Morphea (Localized Scleroderma)

Descriptive text is not available for this imageBASICS

DESCRIPTION

  • Localized scleroderma (LS), also known as morphea, is a condition in which inflammation leads to areas of cutaneous fibrosis.
  • LS is a distinct condition from systemic sclerosis (SSc).
  • In contrast to SSc, morphea is not typically associated with Raynaud phenomena, abnormal nailfold capillaries, or fibrosis of internal organs.
  • Subtypes of morphea:
    • Circumscribed: localized indurated plaques
    • Generalized: ≥4 plaques, >3 cm, >2 body sites
    • Linear: linear plaques on the face, scalp, extremity, or trunk
      • Lesions affecting the head include en coup de sabre and Parry-Romberg syndrome (progressive hemifacial atrophy).
    • Pansclerotic: confluent full-thickness involvement that generally spares the distal fingertips and toes
      • Subtype with greatest morbidity and highest mortality
    • Mixed: more than one subtype
    • Any subtype can have a deep component, but this is of particular concern in the linear subtype.
ALERT

Patients with suspected morphea are initially diagnosed and treated by dermatologists but should be referred to a pediatric rheumatologist for comanagement. Morphea patients can exhibit extracutaneous manifestations. Morphea often requires systemic immunosuppressive treatments, as topical treatments may not be sufficient to stop progression.

EPIDEMIOLOGY

  • In children, morphea is approximately 10 times more common than systemic sclerosis.
    • Estimated incidence: 2.7 cases per 100,000 per year
    • Estimated prevalence: 50 cases per 100,000
  • Mean age of onset is 6.4 to 10.5 years; however, diagnosis is often delayed by up to 2 years.
    • Rarely can be present at birth
  • 1/3 of morphea cases start in childhood.
  • 70–80% female
  • 75% of patients are White.
  • Linear morphea is the most frequent subtype, followed by circumscribed morphea, mixed morphea, and then generalized morphea.
  • Pansclerotic morphea is exceedingly rare and accounts for <1% of patients.

PATHOPHYSIOLOGY

  • Speculated to share common pathophysiology with systemic sclerosis despite differing clinical presentations
  • Etiology is likely multifactorial with genetic predisposition, autoimmunity, and environmental factors that trigger local inflammation leading to persistent increased collagen synthesis and deposition in the skin, which causes fibrosis. This process may extend deeper to the subcutaneous tissue, muscle, and bone.

There's more to see -- the rest of this topic is available only to subscribers.