Rodriguez-Jurado R, Duran-McKinster C, Ruiz-Maldonado R
Department of Pathology, National Institute of Pediatrics of Mexico, Mexico City.
Am J Dermatopathol. 2000 Feb;22(1):70-4. doi: 10.1097/00000372-200002000-00014.
Benign cephalic histiocytosis (BCH) is best understood as a form of non-Langerhans cell histiocytosis, specifically as an early mononuclear variant of juvenile xanthogranuloma (JXG). However, the progression of BCH into JXG in the same patient has only been reported once before. We describe the case of a 2-year-old girl with asymptomatic, large, ill-defined infiltrated flat plaques over both cheeks, in addition to isolated papules. A punch biopsy of a plaque revealed dermal infiltration by vacuolated and scalloped histiocytes positive for CD68 KP-1, and that lacked expression of CD1a and S-100 protein, favoring macrophages over Langerhans cells. Electron microscopy study showed comma-shaped intracytoplasmic bodies in the histiocytic cells leading to the diagnosis of BCH. One year later, after an episode of varicella-zoster infection, the flat plaques over the cheeks became large reddish-yellow nodules, and in a second biopsy appeared to progress to JXG. Virus-related mechanisms of progression are discussed.
良性头部组织细胞增多症（BCH）最好被理解为一种非朗格汉斯细胞组织细胞增多症，具体而言是幼年性黄色肉芽肿（JXG）的早期单核细胞变体。然而，同一患者中BCH进展为JXG的情况此前仅被报道过一次。我们描述了一名2岁女童的病例，其双颊有无症状的、边界不清的大片浸润性扁平斑块，此外还有孤立的丘疹。对一块斑块进行的打孔活检显示，真皮内有呈空泡状且边缘呈扇贝样的组织细胞浸润，这些组织细胞CD68 KP-1呈阳性，且不表达CD1a和S-100蛋白，提示为巨噬细胞而非朗格汉斯细胞。电子显微镜研究显示组织细胞内有逗号状胞浆体，从而确诊为BCH。一年后，在一次水痘-带状疱疹感染发作后，双颊的扁平斑块变成了大的红黄色结节，第二次活检显示似乎进展为JXG。本文讨论了与病毒相关的进展机制。
