成人皮肤弥漫性朗格汉斯细胞组织细胞增生症

Cutaneous diffuse Langerhans' cell histiocytosis in adult

目的 探讨成人皮肤朗格汉斯细胞组织细胞增生症 (LCH)的临床病理与免疫组化、电镜特征及鉴别诊断。方法 应用光镜、免疫组化及电镜技术 ,观察 1例病程长及 4次活检证实为成人皮肤弥漫性LCH ,并复习文献。结果 初发皮损全身弥散分布丘疹 ,第一次活检病变限在真皮网状层分布 ,以单核细胞样朗格汉斯细胞 (LC)为主。 4年后 ,皮损发展至全身 ,呈弥漫分布结节状。活检病变真皮全层受累。LC形态多样 :单核细胞、多核细胞、多核巨细胞、泡沫细胞 ;核形圆、椭圆、肾形、分叶状及纵形核沟等。淋巴细胞浸润 ,嗜酸性粒细胞少见。LC表达CD1a、S 10 0蛋白、CD6 8、HLA DR、Vim阳性 ,CD4 5RO异型表达 ,2次电镜观察未见Berbeck颗粒。结论 LCH是一种多态性疾病 ,发生在成人皮肤弥漫性LCH罕见。随病程进展 ,皮损加重 ,病变扩大 。

Purpose To study the clinicopathological, immunohistochemical and ultrastructural characteristics of cutaneous diffuse Langerhans' cell histiocytosis (CDLCH) in adult and the diagnosis of the disease. Methods Tissues from a long-history patient with 4 times biopsy verified as CDLCH at last was examined by using light and electron microscope and immunohistochemistry. The relative literatures were also reviewed. Results The primary skin lesion was diffuse distributing papules in the body, but it was limited in dermis reticular stratum in the first biopsy, mononuclearoid cells were the mainly part. After 4 years, the leision was developed to be diffuse distributing nodules, all the dermis was affected. Langerhans' cells morphy varied from mononuclearoid cells to multinuclearted cells, multinuclearted giant cells, foamy cells. The nuclei shape was round, oval, kidney-like, multilobal or had nuclei grooves. More lymphocyte were infiltrated, few eosinophilic cells were found. Langerhans' cells were positive for CDla, S-100 protein, CD68, HLA-DR, vimentin, but CD45RO expressed atypically. Berbeck body was not found by two times under electron microscope. Conclusion Langerhans' cell histiocytosis is a multimorph kind of disease. CDLCH is rarely seen in adult. With the progression of the disease, the skin lesions become biggerly, more severe, and Langerhans' cells become more multimorphological.