Reichert C M, Costa J, Barsky S H, Claysmith A P, Liotta L A, Enzinger F M, Triche T J
Clin Orthop Relat Res. 1985 Apr(194):252-7.
Pachydermodactyly is a rare distinct form of fibromatosis characterized by dense fibrosis and fibroblastic proliferation around one or more proximal interphalangeal joints. Pachydermodactyly affects the appositional and dorsal aspects of proximal digits II-V. The lateral aspects of the index fingers, the medial aspect of the fifth digits, and the thumbs are usually unaffected. A typical patient was a young adult man with a long history of idiopathic asymptomatic swelling in the fingers. Skin biopsy specimens obtained from three patients with pachydermodactyly showed marked thickening of the dermis with extension of collagenous fibers into the subcutaneous tissues. There was poor demarcation between the papillary and reticular dermis. The cytology of the fibroblasts appeared benign. Isolation of collagen from the involved sites revealed collagen Types I, III, and V in a pattern typical of fibromatoses; i.e. increased Types III and V. Compared with the collagen profile of normal reticular dermis, increased numbers of fine-diameter collagen fibers were notable in electron micrographs.
厚皮性多指（趾）纤维瘤病是一种罕见的特殊类型的纤维瘤病，其特征是一个或多个近端指间关节周围出现致密纤维化和成纤维细胞增殖。厚皮性多指（趾）纤维瘤病累及示指至小指近端指节的掌侧和背侧。示指的外侧、小指的内侧和拇指通常不受影响。典型患者为青年男性，有手指特发性无症状肿胀的长期病史。对3例厚皮性多指（趾）纤维瘤病患者进行的皮肤活检标本显示，真皮明显增厚，胶原纤维延伸至皮下组织。乳头层和网状真皮之间分界不清。成纤维细胞的细胞学表现为良性。从受累部位分离出的胶原蛋白显示出纤维瘤病典型的I型、III型和V型胶原蛋白模式；即III型和V型增加。与正常网状真皮的胶原蛋白谱相比，电子显微镜照片中可见细直径胶原纤维数量增加。
