Korean J Dermatol.  2021 Nov;59(9):699-706.

Clinical Features and Outcome of Juvenile Dermatomyositis: A Retrospective Study and Literature Review

• Kim TR
• Bae KN
• Son JH
• Shin K
• Kim HS
• Ko HC
• Kim MB
• Kim BS

Abstract

Background
Juvenile dermatomyositis (JDM) is a rare but common childhood idiopathic inflammatory myopathy. Proximal muscle weakness and pathognomonic skin rash, Gottron papules, and heliotrope rash are characteristic clinical features of JDM. However, clinical analysis of JDM has rarely been reported in the Korean dermatologic literature.
Objective
This study aimed to investigate the clinical features and outcomes of JDM in Korea and previous studies.
Methods
We retrospectively reviewed the medical records and clinical photographs of patients diagnosed with JDM at Pusan National University Hospital (Busan and Yangsan) for 17 years (2005∼2021).
Results
We encountered 12 patients with JDM (male to female ratio=7:5) with a mean age of 7.2 years. The most common clinical features were Gottron papules (100%), followed by Gottron sign (83.3%), malar rash (58.3%), heliotrope rash (41.7%), shawl sign (16.7%), calcinosis cutis (8.3%), and ulcer (8.3%). In all cases, there was no concurrent interstitial lung disease or an underlying malignancy. Only 1 of 12 patients complained of proximal muscle weakness, and four patients showed an increase in muscle enzymes in the laboratory test. The skin lesions gradually improved after systemic steroid or topical treatment in all cases. No additional proximal muscle weakness was found during the follow-up.
Conclusion
Although proximal muscle weakness is a common symptom in JDM, clinically amyopathic JDM is commonly found in dermatologic clinics. Regardless of subtype, all patients responded well to treatment and rarely encountered recurrence.

Keyword

Dermatomyositis; Muscle weakness