Last Updated: 2021-11-19
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
- Localisation: mainly extremities and face
- Type III: Subcutaneous, profound scleroderma (ca.12% of children)
- Pansclerotic disabling disease of children
- 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)
- 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)
- 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:
- 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
Most of the time, progression stops after about 7 years.
- 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
- 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.
- UVA, UVB
- Recommended: medium-dose UVA1 to be used 3- 5 times a week for a total of 40 sessions.
- Frequently used, although no studies exist.
- Conjunctive tissue massage
- Manual lymphatic drainage
- Recommended: 1-2x weekly for at least 3 months.
- 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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