Department of Dermatology, Affiliated 3rd Hospital of Hangzhou, Hangzhou, China.
J Dermatol. 2011 Aug;38(8):794-7. doi: 10.1111/j.1346-8138.2010.01098.x.
Langerhans cell histiocytosis (LCH), especially with involvement limited to the skin in adults is a rare entity. Primary cutaneous LCH in older patients usually follows a benign course and can regress spontaneously. We report a fatal case of disseminated cutaneous LCH in an elderly woman. A 64-year-old woman presented with confluenced erythematous macule and diffuse papuloscaling and papulocrusted lesions on the trunk that became erosive on the area of axillae and groins. Histopathological study of skin specimens showed extensive epidermotropic and folliculotropic, lichenoid infiltration of Langerhans' cells. Positive immunohistochemical staining for CD1a and S-100 protein in Langerhans' cells, and numerous typical Birbeck granules in the cytoplasm of Langerhans' cells by electron microscopy study confirmed the diagnosis of LCH. Chest X-rays and computed tomography scans showed mild interstitial pneumonia without a honeycomb appearance. The patient was diagnosed with LCH. Her general condition worsened rapidly and she died 1 month after diagnosis. Because extracutaneous involvement of LCH had been ruled out by laboratory and imaging investigations, we would like to believe this case should be classified as "malignant" LCH based on the clinical course.
朗格汉斯细胞组织细胞增生症（LCH），尤其是局限于成人皮肤的病变，较为罕见。老年患者的原发性皮肤 LCH 通常呈良性病程，并可自发消退。我们报告一例老年女性播散性皮肤 LCH 的致死病例。一名 64 岁女性因躯干部融合性红斑性斑疹和弥漫性丘疹鳞屑及丘疹鳞屑性皮损就诊，腋窝和腹股沟处皮损出现糜烂。皮肤标本的组织病理学研究显示广泛的表皮内和毛囊内朗格汉斯细胞的苔藓样浸润。朗格汉斯细胞的 CD1a 和 S-100 蛋白免疫组织化学染色阳性，电镜研究显示细胞质内存在大量典型的 Birbeck 颗粒，从而确诊为 LCH。胸部 X 线和计算机断层扫描显示轻度间质性肺炎，无蜂窝状外观。该患者被诊断为 LCH。她的一般状况迅速恶化，在诊断后 1 个月死亡。由于实验室和影像学检查排除了 LCH 的皮肤外受累，我们认为根据该病例的临床病程，应将其归类为“恶性”LCH。
