Taylor-Gjevre Regina, Saxena Anurag, El Maadawy Sharif, Classen Dale, Nair Bindu, Gjevre John
Department of Medicine, University of Saskatchewan, Saskatoon, Canada.
J Clin Rheumatol. 2009 Mar;15(2):78-80. doi: 10.1097/RHU.0b013e31819b87cd.
We report a 39-year-old man with pachydermodactyly resulting in progressive digital deformation. Pachydermodactyly is a rare superficial benign digital fibromatosis affecting the proximal interphalangeal joints (PIPs). Our patient had flexion deformities and mal-alignment primarily at the PIPs with associated soft tissue swelling. Radiographs revealed nonerosive subluxation. Surgical examination and biopsy with subsequent immunohistopathological study, revealed increased collagen production and proliferation from both fibroblasts and myofibroblasts consistent with pachydermodactyly. This patient differs from cases previously reported as the extent of soft tissue involvement altered the integrity of the articular supporting structures. This has resulted in deformity and functional impairment.
我们报告了一名39岁患有厚皮性厚骨膜病的男性患者,该病导致了渐进性手指变形。厚皮性厚骨膜病是一种罕见的累及近端指间关节(PIP)的浅表良性手指纤维瘤病。我们的患者主要在近端指间关节处出现屈曲畸形和排列不齐,并伴有软组织肿胀。X线片显示无侵蚀性半脱位。手术检查及活检,随后进行免疫组织病理学研究,结果显示成纤维细胞和肌成纤维细胞的胶原蛋白生成及增殖增加,这与厚皮性厚骨膜病相符。该患者与先前报道的病例不同,因为软组织受累的程度改变了关节支撑结构的完整性,进而导致了畸形和功能障碍。