Localized scleroderma en coup de sabre exacerbated during pregnancy followed by postpartum development of rheumatoid arthritis.

摘要

A 32-year-old Japanese woman with a history of Hashimoto's disease visited us complaining of a 10-year history of a frontal, slightly depressed, linear, shiny, scleros-ing plaque and left frontotemporal pigmentation (figure 1A). Based on the typical clinical manifestations and the nistopathological examination showing dermal fibrosis and mild perivascular lymphocytic infiltrates (figure IB), she was diagnosed with localized scleroderma (LSc) en coup de sabre. Erythrocyte sedimentation rate (58 mm/h, normal: <18), immunoglobulin G (2,547 mg/dL, normal: < 1,700), rheumatoid factor (91 IU/mL, normal: <20.0), and an antinuclear antibody titer (1:320) were elevated, while anti-single-stranded DNA antibody titer was within normal range. The disease course remained stable under topical corticosteroids over a couple of years. Five years later, however, the frontal sclerotic lesion gradually became wider and extended to the vertex at around gestation week 25 of her first pregnancy. Furthermore, the left frontotemporal pig-mented lesion progressed to a sclerosing plaque (figures 1C, D).