Korean J Pediatr.  2007 Nov;50(11):1116-1124. 10.3345/kjp.2007.50.11.1116.

A clinical analysis of juvenile dermatomyositis; focus on clinical manifestations at diagnosis

• Lee SY
• Bang JS
• Kim HS
• Kim JG

• KMID: 2279372
• DOI: http://doi.org/10.3345/kjp.2007.50.11.1116

Abstract

PURPOSE: Juvenile dermatomyositis (JDM) is the most common of the idiopathic inflammatory myopathies in children. The purpose of this study is to observe demographic, initial presentations, duration of time between disease onset and diagnosis, clinical manifestations and laboratory findings at diagnosis of patients with JDM.
METHODS
Forty seven patients identified at Seoul National University Children's Hospital from January 1986 to May 2007. Medical records were reviewed retrospectively focusing on initial presentations, clinical manifestations and laboratory findings at the time of diagnosis of patients with JDM.
RESULTS
Male and female patients were 25 and 22, respectively and sex ratio was 1.14:1. The average age at the time of diagnosis was 6.51 years. Skin rash (94%) was the most common symptom, followed by the proximal muscle weakness (89%). The disease activity score was 10.8. The duration between the onset of the skin rash and the muscle weakness and diagnosis was 7.18 and 4.70 months, respectively. The serum muscle enzymes, LDH, AST, CK and aldolase, were elevated in the patient with JDM. Autoimmune antibodies, antinuclear antibody, anti SSA antibody and anti SSB antibody, were negative findings. Electromyography findings were consistent with JDM in 88% of the patients, the muscle biopsy was in 91% and all MRI findings were compatible with those of patients with JDM. The most common symptom besides musculocutaneous lesions was the calcinosis (62.5%). The most common site of calcinosis was the pelvic area and buttocks.
CONCLUSION
This study shows that the major symptoms are proximal muscle weakness and cutaneous lesion, and they are important to diagnose JDM.

Keyword

Juvenile dermatomyositis; Proximal muscle weakness; Rash; Calcinosis

MeSH Terms

Antibodies, Antinuclear
Biopsy
Buttocks
Calcinosis
Child
Dermatomyositis*
Diagnosis*
Electromyography
Exanthema
Female
Fructose-Bisphosphate Aldolase
Humans
Magnetic Resonance Imaging
Male
Medical Records
Muscle Weakness
Myositis
Retrospective Studies
Seoul
Sex Ratio
Antibodies, Antinuclear
Fructose-Bisphosphate Aldolase