Sass U, André J, Song M
Department of Dermatology, C.H.U. Saint-Pierre, Free University of Brussels, Boulevard de Waterloo 129, B-1000 Brussels, Belgium.
Br J Dermatol. 2000 Jan;142(1):133-7. doi: 10.1046/j.1365-2133.2000.03255.x.
Hereditary progressive mucinous histiocytosis is a rare autosomal dominant non-Langerhans cell histiocytosis. We describe a sporadic case of this syndrome in a 64-year-old woman who had multiple dark-red dome-shaped papulonodules located mainly on the back of her hands, forearms and thighs. Light microscopy revealed a circumscribed upper dermal aggregate of ovoid or spindle-shaped histiocytes with abundant mucin deposition. Iron deposits and numerous mast cells were scattered throughout the tumour but giant cells were rare. Electron microscopy revealed a high number of zebra bodies and myeloid bodies in the cytoplasm of the histiocytes. Immunohistochemistry showed positive labelling with alpha-1 antitrypsin, Factor XIIIa and CD68, while CD1a, CD34 and S100 protein were negative. The differential diagnosis of histiocytic syndromes is discussed.
遗传性进行性黏液性组织细胞增生症是一种罕见的常染色体显性非朗格汉斯细胞组织细胞增生症。我们描述了一名64岁女性散发性该综合征病例，其主要在手背、前臂和大腿出现多个暗红色圆顶状丘疹结节。光镜检查显示，真皮上层有边界清楚的卵圆形或梭形组织细胞聚集，伴有大量黏蛋白沉积。铁沉积和大量肥大细胞散在分布于肿瘤中，但巨细胞罕见。电镜检查显示组织细胞胞质内有大量斑马小体和髓样小体。免疫组化显示α-1抗胰蛋白酶、因子ⅩⅢa和CD68呈阳性标记，而CD1a、CD34和S100蛋白呈阴性。文中讨论了组织细胞综合征的鉴别诊断。
