Waton J, Fays-Michel S, Chandeclerc M L, Corby S, Cuny J F, Barbaud A, Schmutz J-L
Service de dermatologie, hôpital Fournier-CHU, 36, quai de la Bataille, 54000 Nancy, France.
Ann Dermatol Venereol. 2008 Feb;135(2):119-22. doi: 10.1016/j.annder.2007.01.003. Epub 2008 Feb 1.
AA amyloidosis, secondary to inflammatory chronic diseases like rheumatoid arthritis, is often complicated by renal failure. Chronic inflammatory dermatoses constitute rare causes of AA amyloidosis.
CASE-REPORT: We describe two cases of AA amyloidosis discovered after renal failure in patients presenting leg ulcers for several years. AL amyloidosis was suspected in both cases because of a history of monoclonal gammopathy in one patient and of plasmocytoma in the other. The diagnosis of AA amyloidosis was confirmed on renal histology through the detection of AA antibodies in amyloid deposits. No extrarenal amyloidosis was seen in either patient and there were no inflammatory diseases other than chronic leg ulcers.
AA amyloidosis is caused by serum amyloid protein A (SAA), a reactive inflammatory protein. AA amyloidosis is thus caused by chronic inflammatory diseases, but only rarely by cutaneous inflammatory diseases. To our knowledge, the literature contains only seven other published cases of AA amyloidosis secondary to chronic leg ulcers. A review of the literature does not indicate whether cure of ulcers has any effect on the accompanying renal failure. We imagine that AA amyloidosis secondary to leg ulcer is in fact under-diagnosed. However, since the first specific treatment for AA amyloidosis is currently being evaluated by the Food and Drug Administration, it is essential that this serious complication of chronic leg ulcers be widely recognised.
继发于类风湿关节炎等慢性炎症性疾病的AA型淀粉样变性常并发肾衰竭。慢性炎症性皮肤病是AA型淀粉样变性的罕见病因。
我们描述了两例在出现腿部溃疡数年的患者发生肾衰竭后发现的AA型淀粉样变性病例。由于其中一名患者有单克隆丙种球蛋白病病史,另一名患者有浆细胞瘤病史,两例均怀疑为AL型淀粉样变性。通过在淀粉样沉积物中检测到AA抗体,肾组织学检查确诊为AA型淀粉样变性。两名患者均未发现肾外淀粉样变性,除慢性腿部溃疡外无其他炎症性疾病。
AA型淀粉样变性由血清淀粉样蛋白A(SAA)引起,SAA是一种反应性炎症蛋白。因此,AA型淀粉样变性由慢性炎症性疾病引起,但很少由皮肤炎症性疾病引起。据我们所知,文献中仅另有七例继发于慢性腿部溃疡的AA型淀粉样变性病例报道。文献综述未表明溃疡的治愈对伴发的肾衰竭是否有任何影响。我们认为继发于腿部溃疡的AA型淀粉样变性实际上诊断不足。然而,由于美国食品药品监督管理局目前正在评估AA型淀粉样变性的首个特异性治疗方法,慢性腿部溃疡的这一严重并发症必须得到广泛认识。