Congenital Self-Healing Reticulohistiocytosis Mimicking Diffuse Neonatal Hemangiomatosis
Congenital Self-Healing Reticulohistiocytosis Mimicking Diffuse Neonatal Hemangiomatosis
复制标题
先天性自愈性网状组织细胞增多症，类似弥漫性新生儿血管瘤病
DOI：
10.1159/000076488
复制
发表时间：
2004
期刊：
影响因子：
3.4
通讯作者：
Julia Yu‐Yun Lee
中科院分区：
文献类型：
--
作者：
Ching;S. Chao;S. Ho;Julia Yu‐Yun Lee
先天性自愈性网状组织细胞增生症（CSRH）是郎格罕细胞组织细胞增生症（LCH）的一种罕见的良性变异，其特征在于（a）先天性皮肤病变，（B）健康婴儿无全身受累，（c）郎格罕细胞浸润的组织病理学发现，（d）出生后第一年内自发退化而无后遗症。我们报告一个台湾女孩，出生时就有广泛的血管瘤样病变。通过发现S-100蛋白和OKT 6（CD 1a）阳性单核细胞的弥漫性真皮浸润和10%的单核细胞超微结构中存在Birbeck颗粒，证实了LCH的诊断。3个月内病灶迅速完全消退，进一步确立了CSRH的诊断。术后7年无复发。我们的病例说明CSRH在临床上可以模仿弥漫性新生儿血管瘤病，因此，将CSRH纳入先天性或新生儿血管瘤病的鉴别诊断是很重要的。
Congenital self-healing reticulohistiocytosis (CSRH), a rare benign variant of Langerhans cell histiocytosis (LCH), is characterized by (a) congenital skin lesions, (b) a healthy infant with no systemic involvement, (c) the histopathologic finding of a Langerhans cell infiltrate and (d) spontaneous involution within the first year of life without sequelae. We report a Taiwanese girl born with widespread hemangioma-like lesions. The diagnosis of LCH was confirmed by finding a diffuse dermal infiltrate of S-100-protein- and OKT6 (CD1a)-positive mononuclear cells and the presence of Birbeck granules in 10% of the mononuclear cells ultrastructurally. The diagnosis of CSRH was further established by rapid and complete involution of the lesions in 3 months. No recurrence was noted for 7 years. Our case illustrates that CSRH can mimic diffuse neonatal hemangiomatosis clinically; thus, it is important to include CSRH in the differential diagnosis of congenital or neonatal hemangiomatosis.