Connective tissue nevus misdiagnosed as juvenile localized scleroderma
Authors' conclusion
CTN can be misdiagnosed as JLS and therefore aggressively treated with prolonged and inappropriate immunosuppression.
Study context
- Population
- Children referred over the last two decades for juvenile localized scleroderma (JLS) who received a final diagnosis of connective tissue nevi (CTN)15 certain, 2 suspected JLS
- Participants
- 17 children
- Outcome
- Characteristics of CTN misdiagnosed as JLS
Methodological detailssingle-centre
- Single-centre
Main finding
As reported in the abstract
- Mean age at onset
- 4.6 years
- Lesions mainly at lower limbs
- 14 (82.4%)
- Lesions at upper limbs
- 3 (17.6%)
- Laboratory inflammatory changes or positive autoantibodies
- 0
- Non-familial collagenoma on biopsy
- 11 (64.7%)
- Mixed CTN on biopsy
- 4 (23.5%)
- Familial CTN on biopsy
- 2 (11.8%)
- Mean age at final diagnosis
- 9.5 years
- Mean diagnostic delay
- 4.8 years (range 1-15 years)
- Musculoskeletal MRI abnormal (muscle perifascial enhancement), of 16 scanned
- 2
- Thermography normal
- all patients
- On systemic treatment at first evaluation
- 11 (64.7%)
- Systemic treatment: MTX / corticosteroids / biologics
- 11 / 7 / 2
- On topical corticosteroids
- 3 (17.6%)
- Untreated
- 3
Observational data: associations, not effects.
Original publication
Connective tissue nevus misdiagnosed as juvenile localized scleroderma. Pediatr Rheumatol Online J. Published 17 October 2023. doi:10.
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