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Morphea (Localized Scleroderma), Pediatric

Lisa M. Arkin, MD and Kaveh Ardalan, MD, MS Reviewed 10/2018
 


BASICS

DESCRIPTION

  • The term for localized scleroderma is morphea, which is derived from the Greek word for “form” or “structure.” Fibrosis is characteristic and may involve the skin, subcutaneous (SC) ...

DIAGNOSIS

PHYSICAL EXAM

  • General concepts:

    • Fibrosis limited to skin, SC tissue, muscle, and bone

    • Systemic features including Raynaud phenomenon and true visceral fibrosis should prompt reconsideration of ...

TREATMENT

ALERT

Differentiating disease activity (inflammation) and damage (fibrosis) has important implications for therapy: Disease activity responds to therapy but longstanding disease damage does n...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

  • Once systemic immunosuppressive therapy is initiated, disease activity typically begins to resolve within months, and features of damage may als...

CODES

ICD9

701.0 Circumscribed scleroderma 

ICD10

L94.0 Localized scleroderma [morphea] 

SNOMED

  • 201048007 Localized scleroderma

  • 201049004 Morphea

  • 403522004 Subcutaneous morphea

  • 403521006 Guttate morphea

  • 227840...

ADDITIONAL READING

  • Li SC, Torok KS, Pope E, et al; and Childhood Arthritis and Rheumatology Research Alliance (CARRA) Localized Scleroderma Workgroup. Development of consensus treatment plans for juve...

FAQ

  • Q: Is a biopsy necessary?

  • A: Biopsy is often useful primary to evaluate for the extent of disease activity (lymphoplasmacytic infiltrate) versus damage (homogenized collagen and fibrosis) and to ex...

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