CAJ | 학술논문

目的探讨硬肿病的临床特点.方法北京协和医院1998-2012年住院治疗的8例硬肿病患者的临床表现、病理特征及治疗转归.结果男女患者各4例,平均年龄44岁.皮肤变硬增厚累及颈肩、背部6例；合并多系统受累6例；2型糖尿病5例中3例有糖尿病并发症；2例出现血清异常免疫球蛋f白,λ、κ型M蛋白各1例；发热2例；合并干燥综合征1例.4例行皮肤活检,组织病理主要表现为真皮全层增厚,胶原增生明显,胶原纤维空隙可见阿新蓝染色阳性.8例中3例接受糖皮质激素、免疫抑制剂治疗有效,1例青霉素治疗有效,1例紫外线治疗效果欠佳.结论硬肿病患者应常规监测血糖、筛查血清免疫球蛋白,警惕脏器受累及多发性骨髓瘤、淋巴瘤等恶性疾病.
목적 탐토경종병적림상특점.방법 북경협화의원1998-2012년주원치료적8례경종병환자적림상표현、병리특정급치료전귀.결과 남녀환자각4례,평균년령44세.피부변경증후루급경견、배부6례；합병다계통수루6례；2형당뇨병5례중3례유당뇨병병발증；2례출현혈청이상면역구단f백,λ、κ형M단백각1례；발열2례；합병간조종합정1례.4례행피부활검,조직병리주요표현위진피전층증후,효원증생명현,효원섬유공극가견아신람염색양성.8례중3례접수당피질격소、면역억제제치료유효,1례청매소치료유효,1례자외선치료효과흠가.결론 경종병환자응상규감측혈당、사사혈청면역구단백,경척장기수루급다발성골수류、림파류등악성질병.
Objective To assess the clinical characteristics of patients with scleredema.Methods This retrospective study included eight patients with scleredema hospitalized in the Peking Union Medical College Hospital (PUMCH) from 1998 to 2012.Data were collected from medical records.Clinical manifestations,pathological findings and therapeutic outcomes were analyzed.Results There were four male patients and four female patients with an average age of 44 years.Sclerotic induration of the skin on the neck,shoulders and back were observed in 6 (75％) patients,and multi-systemic involvement in 6 patients.Three of five patients with type 2 diabetes millitus (DM) suffered from diabetes-associated complications.Lanbda type M-protein was observed in one patient,and Kappa type M-protein in one patient.Two patients also suffered from fever,and one from Sj?gren's syndrome.Pathological examination of biopsy specimens from four patients revealed thickening of the whole dermis as well as marked hyperpasia of collagen.Alcian blue staining was positive in the space between collagen fibers.The condition was improved in three patients treated with glucocorticoid and immunosuppressants as well as in one patient receiving benzylpenicillin treatment,but poorly responded to ultraviolet therapy in one patient.Conclusions It is recommended to monitor blood glucose and serum immunoglobulins as well as to screen for the involvement of multiple systems and occurrence of multiple myeloma,lymphoma and other malignancies among patients with scleredema.