Di Blasi A, de Seta L, Marsilia G M, Coletta S, Siani P, de Rosa I
Servizio di anatomia ed istologia-patologica, Ospedale A. Cardarelli di Napoli (USL 40 Regione Campania).
Pathologica. 1993 Jan-Feb;85(1095):85-90.
Juvenile Xanthogranuloma. Report of a case with hepatic involvement. The Authors present a case of Juvenile Xanthogranuloma (JX) in a 3 months female child with cutaneous and hepatic nodules associated to dyspnea attributable to obstructive bronchopneumopathy. Histologically the lesions are xanthomatous with proliferation of fat-laden histiocytes. The hepatic involvement is characterized by hepatomegaly and yellow nodules on liver surface as seen at laparoscopy. On liver biopsy there is remarkable expansion of portal triad caused by aggregates of large foamy mono-polynuclear histiocytes with Touton giant cells. The cutaneous nodule biopsy shows histiocytic infiltrate in inter-adnexal dermal space with many giant cells holding great lipidic vacuoles. The patient's follow-up is characterized by slow and progressive clinical improvement with resolution of cutaneous, hepatic and pulmonary pathology. The Authors emphasize the differential diagnosis between this systemic form of JX and Langerhans cell Histiocytosis (Histiocytosis X) with multiorgan involvement. This diagnosis is necessary in order to establish therapy and prognosis.
青少年黄色肉芽肿。1例合并肝脏受累的病例报告。作者报告1例3个月大女童的青少年黄色肉芽肿（JX），伴有皮肤和肝脏结节，并因阻塞性支气管肺炎出现呼吸困难。组织学上，病变为黄色瘤，富含脂质的组织细胞增生。肝脏受累表现为肝肿大，腹腔镜检查可见肝脏表面有黄色结节。肝活检显示，由大的泡沫状单核-多核组织细胞和杜顿巨细胞聚集导致门三联显著扩张。皮肤结节活检显示，附属器间真皮间隙有组织细胞浸润，有许多含有大量脂质空泡的巨细胞。患者的随访结果显示，皮肤、肝脏和肺部病变逐渐缓慢好转。作者强调了这种全身性JX与多器官受累的朗格汉斯细胞组织细胞增生症（组织细胞增生症X）之间的鉴别诊断。为了确定治疗方案和预后，这种诊断是必要的。
