Morphoea (Localised Scleroderma)

Definition

• Morphoea (also called localised scleroderma) is an inflammatory and fibrotic process affecting only the skin, characterised by excess collagen deposition.

• It typically presents with purple erythematous patches that evolve into ivory-white, shiny plaques, often lacking hair (alopecia in the lesion).

• Unlike systemic sclerosis, morphoea does notinvolve internal organs.

Differences from Systemic Sclerosis

• No skin thickening of the fingers or toes

• No specific autoantibodies commonly linked to systemic disease (e.g., anti-Scl-70, anti-centromere)

• No small vessel disease: Absent Raynaud’s phenomenon, digital ulcers

• No internal organ (lung, GI, renal) involvement

Pathophysiology

• Collagen overproduction leads to inflammation and fibrosis in the skin’s dermal and sometimes subcutaneous layers.

• Incidence is rare, mainly in children (~1–3 per 100,000).

Diagnosis

• Often clinical but can require biopsy for confirmation.

• Blood Tests:

  • May show eosinophilia, raised ESR/CRP.

  • ANA can be positive, but ENA is usually negative or unremarkable.

Management

• No definitive cure; aim to halt progression and limit complications.

Topical Therapy

• Potent topical steroids (reduce local inflammation)

• Tacrolimus ointment, calcipotriol, or imiquimod in some cases

Phototherapy

• UVA1 can help deeper lesions by reducing collagen deposition

Systemic Therapy (Severe Cases)

• Methotrexate plus short-course systemic steroids

• Mycophenolate mofetil as an alternative immunosuppressant

Supportive Measures

• Monitor for joint contractures in lesions crossing joints

• Physiotherapy may be needed if limb range of motion is threatened

Complications if Untreated

• Joint contractures, especially in children

• Growth defects (when affecting growth plates)

• Severe cosmetic deformity (atrophy, disfigurement)