Scleredema and paraproteinemia. Enhanced collagen production and elevated type I procollagen messenger RNA level in fibroblasts grown from cultures from the fibrotic skin of a patient.
An edematous rash developed on the abdominal skin of a 76-year-old woman who had had diabetes mellitus for ten years. Some months later, the affected skin became thickened and indurated. Histopathologic examination revealed marked dermal fibrosis with excessive deposition of collagen. The patient also had IgA (k-type) paraproteinemia. Fibroblast cultures from the affected and unaffected skin were studied for collagen metabolism. Procollagen synthesis was elevated about sixfold on fibroblasts derived from the affected skin. A similar increase was detected in messenger RNA (mRNA) levels using a complementary DNA clone specific for human pro alpha 1(l) collagen mRNA. The elevated mRNA level could be the result of increased transcriptional activity of collagen genes or decreased degradation of collagen mRNAs. Our findings suggest that increased collagen deposition may account for the marked dermal fibrosis that we observed in this patient.
D000368:Aged; D002478:Cells, Cultured; D003094:Collagen / Q000096:biosynthesis*; D005260:Female; D005347:Fibroblasts / Q000378:metabolism; D005355:Fibrosis; D006801:Humans; D010265:Paraproteinemias / Q000150:complications / Q000378:metabolism; D011347:Procollagen / Q000096:biosynthesis*; D012333:RNA, Messenger / Q000378:metabolism; D012592:Scleredema Adultorum / Q000150:complications / Q000378:metabolism*; D012867:Skin / Q000378:metabolism
D011347:Procollagen; D012333:RNA, Messenger; D003094:Collagen