摘要
目的总结系统性硬化症(SSc)并发原发局限性皮肤淀粉样变患者的临床表现。方法对过去20年内北京协和医院诊断的系统性硬化症并发原发局限性皮肤淀粉样变患者的临床资料进行分析并与国际报道病例资料进行比较。结果6例患者的平均年龄为51.1岁(43～60岁),均为女性,平均在诊断系统性硬化症8.5年(5～14年)后发生原发局限性皮肤淀粉样变,最常见的受累部位为四肢(5例)、背部(2例)、胸部(2例)、肩部(2例)和面部(2例)。所有患者都有Raynaud’s现象和肢端硬化,皮肤改变为苔癣样皮疹伴有色素沉积。病理学改变为皮肤乳头内粉红色无定形透明样物质沉积,但血管周围没有类似沉积,刚果红和结晶紫染色均为阳性。3例患者的ESR是升高的,所有患者都有高滴度的ACA,6例ANA阳性,抗Scl-70抗体及其他自身抗体均为阴性,没有其他脏器淀粉样变的证据,临床预后良好。国际报道的7例患者皮肤最常见受累部位为背部,ACA和ANA阳性率低于国内病例。结论原发局限性皮肤淀粉样变是SSc的罕见并发症,最常见的受累皮肤位于四肢和胸背部,所有患者都有高滴度的ACA。局部治疗即可以改善皮肤改变,预后良好。
Objective Report 6 cases of primary localized cutaneous amyloidosis （PLCA） associated with （laSS） in order to understand the clinical characteristics of systemic sclerosis （SSc）. Methods The clinical as well as laboratory features of 6 laSS patients with PLCA were retrospectively reviewed and the data was analyzed. The data reported in the literature were reviewed and compared. Results All 6 patients were female with an average age of 51.1 years old （range：43-60yd）. The average duration of laSS when PLCA was diagnosed was 8.5 years （range： 5-14 years）. The most common skin changes occurred in the extremities（in 5 patients）, back（in 2 patients）, chest（in 2 patients）, shoulder area（in 2 patients） and face （ in 2 patients）, All patients had Raynaud＇s phenomena and sclerodactly. All skin changes were lichen forms with pigmentation. The pathological changes showed pink amorphous hyaline substance deposited within the dermal papillae but not around blood vessels. Both Congo red staining and crystal violet stains were positive in all cases. For laboratory tests, 3 patients had elevated ESR rate. All patients had high titer of Anti-centromere antibody （ACA）, 4 patients had positive anti-nuclear antibody and 1 patient had positive anti-Scl-70 antibody. Other auto-antibodies such as anti-SSA, anti-SSB, anti-RNP and anti-dsDNA antibody were not found. No Bence-Jones protein or M-protein could be detected and no evidence of systemic amyloidosis could be found. All patients were treated with local medications and the skin changes were improved, No death happened due to PLCA. Conclusions PLCA is a rare complication of laSS. It usually happens in patients with long-standing laSS. In generally, all patients had CREST and high titer ACA. Local medication can improve skin changes and the long-term outcome is good.
出处
《北京医学》
CAS
2007年第11期650-652,共3页
Beijing Medical Journal
关键词
硬化症
系统性
淀粉样变
皮肤
Systemic sclerosis Amyloidosis Cutaneous
