Bolibar Esther Pages, Raya Judith Sánchez, Simeon Carmen-Pilar, Montesinos Lluisa, Jou Nuria, Cuxart Ampar
Vall d'Hebron Hospital, Physical Medicine and Rehabilitation, Avda Vall d'Hebron 115, Barcelona, Barcelona, 08035, Spain.
BMJ Case Rep. 2009;2009. doi: 10.1136/bcr.05.2009.1899. Epub 2009 Dec 22.
This case report concerns a 49-year-old woman whose physical examination was remarkable for symmetrical swollen hands, fingers and palmar fascial thickening with erythema. The fingers showed flexion contractures. Examination also revealed markedly limited bilateral shoulder and limited knee flexion.The patient's symptoms were treated with rehabilitation. The immunological laboratory investigations were normal. A technetium scan showed a slightly increased uptake in both shoulders, wrists, hips, knees and ankles. CT revealed a pelvic solid mass next to the uterus. After the patient underwent a total hysterectomy and anexectomy, the polyarthritis showed a gradual improvement but the contractures in the hands persisted.Palmar fasciitis and polyarthritis syndrome (PFPAS) is an uncommon rheumatic disorder consisting of pain, swelling, stiffness, progressive flexion contractures of both hands and thickening of palmar fascia, with erythrosis. It was first described as a paraneoplastic phenomenon with ovarian carcinoma in 1982.The characteristic hand deformities of PFPA should alert the clinician to search for an underlying malignant disease.
本病例报告涉及一名49岁女性，其体格检查显示双手、手指对称性肿胀，掌筋膜增厚伴红斑，十分引人注目。手指出现屈曲挛缩。检查还发现双侧肩部明显活动受限，膝关节屈曲受限。患者的症状接受了康复治疗。免疫实验室检查结果正常。锝扫描显示双肩、双腕、双髋、双膝和双踝摄取略有增加。CT显示子宫旁有盆腔实性肿块。患者接受全子宫切除及附件切除术后，多关节炎逐渐改善，但手部挛缩仍然存在。掌筋膜炎和多关节炎综合征（PFPAS）是一种罕见的风湿性疾病，表现为疼痛、肿胀、僵硬、双手进行性屈曲挛缩以及掌筋膜增厚伴红斑。它于1982年首次被描述为一种与卵巢癌相关的副肿瘤现象。PFPA的典型手部畸形应提醒临床医生寻找潜在的恶性疾病。
