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Dermatomyositis/Polymyositis, Pediatric

Megan L. Curran, MD Reviewed 10/2018
 


BASICS

DESCRIPTION

Juvenile dermatomyositis (JDM) and juvenile polymyositis (JPM) are inflammatory myopathies in which inflammation within tissues and capillary endothelium of muscle, skin, and other o...

DIAGNOSIS

Bohan and Peter criteria (1975): not validated in children and becoming outdated, but nonetheless used. Definite JDM requires rash plus three other criteria; probable JDM requires rash plus ...

TREATMENT

MEDICATION

  • Early aggressive therapy improves overall outcome and reduces frequency of calcinosis.

  • Prednisone 1 to 2 mg/kg/24 h PO (maximum 60 mg) for 1 month, taper over months to years

  • IV meth...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

  • Serial evaluation of muscle strength and function using validated measures such as the Childhood Myositis Assessment Scale or Manual Muscle Test...

ADDITIONAL READING

  • Feldman BM, Rider LG, Reed AM, et al. Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood. Lancet.  2008;371(9631): 2201–2212. [View Abstract on ...

CODES

ICD9

  • 710.3 Dermatomyositis

  • 710.4 Polymyositis

ICD10

  • M33.00 Juvenile dermatopolymyositis, organ involvement unspecified

  • M33.02 Juvenile dermatopolymyositis with myopathy

SNOMED

  • 1212005 Childhood type de...

FAQ

  • Q: Is it mandatory to perform a muscle biopsy to confirm the diagnosis?

  • A: Biopsy is indicated if diagnosis is in any way uncertain. In patients with classic rash, weakness, and elevated muscle enz...

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