation of the role of exercise in improving prognosis. Validated tools standardise assessment of disease activity and damage in musculoskeletal, mucocutaneous, pulmonary, cardiac, gastrointestinal and endocrine systems. Recently, an internationally agreed dataset for JDM has been defined for clinical practice and incorporation into registries. In the future, with bigger datasets, statistical models may guide stratification for personalised medicine and discern the most relevant outcome markers for research.

View all citing articles on Scopus

^*: Supported in part by National Institutes of Health Training Grant, United States Public Health Service 2A-5284.

^*: Department of Pediatrics, University of Texas Medical Center, Galvesion, Texas.

^**: Address, Department of Pediatrics, University of Texas Medical Branch, Galveston, Texas.

View full text

Dermatomyositis*

J. Pediat.

Lancet

Blood

Am. J. Med.

Am. Heart J.

Lancet

Blood

Bull. Johns Hopkins Hosp.

Minnesota Med.

Ein Fall von acuter Polymyositis

Deutsches Arch. klin. Med.

Ueber einen Fall von acuter Parenchymatoser Myositis, welche Geschwülste bildete und Fluctuation vortäuschte

Klin. Wchnschr.

Ueber Pseudotrichinose, eine besondere Form von acuter Parenchymatoser Polymyositis

Klin. Wchnschr.

Über eine Eigentümliche Form von acuter Muskelentzündung mit einen der Trichinose ahnlichen Ahneinden Krankheitsbilde

München med. Wchnschr.

Myositis universalis acuta infectiosa, with a case

Boston M. Surg. J.

Dermatomyositis, with report of a case which presented a rare muscle anomaly but once described in man

J. Exper. Med.

Polymyositis

Dermatomyositis

Am. J. Dis. Child.

Zur Klinik und Pathogenese der Dermatomyositis (Polymyositis)

Arch. Dermat. u. Syph.

Dermatomyositis in childhood: Report of a case with necropsy

Am. J. Dis. Child.

Dermatomyositis in children

Acta pediat.

Dermatomyositis in early childhood

Acta med. scandinav.

Dermatomyositis

A. M. A. Arch. Int. Med.

Dermatomyositis, Report of 26 cases in children with a discussion of endocrine therapy in 13

Pediatrics

Dermatomyositis: unusual features, complications and treatment

Arch. Dermat.

Dermatomyositis, review of 19 cases in adolescents and children

A. M. A. Arch. Int. Med.

Germatomyositis in childhood, Report of studies in 7 cases and a review of literature

Journal-Lancet

Percutaneous renal biopsy in children. I. General Considerations

Pediatrics

Les Polymyositis

Rev. Neurol.

Acute polymyositis

Am. J. Dis. Child.

Dermatomyositis: Report of a case with necropsy

Am. J. Dis. Child.

Dermatomyositis in children: a report of 2 cases with fatal termination

South. M. J.

Ueber Die Polymyositis

Klin. Wchnschr.

Dermatomyositis and systemic lupus erythematosis: clinical report of transitional cases

Arch. Int. Med.

Is there a common denominator in scleroderma, dermatomyositis, disseminated lupus erythematosis, the Libman-Sachs syndrome and polyarteritis nodosa?

New England J. Med.

Dermatomyositis and poikiloderma atiophicans vascularis

Brit. J. Dermat.

Dermatomyositis: a study of 40 cases

Arch. Dermat.

Remarques a propos de six cas de dermatomyosite

Semaine hôp. Paris

Dermatomyositis

Proc. Roy. Soc. Med.

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Bull. Johns Hopkins Hosp.

Hypersensitivity in disease, with especial reference to polyarteritis nodosa, rheumatic fever, disseminated lupus and rheumatoid arthritis

Harvey Lectures

Dermatomyositis in carcinoma: possible etiological mechanism

Cancer

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J. A. M. A.

Dermatomyositis following penicillin injections

Neurology

Dermatomyositis in children; a summary of 40 cases

Postgrad. Med.

Dermatomyositis