Morphea

Localised Scleroderma, circumscripted scleroderma

A self-limited autoimmune disease belonging to the group of collagenoses, characterised by fibrosclerosis of the skin. Both the involvement of internal organs and the transition to systemic scleroderma are a rarity.

• Age peak: infancy 2-8 years and between 20-50 years
• F:M = 2.6 - 6:1
• 85% of all affected individuals are Caucasian
• Association to chronic polyarthritis or lupus erythematosus

• Type I: Plaque-like scleroderma (approx. 70% of adults, approx. 30% of children)
  • Localisation: mainly the trunk area
  • Subform: Atrophodermia idiopathica et progressiva (Pierini-Pasini type)
• Type II: Linear scleroderma: (10-30% of adults, approx. 65% of children)
  • Localisation: mainly extremities and face
    • 1a: localised, 1b: generalised type
    • 2: scleroderma en coup de sabre
• Type III: Subcutaneous, profound scleroderma (ca.12% of children)

Special forms:

• Pansclerotic disabling disease of children
• Sclerofascia
• Eosinophilic fasciitis (Shulman type)
• Hemiatrophia faciei progressiva (Romberg type)

• Initially, there are centrifugally growing whitish, brownish or red plaques. An indurated, ivory-coloured atrophic zone is often visible centrally. The lesions are bordered by a blue-red erythema rim, the so-called Lilac Ring, in case of progression. 
• Depending on the form, subcutaneous, fascia, muscle, periosteum and skeletal tissue are affected during progression.
• Division according to S1- Guideline "Circumscribed Scleroderma"
• Limited circumstrictive forms of scleroderma (type I)
  • Plaque-type morphea (with 26%, the 2nd most common scleroderma form in children)
  • Morphea guttata (is called "extragenital lichen sclerosus et atrophicus")
  • Atrophodermia Pierini-Pasini (Atrophodermia idiopathica et progressiva)
• Generalised forms of scleroderma
  • Generalised morphea (for this, at least 3 different anatomical localisations must be affected)
  • Disabling pansclerotic morphea
  • Eosinophilic fasciitis
• Linear forms of scleroderma (type II)
  • Linear scleroderma of the "en coup de sabre" type: subcortical calcifications or brain atrophy, which can lead to e.g.: migraine, epilepsy.
  • Linear scleroderma
  • Progressive fascial hemiatrophy (Parry-Romberg syndrome): subcortical calcifications or brain atrophy, which can lead to e.g.: Migraine, epilepsy.
• Deep form of scleroderma (type III)
  • Morphea profunda

Severity: depends on the localisation and extent of the skin lesions.

• Clinic (is sufficient in most cases for diagnosis)
• Biopsy
• There are no serological progression parameters!
• Laboratory (useful for differential diagnosis)
  • blood count (sometimes eosinophilia in linear forms)
  • creatinine kinase (e.g. of concomitant myositis)
  • Autoantibodies (ANA (positive in up to 80% even without systemic involvement), anti-Scl-70)
  • Borrelia serology (pseudoscleroderma in Borrelia infection)

Further diagnostics:

• Modified Rodnan Skin Score (thickness of the skin is measured by palpation)
• Cutometer (elasticity measurement)
• Durometer (hardness test)
• Quantification of induration:
  • 20 MHz sonography
  • Laser Doppler
• Depth extension:
  • CT
  • MRI

• Torso (58%)
• Legs (24%)
• Arms (12%)
• Head (6%)

• Early stage (inflammatory stage):
  • Perivascular and periadnexal lymphohistiocytic infiltrates in the reticular dermis and subcutis
  • Thickening of the dermal oedematous collagen bundles
  • Septal panniculitis
  • Round cell infiltrates at the dermo-subcutaneous junction
• Late stage (sclerotic stage)
  • Rarefaction of adnexal glands (e.g. sweat glands)
  • Sclerosis of the dermis
  • More dermal connective tissue, less subcutaneous adipose tissue
  • Homogenised widened collagen fibre bundles run parallel to the skin surface
  • Narrowed vessels
  • Edematous adipose tissue septa

• Joint contractures
• Cosmetic impairment

None known.

Most of the time, progression stops after about 7 years.

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