Abstract
Juvenile localised scleroderma (JLS) is a rare condition (incidence 3.4 cases per million children per year) with disease predominantly affecting cutaneous and subcutaneous tissue but without internal organ involvement. A recent international prospective cohort study reported 38% of patients experienced delay in diagnosis of > 1 year. Delayed diagnosis leads to increased damage (cutaneous and extracutaneous). Almost 20% of patients with JLS have extracutaneous manifestations, most commonly musculoskeletal. Scoliosis has not been widely reported. We present an 8-year-old girl referred to paediatric rheumatology with skin lesions consistent with JLS predominantly affecting her back and causing severe scoliosis. It is notable that this child experienced significant delay in accessing specialist care. First review with paediatric rheumatology took place 3 years after onset of skin changes.
The patient had extensive skin changes over her back consistent with JLS, with significant skin thickening. Scoliosis was evident (Cobb angle 33 degrees on X-ray). She had previously received topical treatments including corticosteroids and tacrolimus, and UV light therapy. Localised Scleroderma Assessment Tool (LoSCAT) was completed at each visit (Table 1).
Treatment was commenced with IV methylprednisolone (3 consecutive doses of 30mg/kg, followed by one 30mg/kg dose monthly for 3 months) and subcutaneous methotrexate 15mg/m2 weekly. MRI spine confirmed scoliosis but no abnormality of the spinal cord or vertebral bodies and no para-spinal or abdominal lesions. Skin improved on treatment (Table 1). Following assessment by spinal and plastic surgeons, and a pre-operative multidisciplinary meeting, the patient underwent surgical division of a deep scar contracture on the lower back. She was fitted with a removable spinal brace, allowing skin to be assessed in clinic. Methotrexate was paused peri-operatively for 2 weeks. She did not require corticosteroids in the peri-operative period. There was no flare of scleroderma post-operatively and wound healing was uncomplicated, with no infection. Post-operative X-ray confirmed reduction of Cobb angle from 30 to 12 degrees. LoSCAT remains stable post-surgery (Table 1).
We highlight a very rare but significant complication of JLS. Through multidisciplinary coordination, a positive surgical outcome has been achieved, with no disease flare and uncomplicated wound healing.
E. Willis: None. E. Heaf: None. C. Pain: None.
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