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February 1991

Focal Scleroderma and Severe Cardiomyopathy: Patient Report and Brief Review

Author Affiliations

From the Divisions of Clinical Immunology and Rheumatology (Drs Moore and Cohen), Pediatric Cardiology (Dr Farooki), and Surgical Pathology (Dr Chang), Children's Hospital of Michigan, and the Departments of Pediatrics (Drs Moore, Cohen and Farooki) and Pathology (Dr Chang), Wayne State University School of Medicine, Detroit.

Am J Dis Child. 1991;145(2):229-231. doi:10.1001/archpedi.1991.02160020123031

• A 21-month-old infant presented with simultaneous localized scleroderma and severe cardiomyopathy with heart failure. Cardiac abnormalities and serological changes (positive rheumatoid factor assay, elevated IgM and IgG levels, and elevated erythrocyte sedimentation rate) reverted to normal with prednisone therapy, and there was substantial, though incomplete, resolution of her skin changes during the same period. To our knowledge, this is the first patient with definite, clinically significant cardiac involvement associated with focal scleroderma. The possibility of internal organ involvement, including cardiac involvement, must be considered with focal scleroderma as well as with progressive systemic sclerosis.

(AJDC. 1991;145:229-231)

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