先天性自愈性网状组织细胞增生症1例
|
摘要
报告新生儿先天性自愈性网状组织细胞增生症1例。新生儿男,出生5 h。出生时全身多发红色丘疹5 h。皮肤科检查:面部、躯干、双下肢及双足散在分布鲜红色丘疹,黄豆至豌豆大,半球形,表面光滑、无溃疡。反射式共聚焦激光扫描显微镜(RCM)显示:表皮结构紊乱,正常蜂窝状结构消失,表皮浅层可见低折光空洞样外观及较多中度折光的炎性细胞浸润,正常环状真皮乳头状结构消失,真皮层可见大量中度折光的炎性细胞及大量大小不等、形态不一的高折光颗粒状物质浸润。皮肤超声示:表皮隆起、连续性中断,累及表皮层及真皮层,表面光滑,呈均匀低回声,形态不规则,边界欠清,真皮上层可见点状、片状中强回声,可除外血管病变。皮损组织病理:真皮上部可见较多体积较大、胞质丰富的朗格汉斯细胞,细胞核不规则,为肾型或咖啡豆样,可见淋巴细胞及嗜酸性粒细胞浸润。免疫病理:CD1a(+)、S-100蛋白(+)、Langerin(+)。3个月内皮损基本消退,随访10个月余,未见新发皮损及系统性损害。诊断:先天性自愈性网状组织细胞增生症。
A case of congenital self-healing reticulohistiocytosis is reported. A newborn boy was born with multiple hemangioma-like rashes. Physical examination showed soybean to pea sized hemispherical red papules without rupture on his face,trunk, limbs and feet. Reflectance confocal microscopy(RCM) imaging showed that the epidermal structure was disorganized with disappearance of normal honeycomb structures, slightly refractive vacuolar pattern and multiple moderately refractive inflammatory cells in the upper epidermis. Normal dermal papillary rings disappeared. Moderately refractive inflammatory cells and plenty of high-refractive index particles, varying in size and shape, were present in the dermis. Cutaneous ultrasonography showed elevated epidermis with discontinuity. The lesions were irregular with hypoechogenicity, smooth surface and unclear borders in the epidermis and the dermis. Hyperechoic spots were observed in the upper dermis. No evidence supported the diagnosis of vascular diseases. Histopathological examination showed many large Langerhans cells with abundant eosinophilic cytoplasm and irregular, kidney-type or coffee-like nuclei in the upper dermis. Focal infiltration of lymphocytes and eosinophils could also be seen. Immunohistochemical staining showed that histiocytes were positive for CD1 a, S-100 protein and Langerin. Skin rashes resolved spontaneously within 3 months. During over 10 months of follow-up, no new rash and systemic damage were observed. The diagnosis of congenital self-healing reticulohistiocytosis was made.
A case of congenital self-healing reticulohistiocytosis is reported. A newborn boy was born with multiple hemangioma-like rashes. Physical examination showed soybean to pea sized hemispherical red papules without rupture on his face,trunk, limbs and feet. Reflectance confocal microscopy(RCM) imaging showed that the epidermal structure was disorganized with disappearance of normal honeycomb structures, slightly refractive vacuolar pattern and multiple moderately refractive inflammatory cells in the upper epidermis. Normal dermal papillary rings disappeared. Moderately refractive inflammatory cells and plenty of high-refractive index particles, varying in size and shape, were present in the dermis. Cutaneous ultrasonography showed elevated epidermis with discontinuity. The lesions were irregular with hypoechogenicity, smooth surface and unclear borders in the epidermis and the dermis. Hyperechoic spots were observed in the upper dermis. No evidence supported the diagnosis of vascular diseases. Histopathological examination showed many large Langerhans cells with abundant eosinophilic cytoplasm and irregular, kidney-type or coffee-like nuclei in the upper dermis. Focal infiltration of lymphocytes and eosinophils could also be seen. Immunohistochemical staining showed that histiocytes were positive for CD1 a, S-100 protein and Langerin. Skin rashes resolved spontaneously within 3 months. During over 10 months of follow-up, no new rash and systemic damage were observed. The diagnosis of congenital self-healing reticulohistiocytosis was made.
引文
[1]Wang J,Wu X,Xi ZJ.Langerhans cell histiocytosis of bone in children:a clinicopathologic study of 108 cases[J].World JPediatr,2010,6(3):255-259.
[2]Huang CY,Chao SC,Ho SF,et al.Congenital self-healing reticulohistiocytosis mimicking diffuse neonatal hemangiomatosis[J].Dermatology,2004,208(2):138-141.
[3]谢杰,林尔艺,杨斌,等.先天性自愈性朗格汉斯组织细胞增生症一例[J].中华皮肤科杂志,2017,50(6):458.
[4]lasek-Duriez A,Charkaluk Ml,Gosset P,et al.Blueberry Muffin Baby and Langerhans'congenital cell histiocytosis[J].Ann Dermatol Venereol,2014,141(2):130-133.
[5]李伟,戴辉,黎钊,蕈样肉芽肿在反射式共聚焦下的特征[J].中华皮肤科杂志,2013,46(6):431-432.
[6]苏海辉,陈立新,宫泽琨,等.反射式共聚焦显微镜观察儿童朗格汉斯组织细胞增生症14例[J].中华皮肤科杂志,2015,48(10):710-712.
[7]Crisan M,Crisan D,Sannino G,et al.Ultrasonographic staging of cutaneous malignant tumors:an ultrasonographic depth index[J].Arch Dermatol Res,2013,305(4):305-313.
[8]张梅,荣辉,武卓,高频超声在皮肤病诊断及皮肤美容的应用价值[J].临床超声医学杂志,2011,13(6):406-408.
[2]Huang CY,Chao SC,Ho SF,et al.Congenital self-healing reticulohistiocytosis mimicking diffuse neonatal hemangiomatosis[J].Dermatology,2004,208(2):138-141.
[3]谢杰,林尔艺,杨斌,等.先天性自愈性朗格汉斯组织细胞增生症一例[J].中华皮肤科杂志,2017,50(6):458.
[4]lasek-Duriez A,Charkaluk Ml,Gosset P,et al.Blueberry Muffin Baby and Langerhans'congenital cell histiocytosis[J].Ann Dermatol Venereol,2014,141(2):130-133.
[5]李伟,戴辉,黎钊,蕈样肉芽肿在反射式共聚焦下的特征[J].中华皮肤科杂志,2013,46(6):431-432.
[6]苏海辉,陈立新,宫泽琨,等.反射式共聚焦显微镜观察儿童朗格汉斯组织细胞增生症14例[J].中华皮肤科杂志,2015,48(10):710-712.
[7]Crisan M,Crisan D,Sannino G,et al.Ultrasonographic staging of cutaneous malignant tumors:an ultrasonographic depth index[J].Arch Dermatol Res,2013,305(4):305-313.
[8]张梅,荣辉,武卓,高频超声在皮肤病诊断及皮肤美容的应用价值[J].临床超声医学杂志,2011,13(6):406-408.