Lauter S A, Vasey F B, Hüttner I, Osterland C K
J Rheumatol. 1978 Spring;5(1):85-95.
Pachydermoperiostosis (primary hypertrophic osteoarthropathy) is a familial disorder characterized by clubbing, periosteal bone changes, and furrowing of the skin. A 40 year old male with this syndrome and chronic arthritis was recently studied. The synovial fluid was non-inflammatory. Histologically the synovium exhibited hyperplasia and microvascular alterations. Immunofluorescent staining failed to identify deposits of immunoglobulin. Electron microscopy revealed multilayered basement laminae around small subsynovial blood vessels but no electron-dense deposits as described in studies of hypertrophic pulmonary osteoarthropathy. The pathogenesis is obscure and appears to be non-immunological.
厚皮性骨膜病(原发性肥大性骨关节病)是一种家族性疾病,其特征为杵状指、骨膜骨质改变和皮肤皱纹。最近对一名患有该综合征和慢性关节炎的40岁男性进行了研究。滑膜液无炎症。组织学上,滑膜表现为增生和微血管改变。免疫荧光染色未发现免疫球蛋白沉积。电子显微镜检查显示,滑膜下小血管周围有多层基底膜,但未发现如肥厚性肺骨关节病研究中所描述的电子致密沉积物。其发病机制尚不清楚,似乎是非免疫性的。