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Linear Scleroderma (En Coup de Sabre)

Localized morphea variant: pediatric onset and CNS involvement risk

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dermatoloji department. Book Appointment →

What is Linear Scleroderma (En Coup de Sabre)?

Linear scleroderma is a localized scleroderma variant that develops most commonly between 2-14 years of age. Linear, indurated, hyperpigmented or hypopigmented plaques follow Blaschko's lines on extremities, trunk, or scalp; muscle, tendon, joint capsule, and bone involvement may occur, leading to limb-length discrepancy and contractures.

En coup de sabre is a special form located on the forehead and scalp; bone resorption, atrophy, alopecia, and ipsilateral CNS involvement (seizures, headache, focal deficits) occur in 10-20% of cases. MRI is essential for evaluation. Parry-Romberg syndrome (progressive hemifacial atrophy) may be in the same spectrum.

Diagnosis is clinical; skin biopsy demonstrates dense collagen deposition, perivascular lymphocytic infiltrate, and reduced adnexa. ANA may be positive in 25-40% of cases; absence of anti-Scl-70 and anti-centromere distinguishes it from systemic scleroderma. Active phase (erythema, expanding edge) lasts 3-5 years; inactive phase has reduced inflammation but persistent fibrosis.

Symptoms

Linear, indurated plaque (along extremity or scalp)
Hyperpigmentation or hypopigmentation
Reduced subcutaneous tissue and muscle atrophy
Joint contracture and limb-length discrepancy
Frontal/scalp atrophic depressed plaque (en coup de sabre)
Headache and seizures (CNS involvement)

Risk Factors

Childhood (2-14 years onset)
Female gender (2:1 predominance)
Trauma or vaccination history (precipitating)
Family history of autoimmune disease
Borrelia burgdorferi exposure (controversial)
Linear distribution along Blaschko's lines

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Linear, hyperpigmented, indurated plaque in a child
  • Limb-length discrepancy or joint contracture
  • Expanding lesion on forehead/scalp
  • Headache, seizures, neurologic symptoms
  • Cosmetic distortion or atrophy
  • Erythematous and rapidly expanding plaque (active phase)

Treatment Methods

01
Methotrexate (1 mg/kg/wk, maximum 25 mg) - first line
02
Systemic glucocorticoid (initial 3-6 months, pulse with MTX)
03
Mycophenolate mofetil (MTX-resistant)
04
Phototherapy (UVA1, narrowband UVB) - inactive phase
05
Physiotherapy (contracture prevention)
06
Reconstructive surgery (after disease inactivation, atrophy)

Which Department to Visit?

You can visit our Dermatoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.