Emir S, Kutluk T, Topaloğlu R, Bakkaloğlu A, Büyükpamukçu M
Department of Pediatric Oncology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Clin Exp Rheumatol. 2001 Mar-Apr;19(2):221-3.
The association of malignancy with scleroderma is very rare in childhood. A 13-year-old girl was diagnosed as having thymic carcinoma and received systemic chemotherapy. She presented with symptoms of Raynaud's phenomenon 9 months after the cessation of chemotherapy. She also had difficulty in swallowing. Based on the presence of Raynaud's phenomenon, characteristic skin changes over the face and hands, oesophageal involvement and pulmonary restrictive defect demonstrated by pulmonary function tests, the diagnosis of generalised scleroderma was established. There was no evidence of tumor recurrence. Although she was treated with penicillamine and prednisolone, no significant improvement was achieved in her condition during the 14-month follow up.
恶性肿瘤与硬皮病的关联在儿童时期非常罕见。一名13岁女孩被诊断为胸腺癌并接受了全身化疗。化疗结束9个月后，她出现了雷诺现象的症状。她还存在吞咽困难。基于雷诺现象的存在、面部和手部典型的皮肤变化、食管受累以及肺功能测试显示的肺限制性缺陷，确诊为全身性硬皮病。没有肿瘤复发的证据。尽管她接受了青霉胺和泼尼松龙治疗，但在14个月的随访期间病情没有明显改善。
