Erdheim-Chester Disease: Report of a Case with PCR-based Analysis of the Expression of Osteopontin and Survivin in Xanthogranulomas Following Glucocorticoid Treatment

Endocrine Journal

Hideaki NAKAJIMA¹  Kozo HASHIMOTO²  Koichi ASABA²  Hajime KODAMA¹  Toshinori YOSHIDA²  Toshihiro TAKAO²  Yasumasa IWASAKI²  Fuminori IKENO¹  Takafumi TAGUCHI² 

[1] Department of Dermatology, Kochi Medical School, Kochi University;Departments of Endocrinology, Metabolism, and Nephrology, Kochi Medical School, Kochi University

关键词: Erdheim-Chester disease;    Osteopontin;    Survivin;    Xanthogranuloma;    Diabetes insipidus;    Non-Langerhans histiocytosis;   

DOI  :  10.1507/endocrj.K07E-044

学科分类：内分泌与代谢学

来源: Japan Endocrine Society

【 摘 要 】

References(30)Cited-By(6)Erdheim-Chester disease (ECD) is a form of non-Langerhans histiocytosis. In this report, we show a case of ECD presenting diabetes insipidus and multiple xanthogranulomas received glucocorticoid treatment over a year. During this period, xanthogranulomas improved in response to the glucocorticoid therapy. Furthermore, the expression of osteopontin in xanthogranulomatous tissues significantly decreased following the treatment. Our data show the expression of osteopontin in xanthogranulomatous tissues of ECD. Furthermore, the osteopontin mRNA decreased following glucocorticoid therapy with xanthogranuloma regression, suggesting that the expression level of osteopontin could be a marker of the disease activity of ECD.

【 授权许可】

Unknown