Treatment of Juvenile xanthogranuloma
Treatment of Juvenile xanthogranuloma
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DOI：
10.1002/pbc.21523
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发表时间：
2008-07-01
影响因子：
3.2
通讯作者：
Whitlock, James A.
中科院分区：
文献类型：
--
作者：
Stover, Daniel G.;Alapati, Srilatha;Whitlock, James A.
青少年黄色肉芽肿（JXG）通常是一种良性的，自限性的皮肤组织细胞疾病。我们报告两例多系统JXG的临床特征更常见于朗格汉斯细胞组织细胞增生症（LCH），包括尿崩症和溶骨性病变。在组织学上，皮肤病变表现出CD-68染色的组织细胞真皮浸润，但S-100和CD la染色为阴性。根据LCH为基础的化疗方案治疗导致症状迅速解决。对多系统JXG病例化疗的文献综述表明，有症状的患者可以成功地用LCH为基础的方案，包括皮质类固醇和长春花生物碱治疗。
Juvenile xanthogranuloma (JXG) is generally a benign, self-limited histiocytic disorder of the skin. We report two cases of multisystem JXG presenting with clinical features more commonly seen in Langerhans cell histiocytosis (LCH), including diabetes insipidus and lytic bony lesions. Histologically, the skin lesions demonstrated a histiocytic dermal infiltrate that stained for CD-68, but S-100 and CD1 a stains were negative. Treatment according to LCH-based chemotherapy regimens resulted in prompt resolution of symptoms. A literature review of multisystern JXG cases treated with chemotherapy suggests that symptomatic patients can successfully be treated with LCH-based regimens that include both corticosteroids and vinca alkaloids.