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[将儿童结节病误诊为非朗格汉斯细胞组织细胞增多症并使用肿瘤坏死因子-α拮抗剂治疗]

[Misdiagnosed childhood sarcoidosis as non-Langerhans' cell histiocytosis treated with tumor necrosis factors-α antagonists].

作者信息

Petiti Martin G, Castellanos González M, Sanz Bueno J, Burgués Calderón M, Villar Buil M, Vanaclocha F, Peralto J L

机构信息

Servicio de Dermatología y Anatomía Patológica, Hospital Universitario 12 de Octubre, Madrid, España.

出版信息

An Pediatr (Barc). 2012 Oct;77(4):267-71. doi: 10.1016/j.anpedi.2012.01.024. Epub 2012 May 17.

Abstract

Sarcoidosis is a chronic multisystemic granulomatous disease of unknown origin. Generalised eruptive histiocytosis is a rare, benign, self-healing, non-Langerhans' cell histiocytosis (non-LCH). We report the case of an 8-year-old girl with sarcoidosis who was misdiagnosed as non-LCH. She was treated with oral corticosteroids, methotrexate and adalimumab, but there was insufficient control of ocular disease. The introduction of infliximab achieved a control of the uveitis and enabled the corticosteroid dose to be tapered. In some cases of sarcoidosis the lack of well-organised granuloma formation at the beginning of the disease, and the presence of prominent giant cells may suggest alternative diagnoses, such as non-LCH. Although the experience of tumour necrosis factor-α antagonists use in children with sarcoidosis is limited, these drugs may be helpful for those patients experiencing a severe and refractory disease.

摘要

结节病是一种病因不明的慢性多系统肉芽肿性疾病。全身性发疹性组织细胞增多症是一种罕见的、良性的、可自愈的非朗格汉斯细胞组织细胞增多症(非LCH)。我们报告了一例8岁结节病女孩被误诊为非LCH的病例。她接受了口服糖皮质激素、甲氨蝶呤和阿达木单抗治疗,但眼部疾病控制不佳。英夫利昔单抗的使用实现了葡萄膜炎的控制,并使糖皮质激素剂量得以逐渐减少。在某些结节病病例中,疾病初期缺乏组织良好的肉芽肿形成以及存在突出的巨细胞可能提示其他诊断,如非LCH。尽管肿瘤坏死因子-α拮抗剂在儿童结节病中的使用经验有限,但这些药物可能对那些患有严重难治性疾病的患者有帮助。

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