Morphea

Last Updated: 2021-11-19

Author(s): Anzengruber, Navarini

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.

Topical therapy

  • Mometasone fuorate cream / solution / ointment
  • Clobetasol cream 1 daily for several weeks, then reduce frequency
  • Tacrolimus ointment 0.1% 2x tgl. for 3 months if necessary under occlusion

Systemic therapy

  • Prednisolone p.o. 0.5-1.0 mg/kg/d
  • Methotrexate (MTX) s.c. 15 mg 1x weekly
    • Start with 10 mg 1x weekly
    • Increase by 2.5 mg weekly until 15 mg is reached.
    • Folic acid p.o. 5 mg 1-0-0 the following day (24 hours later) to reduce side effects.
  • Combination therapy with methotrexate & oral steroids
  • Mycophenolate mofetil p.o. 1-1.5 g 2x tgl.

UV therapy:

  • UVA, UVB
    • Recommended: medium-dose UVA1 to be used 3- 5 times a week for a total of 40 sessions.

Physiotherapy

  • Frequently used, although no studies exist.
  • Conjunctive tissue massage
  • Manual lymphatic drainage
    • Recommended: 1-2x weekly for at least 3 months.

Surgery

  • Indicated only for linear circumscritical scleroderma
  • Should be performed in the inactive stage after several years of no disease activity
  • Tendon lengthening
  • Contracture release surgery
  • Autologic fat grafting
  • Application of defect-filling substances (e.g. fillers)
  • Plastic surgical operations
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