Atypical Cases of Scleroderma en Coup de Sabre. (May 2014)

• Record Type: Journal Article
• Authors: Kraus, Verena; Lawson, Erica F.; Scheven, Emily von; Tihan, Tarik; Garza, Judith; Nathan, Rani G.; Cordoro, Kelly M.; Waubant, Emmanuelle
• Is Part Of: Journal of child neurology. Volume 29:Number 5(2014:May)
• Page Start: 698
• Page End: 703
• Publication Date: 2014-05
• Subjects: scleroderma -- acquired demyelinating disease -- multiple sclerosis -- epilepsy -- children; Nervous system -- Diseases -- Periodicals; 618.928
• DOI: 10.1177/0883073813520494
• Languages: English
• ISSNs: 0883-0738

Abstract

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.