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January 1980

Circumscribed Scleroderma With Immunologic Evidence of Systemic Lupus Erythematosus: Report of a Case and Review of the Literature

Author Affiliations

From the Department of Dermatology, University of Michigan (Drs Mitchell and Diaz), and the St Louis Park (Minn) Medical Center (Dr Rusin).

Arch Dermatol. 1980;116(1):69-73. doi:10.1001/archderm.1980.01640250071020

• An 8-year-old girl initially manifested clinical and histopathologic findings of circumscribed scleroderma. She had both linear and plaque lesions. Although she was free of constitutional symptoms, laboratory evaluation revealed substantial evidence of systemic lupus erythematosus (SLE). This included a positive antinuclear antibody test (rim pattern), positive LE cell preparation test, elevated antidouble-stranded DNA activity, positive lupus band test (IgM and C3) in involved and uninvolved skin, and renal biopsy findings consistent with SLE. Sclerodermatous change as an initial sign of SLE is rare. We review previous reports of an association of circumscribed scleroderma, especially the linear form, with serologic evidence of systemic autoimmune disease.

(Arch Dermatol 116:69-73, 1980)