近端指间关节周围胶原沉积症2例并文献复习  

Pachydermodactyly: 2 Cases Report and Literature Review

作　　者：陈宇[1] 姜忠[2] 邵世宏[3] 常红[1] 邵乐平 林毅[1] 

机构地区：[1]青岛大学附属医院儿科,山东 青岛 [2]青岛大学附属医院小儿外科,山东 青岛 [3]青岛大学附属医院病理科,山东 青岛 [4]青岛市立医院肾脏科,山东 青岛

出　　处：《临床医学进展》2023年第6期9385-9390,共6页Advances in Clinical Medicine

摘　　要：目的:探讨近端指间关节周围胶原沉积症(pachydermodactyly, PDD)的临床特点,提高对该病的认识。方法:对2例确诊PDD患儿的临床资料进行总结分析并复习相关文献。结果:2例患儿均表现为双手近端指间关节肿胀,无关节痛;查体可见受累关节侧面呈梭形膨大,肤色、皮温正常,无触痛;炎症指标、免疫学检查均无异常;影像学检查示双手近端指间关节软组织肿胀,无关节、骨质异常改变;皮肤组织病理学检查示复鳞上皮轻度增生伴角化过度,间质纤维胶原组织增生,诊断为PDD,未治疗,均随访3个月以上,关节症状无进展。结论:PDD是一种少见的良性疾病,通常无需特殊处理,临床上需和炎症性关节炎相鉴别,避免误诊、误治。Objective: To explore the clinical features of pachydermodactyly (PDD) and to improve the under-standing of PDD. Methods: Summarize and analyze the clinical data of 2 cases of PDD and review relevant literature. Results: Both patients showed swelling of proximal interphalangeal joints of both hands without arthralgia. Physical examination showed fusiform enlargement of the lateral surface of the involved joint, normal skin color and temperature, no tenderness;inflammatory in-dex and immunological examination were normal;imaging examination showed swelling of the soft tissue of the proximal interphalangeal joints of both hands, without abnormal changes of joints and bones. Histopathological examination of the skin showed mild hyperplasia of squamous epithelium with hyperkeratosis and interstitial fibrous collagen hyperplasia, which was diagnosed as PDD. No treatment was given. All patients were followed up for more than 3 months, and the joint symptoms did not progress. Conclusion: PDD is a rare benign disease, usually without special treatment, clini-cal and inflammatory arthritis should be differentiated to avoid misdiagnosis and mistreatment.

关 键 词：近端指间关节周围胶原沉积症 胶原沉积 儿童 

分 类 号：R73[医药卫生—肿瘤]