Kanitakis J, Zambruno G, Schmitt D, Cambazard F, Jacquemier D, Thivolet J
INSERM U209, Clinique Dermatologique, Hôp. Ed. Herriot, Lyon, France.
Cancer. 1988 Feb 1;61(3):508-16. doi: 10.1002/1097-0142(19880201)61:3<508::aid-cncr2820610316>3.0.co;2-2.
Congenital self-healing histiocytosis (CSHH) is a rare primary histiocytic skin disorder. Only a few cases have been studied by ultrastructure and immunohistochemistry. Here we report a new case that was investigated using an electron microscope and a panel of monoclonal (MCA) and polyclonal (PCA) antibodies. CSHH cells were found to bear the immunohistochemical phenotype of normal epidermal Langerhans cells (LC) and histiocytosis X (HX) cells (CD1a/c+, CD1b-, CD4+/-, human leukocyte antigen [HLA]-DR/DQ+, S-100+). However, an electron microscope showed a paucity of Birbeck granule (BG)-containing cells. This contrasted with their immunophenotype. This finding, along with other ultrastructural characteristics of CSHH cells, suggests that histologic differences exist between CSHH and HX. However, because no absolute histologic criterion is known that allows unequivocally the differential diagnosis between the two diseases, this distinction currently has to rely on clinical criteria, mainly the regressive course observed within a few months in CSHH. The precise nosologic position of CSHH among other histiocytic syndromes remains unsettled.
先天性自愈性组织细胞增多症（CSHH）是一种罕见的原发性组织细胞性皮肤病。仅有少数病例进行过超微结构和免疫组织化学研究。在此，我们报告一例新病例，该病例采用电子显微镜以及一组单克隆抗体（MCA）和多克隆抗体（PCA）进行了研究。发现CSHH细胞具有正常表皮朗格汉斯细胞（LC）和组织细胞增多症X（HX）细胞的免疫组织化学表型（CD1a/c +、CD1b -、CD4 +/ -、人类白细胞抗原[HLA]-DR/DQ +、S-100 +）。然而，电子显微镜显示含伯贝克颗粒（BG）的细胞数量稀少。这与其免疫表型形成对比。这一发现以及CSHH细胞的其他超微结构特征表明，CSHH与HX之间存在组织学差异。然而，由于目前尚无明确的组织学标准能够明确区分这两种疾病，目前这种区分不得不依赖临床标准，主要是CSHH在数月内观察到的消退过程。CSHH在其他组织细胞综合征中的准确疾病分类位置仍未确定。
