Morphea

Morphoea (localised scleroderma) is an autoimmune fibrosing disorder of the skin and underlying tissue without sclerodactyly, Raynaud phenomenon, or visceral involvement.

• Incidence 0.4 to 2.7 per 100,000; women more than men (3:1); onset in childhood or adulthood.

• Subtypes: circumscribed (plaque), linear (children, limbs or "en coup de sabre" on face and scalp), generalised, pansclerotic, mixed.

• Risk of extracutaneous manifestations in 20% (arthritis, uveitis, seizures with craniofacial disease, Parry-Romberg syndrome).

• Associations: autoimmune disease, preceding trauma, radiotherapy, Borrelia infection (disputed).

• Initial erythematous or violaceous inflammatory plaque, then indurated ivory white centre with violaceous border (lilac ring), later atrophic hyperpigmented or hypopigmented patch.

• Linear: limb contractures, growth discrepancy, muscle atrophy, joint restriction.

• En coup de sabre: depressed linear lesion on forehead/scalp with alopecia.

• Generalised morphoea with multiple plaques; deep morphoea affects subcutis and muscle.

• Clinical diagnosis; skin biopsy for uncertain cases (dermal sclerosis, collagen thickening, perivascular lymphoplasmacytic inflammation).

• Antinuclear antibodies (30 to 80%), anti-single stranded DNA, antihistone antibodies; scleroderma specific antibodies absent.

• Eosinophilia, raised ESR may mark activity.

• MRI/ultrasound for depth and activity in linear and deep forms; MRI brain and ophthalmology review in craniofacial disease.

• Exclude systemic sclerosis (capillaroscopy), lichen sclerosus, eosinophilic fasciitis, nephrogenic systemic fibrosis, radiation fibrosis.

• Assess disease activity (LoSCAT).

• Limited superficial plaque: topical calcipotriol 0.005% with betamethasone, tacrolimus 0.1% ointment, high potency corticosteroids (clobetasol 0.05%) for 3 months; narrowband UVB or UVA1 phototherapy (30 to 60 J/cm2, 3 to 5 times weekly for 30 sessions).

• Extensive, progressive, deep, or functionally threatening disease (and all linear craniofacial forms): methotrexate 15 mg/m2 weekly (adults 15 to 25 mg) plus prednisolone 1 mg/kg (maximum 60 mg) for the first 3 months, then taper; continue methotrexate at least 12 months after remission.

• Methotrexate failure: mycophenolate mofetil 1 to 1.5 g twice daily, tocilizumab, abatacept.

• Physiotherapy and occupational therapy for contractures; orthopaedic surgery for limb length discrepancy; plastic surgery (fat grafting) when inactive.

• Ophthalmology screening for uveitis; neurological follow up in craniofacial disease.

• Relapse is common; follow up regularly.