Congenital solitary histiocytoma: A variant of Hashimoto-Pritzker histiocytosis
Congenital solitary histiocytoma: A variant of Hashimoto-Pritzker histiocytosis
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DOI：
10.1159/000111508
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发表时间：
2008-01-01
期刊：
影响因子：
3.4
通讯作者：
Bernard, P.
中科院分区：
文献类型：
--
作者：
Zunino-Goutorbe, C.;Eschard, C.;Bernard, P.
背景资料：自愈性孤立性病变桥本-普利兹克组织细胞增生症（HPH），一种罕见的，先天性，纯皮肤朗格汉斯组织细胞增生症（仅30例报告），预后良好。目的：探讨孤立性肝豆状核变性的临床和病理特点。方法：对8例新生儿进行回顾性观察研究。结果：对于这些婴儿，其他体格检查正常，独特的结节或丘疹（5-15毫米直径）是先天性的。系统常规组织学检查发现真皮浸润主要由组织细胞、淋巴细胞和嗜酸性粒细胞组成。7例患者的蛋白S100和CD 1a免疫标记均为阳性。电镜下观察到Birbeck颗粒。随访2-12年未见内脏受累或复发。结论：由于其自我愈合的性质，先天性孤立HPH的频率可能被低估了。在没有全身受累的情况下，至少2年的定期体格检查似乎是一种有效的方法。版权所有（c）2008 S. Karger AG，巴塞尔。
Background: Self-healing solitary-lesion Hashimoto-Pritzker histiocytosis (HPH), a rare, congenital, purely cutaneous Langerhans histiocytosis (only 30 cases reported), carries a good prognosis. Objective: To describe the clinical and histopathological characteristics of solitary HPH. Methods: To conduct a retrospective, observational study on 8 affected newborns. Results: For these infants, with otherwise normal physical examinations, the unique nodule or papule (5-15-mm diameter) was congenital. Systematic routine histological examination of the lesions found dermal infiltrates constituted predominantly of histiocytes with lymphocytes and eosinophils. Protein S100 and CD1a immunolabelings, done for 7 patients, were positive. Electron microscopy (n = 4) observed Birbeck granules. No visceral involvement or recurrence has ever been observed after 2-12 years of follow-up. Conclusions: Because of its self-healing nature, congenital solitary HPH frequency has probably been underestimated. In the absence of systemic involvement, regular physical examination for at least 2 years seems a valid approach. Copyright (c) 2008 S. Karger AG, Basel.