Division of Rheumatology, Department of Physical Medicine and Rehabilitation, Firat University, Faculty of Medicine, Elazig, Turkey.
Rheumatol Int. 2012 Jun;32(6):1747-50. doi: 10.1007/s00296-011-1895-x. Epub 2011 Mar 26.
Pachydermodactyly is a rare digital fibromatosis characterized by asymptomatic fusiform soft-tissue swellings of the proximal interphalangeal joints of the hands. It usually affects healthy adolescent males with a negative family history. As a rule, clinical presentation of the disease is bilateral and symmetrical enlargement of the joints. So it can be misdiagnosed with inflammatory rheumatic diseases, especially with juvenile chronic arthritis. A prompt clinical diagnosis of the disease would prevent inappropriate treatment with immunosuppressive agents or steroids and unnecessary expensive diagnostic procedures such as biopsy or magnetic resonance imaging. Once diagnosed, patients should be advised in order to avoid repetitive traumas of the hands, rubbing and cracking of the fingers, obsessive-compulsive use of computer and video games. The joint outcome is always benign. Here, we report a case of pachydermodactyly differs from the typical clinical picture of pachydermodactyly in the unilateral distribution of the lesions.
厚皮性骨膜病是一种罕见的手指近端指间关节纤维瘤病,表现为无症状的梭形软组织肿胀。它通常发生于无家族史的健康青少年男性,该病的临床表现通常为双侧、对称性关节肿大,因此易误诊为炎症性风湿性疾病,尤其是青少年慢性关节炎。及时的临床诊断可以避免使用免疫抑制剂或类固醇进行不适当的治疗,以及进行不必要的昂贵诊断程序,如活检或磁共振成像。一旦确诊,应建议患者避免手部重复性创伤、手指摩擦和皲裂、强迫性使用电脑和视频游戏。关节预后通常良好。本文报道了 1 例单侧分布病变的厚皮性骨膜病,其临床表现与典型的厚皮性骨膜病不同。