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[全身性发疹性组织细胞增多症 - 幼年性黄色肉芽肿:一名儿科患者的临床谱]

[Generalized eruptive histiocytosis-Juvenile xanthogranuloma: clinical spectrum in a pediatric patient].

作者信息

Alperovich Ruth, Grassino Pedro T, Asial Raúl, Pasteris Luis, Boente María Del Carmen

机构信息

Servicio de Dermatología. Hospital del Niño Jesús. San Miguel de Tucumán.

Servicio de Diagnóstico por Imágenes. Hospital del Niño Jesús. San Miguel de Tucumán.

出版信息

Arch Argent Pediatr. 2017 Apr 1;115(2):e116-e119. doi: 10.5546/aap.2017.e116.

Abstract

Both, generalized eruptive histiocytosis and juvenile xanthogranuloma are dendritic histiocytic disorders (also known as non-Langerhans cells histiocytosis) that share clinicopathological and immunohistiochemical characteristics. We present a 3-year-old female patient with skin lesions that were clinically compatible with generalized eruptive histiocytosis, confirmed by histopathological and immunohistochemical studies. During her development the disorder compromised the central nervous system, and surgical intervention of one symptomatic lesion was needed. The histopathological exam of the central nervous system lesion showed Touton cells, compatible with a diagnosis of juvenile xanthogranuloma. This case demonstrates the need to consider these diseases as a spectrum of the same entity.

摘要

全身性发疹性组织细胞增多症和幼年性黄色肉芽肿均为树突状组织细胞疾病(也称为非朗格汉斯细胞组织细胞增多症),它们具有临床病理和免疫组织化学特征。我们报告一名3岁女性患者,其皮肤病变在临床上与全身性发疹性组织细胞增多症相符,经组织病理学和免疫组织化学研究证实。在其病程中,该疾病累及中枢神经系统,需要对一个有症状的病变进行手术干预。中枢神经系统病变的组织病理学检查显示有 Touton 细胞,符合幼年性黄色肉芽肿的诊断。该病例表明需要将这些疾病视为同一实体的不同表现形式。

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