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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Congenital localized scleroderma
Francesco Zulian1, Cristina Vallongo, Sheila Knupp Feitosa de Oliveira
1Department of Pediatrics, University of Padua, Italy. zulian@pediatria.unipd.it
The Journal of Pediatrics
|August 5, 2006
Summary
Congenital localized scleroderma (CLS) is a rare condition presenting at birth. This rare form of juvenile localized scleroderma (JLS) exclusively shows linear subtypes, often misdiagnosed, necessitating earlier identification.
Area of Science:
- Pediatric Rheumatology
- Pediatric Dermatology
- Neonatology
Background:
- Juvenile localized scleroderma (JLS) typically manifests in later childhood.
- Congenital localized scleroderma (CLS) is a rare variant with onset at birth.
- Understanding CLS is crucial for early diagnosis and management in neonates.
Observation:
- A multinational study identified 6 cases (0.8%) of CLS among 750 JLS patients.
- All CLS cases presented with linear scleroderma, four with facial 'en coup de sabre' morphology.
- Misdiagnosis was common, with delays averaging 3.9 years.
Findings:
- CLS exhibits a significantly longer disease duration at diagnosis compared to late-onset JLS.
- The 'en coup de sabre' subtype was more frequent in CLS.
- Linear scleroderma was the sole presentation in all studied CLS cases.
Implications:
- CLS is likely underestimated in newborns and requires inclusion in differential diagnoses for infants with skin lesions.
- Prompt diagnosis of CLS can prevent functional and aesthetic complications.
- Early therapeutic intervention is vital for managing CLS and mitigating long-term sequelae.
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