Scleroderma en coup de sabre typically presents with a cutaneous induration, but involvement of the underlying bone, eye, and brain is common. We report on 4 pediatric cases with atypical initial clinical presentations. All cases were seen at the University of California San Francisco. Patients 1 and 2 presented to the Pediatric Rheumatology Clinic with uveitis and orbital pseudotumor in December 2009 and March 2010, respectively. Patients 3 and 4 were mimicking acquired demyelinating disorders of the brain, acute demyelinating encephalomyelitis and multiple sclerosis, and were referred to the Pediatric Multiple Sclerosis center in 2008.
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