Matucci-Cerinic M, Cinti S, Morroni M, Lotti T, Nuzzaci G, Lucente E, di Lollo S, Ceruso M, Cagnoni M
Istituto di Clinica Medica IV, Patologia Medica I, Università di Firenze, Italy.
Ann Rheum Dis. 1989 Mar;48(3):240-6. doi: 10.1136/ard.48.3.240.
A case of pachydermoperiostosis characterised by the presence of finger clubbing, periostosis, sweating of hands and feet is described. Modifications of capillaroscopic pattern and of arteriovenous anastomoses are reported. The periungual border and finger tip tissue showed diffuse endothelial hyperplasia, hyalinosis, and sclerosis with packing of collagen fibres. Electron microscopy showed hypertrophic and activated endothelia (numerous and hypertrophic Golgi complexes, several Weibel-Palade bodies, vesicles of micropinocytosis, and glycogen particles), the basal membrane thickened and reduplicated, perivasal infiltrate in superficial derma, reticulation and segmentary reduplication of basal membrane in arteriovenous shunt. In the perineural connective tissue numerous Luse bodies (long spacing collagen) were evident. The data indicate that in the early phase of pachydermoperiostosis morphological endothelial and collagen fibre abnormalities are present, though there is a normal peripheral blood flow.
描述了一例以杵状指、骨膜增生、手脚多汗为特征的厚皮性骨膜病。报告了毛细血管镜检查模式和动静脉吻合的改变。甲周边界和指尖组织显示弥漫性内皮细胞增生、透明变性和硬化，伴有胶原纤维堆积。电子显微镜显示内皮细胞肥大并活化（高尔基体复合体众多且肥大、多个魏-帕小体、微饮小泡和糖原颗粒），基底膜增厚并重复，真皮浅层血管周围浸润，动静脉分流处基底膜呈网状和节段性重复。在神经周围结缔组织中可见大量卢斯小体（长间距胶原）。数据表明，在厚皮性骨膜病的早期阶段，尽管外周血流正常，但存在形态学上的内皮细胞和胶原纤维异常。
