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表现为早衰综合征和肾衰竭的青少年系统性肉芽肿病。

Juvenile systemic granulomatosis manifesting as premature aging syndrome and renal failure.

作者信息

Umemoto M, Take H, Yamaguchi H, Tokudome T, Tokunaga M

机构信息

Department of Pediatrics, Kagoshima City Hospital, Japan.

出版信息

J Rheumatol. 1997 Feb;24(2):393-5.

PMID:9035003
Abstract

We describe a case of juvenile systemic granulomatosis in a 22-year-old woman. The rash consisted of purple papules and first appeared at the age of one year. She had persistent symmetrical painless boggy tenosynovitis with minimal roentgenographic changes and chronic granulomatous symptoms. Uveitis resulted in visual impairment. She also had granulomatous changes in her vessels. Renal impairment developed; however, neither renal artery stenosis nor hypercalcemia was found. Clinical features included the development of premature aging with alopecia, which differed from the previously reported progeria syndrome. Poikiloderma may cause a prematurely aged appearance. Our report expands the clinical spectrum of systemic granulomatosis to include the development of premature aging with alopecia.

摘要

我们描述了一例22岁女性的青少年系统性肉芽肿病。皮疹表现为紫色丘疹,一岁时首次出现。她患有持续性对称性无痛性肿胀性腱鞘炎,X线检查变化极小,并伴有慢性肉芽肿症状。葡萄膜炎导致视力受损。她的血管也有肉芽肿性改变。出现了肾功能损害;然而,未发现肾动脉狭窄或高钙血症。临床特征包括出现脱发导致的早衰,这与先前报道的早衰综合征不同。皮肤异色症可能导致过早衰老的外观。我们的报告扩展了系统性肉芽肿病的临床谱,使其包括伴有脱发的早衰的发生。

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