🩻 TOACS FCPS Station · Growth Failure of Left Lower Limb in Linear Scleroderma
📷 Clinical Photograph – Linear Scleroderma & Limb Shortening
🔍 Key clinical features:
- ✓ Linear, indurated band – on leg (anteromedial)
- ✓ Hypopigmentation and skin atrophy
- ✓ Muscle wasting and subcutaneous tissue loss
- ✓ Shortening of the limb – due to growth failure
- ✓ Joint contracture – ankle or knee
📋 Clinical scenario (examiner prompt)
A 9‑year‑old girl is brought to the clinic because her mother has noticed that the left leg appears shorter than the right. The child has had a linear, hardened patch on the anteromedial left thigh and leg since age 4, which has been slowly progressing. The skin in the area is hypopigmented and atrophic. There is also muscle wasting and mild ankle contracture. The child walks with a limp. There is no history of trauma. The parents report that the skin lesion was biopsied years ago and diagnosed as linear scleroderma.
🧑⚕️ Examiner tasks · TOACS
1. Identify the diagnosis from the clinical image and context.
2. Describe the clinical features (linear scleroderma, limb shortening, muscle wasting, contracture).
3. Explain the pathogenesis (localized scleroderma affecting deep tissues including bone → growth retardation).
4. Discuss evaluation and management (measuring leg length discrepancy, imaging, methotrexate, PT, shoe lift, orthopedic referral).
🎯 Expected answers (for examiners)
- • Diagnosis: Linear scleroderma with limb growth failure (deep tissue involvement)
- • Clinical features: Linear, indurated, hypopigmented/atrophic skin band (often anteromedial extremity); subcutaneous fat, muscle, and bone involvement → muscle wasting, joint contracture, limb shortening (growth failure)
- • Pathophysiology: Localized scleroderma (not systemic) with inflammation extending beyond dermis to deep tissues; impaired growth due to chronic inflammation and reduced blood supply
- • Evaluation: Measure leg length (clinical, scanogram); MRI to assess soft tissue and bone involvement; monitor disease activity (erythema, progression)
- • Management: Systemic methotrexate (1 mg/kg/week) ± corticosteroids for active disease; physical therapy (passive/active range of motion, stretching); shoe lift for leg length discrepancy >1-2 cm; orthopedic referral if significant discrepancy (epiphysiodesis)
• Most common subtype of localized scleroderma in children
• Deep involvement → muscle, bone, joint
• Limb shortening – due to growth failure from chronic inflammation
• Treatment: MTX ± steroids (active disease)
• Physical therapy – prevent contractures
• Orthopedic – shoe lift, epiphysiodesis if needed
⚡ Quick FCPS‑style MCQ
A child with linear scleroderma of the left leg develops a 2 cm leg length discrepancy and ankle contracture. The most appropriate management is:
A. Surgical amputation B. Methotrexate for disease control + physical therapy + shoe lift C. Observation only D. High-dose corticosteroids alone📌 Topic summary · Growth Failure in Linear Scleroderma
Linear scleroderma – band-like induration of skin/deep tissues
Limb-length discrepancy, contractures
Deep tissue involvement (muscle, bone)
Methotrexate ± corticosteroids
Shoe lift, PT, epiphysiodesis
Better with early aggressive treatment
| Feature | Linear Scleroderma – Limb Growth Failure |
|---|---|
| Definition | Localized scleroderma subtype with linear band of induration; can extend to deep tissues (muscle, bone) |
| Common sites | Extremities (anteromedial leg, arm), scalp/forehead (en coup de sabre), trunk |
| Complications | Limb-length discrepancy (growth failure), joint contractures, muscle wasting, cosmetic deformity |
| Evaluation | Leg length measurement (clinical, scanogram), MRI for deep tissue extent, monitoring disease activity |
| Disease treatment | Methotrexate (1 mg/kg/week) + corticosteroids (for active disease); maintenance MTX for 12-24 months |
| Physical therapy | Range of motion, stretching, splinting to prevent/improve contractures |
| Orthopedic management | Shoe lift for leg length discrepancy >1-2 cm; epiphysiodesis of longer leg if significant discrepancy (>2-3 cm) |
| Prognosis | Early aggressive treatment improves outcomes; residual deformity may remain |