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How Often are Pediatric Patients with Clinically Amyopathic Dermatomyositis Truly Amyopathic? Pediatr Dermatol 2017 Jan;34(1):50-57

Date

11/05/2016

Pubmed ID

27813167

DOI

10.1111/pde.13013

Scopus ID

2-s2.0-84997079565 (requires institutional sign-in at Scopus site)   12 Citations

Abstract

BACKGROUND: Pediatric patients can present with skin manifestations of dermatomyositis without overt weakness (clinically amyopathic juvenile dermatomyositis [JDM]), but it is unclear how often this happens and how often they have subclinical muscle inflammation.

OBJECTIVE: Our goal was to determine the frequency of clinically amyopathic JDM and the frequency with which a thorough evaluation uncovers subclinical myositis at a single institution.

METHODS: A retrospective review was performed of 46 patients diagnosed with JDM at Children's Hospital of Wisconsin.

RESULTS: Of 46 patients presenting with skin findings consistent with dermatomyositis, 10 patients (21.7%) did not have evidence of muscle involvement on history or exam, and these tended to be the younger patients. Of these 10, only 2 (4% of all the JDM patients) were truly amyopathic upon further evaluation (all five muscle enzymes [aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase, creatine kinase and aldolase], magnetic resonance imaging [MRI], muscle biopsy). In our series, muscle biopsy was not helpful in identifying subclinical myositis. In contrast, MRI did uncover subclinical muscle disease.

CONCLUSION: These data suggest that truly amyopathic JDM is rare and that a thorough workup that includes all five muscle enzymes and MRI may uncover occult myositis.

Author List

Oberle EJ, Bayer ML, Chiu YE, Co DO

Author

Yvonne E. Chiu MD Vice Chair, Professor in the Dermatology department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Adolescent
Biopsy
Child
Child, Preschool
Dermatomyositis
Female
Hospitals, Pediatric
Humans
Magnetic Resonance Imaging
Male
Muscle, Skeletal
Retrospective Studies
Sensitivity and Specificity
Wisconsin