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

Takafumi TAGUCHI; Yasumasa IWASAKI; Koichi ASABA; Toshinori YOSHIDA; Toshihiro TAKAO; Fuminori IKENO; Hideaki NAKAJIMA; Hajime KODAMA; Kozo HASHIMOTO

doi:10.1507/endocrj.K07E-044

ORIGINALS

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

Takafumi TAGUCHI, Yasumasa IWASAKI, Koichi ASABA, Toshinori YOSHIDA, Toshihiro TAKAO, Fuminori IKENO, Hideaki NAKAJIMA, Hajime KODAMA, Kozo HASHIMOTO

Author information

Takafumi TAGUCHI
Departments of Endocrinology, Metabolism, and Nephrology, Kochi Medical School, Kochi University
Yasumasa IWASAKI
Departments of Endocrinology, Metabolism, and Nephrology, Kochi Medical School, Kochi University
Koichi ASABA
Departments of Endocrinology, Metabolism, and Nephrology, Kochi Medical School, Kochi University
Toshinori YOSHIDA
Departments of Endocrinology, Metabolism, and Nephrology, Kochi Medical School, Kochi University
Toshihiro TAKAO
Departments of Endocrinology, Metabolism, and Nephrology, Kochi Medical School, Kochi University
Fuminori IKENO
Department of Dermatology, Kochi Medical School, Kochi University
Hideaki NAKAJIMA
Department of Dermatology, Kochi Medical School, Kochi University
Hajime KODAMA
Department of Dermatology, Kochi Medical School, Kochi University
Kozo HASHIMOTO
Departments of Endocrinology, Metabolism, and Nephrology, Kochi Medical School, Kochi University

Keywords: Erdheim-Chester disease, Osteopontin, Survivin, Xanthogranuloma, Diabetes insipidus, Non-Langerhans histiocytosis

JOURNAL FREE ACCESS

2008 Volume 55 Issue 1 Pages 217-223

DOI https://doi.org/10.1507/endocrj.K07E-044

View "Advance Publication" version (February 13, 2008).

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Abstract

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.

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