Lee D D, Huang C Y, Wong C K
Department of Dermatology, Veterans General Hospital-Taipei and School of Medicine, National Yang-Ming University, Republic of China.
Am J Dermatopathol. 1998 Oct;20(5):438-42. doi: 10.1097/00000372-199810000-00001.
The clinical findings of systemic amyloidosis are often variable and nonspecific. The early recognition of this disorder relies upon clinical suspicion, and definite diagnosis requires the confirmation of amyloid deposits in tissue. We reviewed 31 skin biopsy specimens from 20 patients, including 17 with amyloid light chain (AL) protein amyloidosis and 3 with serum amyloid A (AA) protein amyloidosis. Histologically, amyloid can be present in the papillary dermis, reticular dermis, subcutis, around the appendages, and in or around blood vessels. In our series, all 20 patients had at least one positive skin biopsy. Amyloid was found in 30 of 31 specimens (97%), a higher rate than in most series. Skin biopsy is a simple, safe procedure with high yield and may be used to obtain a firm diagnosis of systemic amyloidosis.
系统性淀粉样变性的临床症状往往多变且不具特异性。对该疾病的早期识别依赖于临床怀疑，而明确诊断需要在组织中证实淀粉样沉积物的存在。我们回顾了20例患者的31份皮肤活检标本，其中17例为淀粉样轻链（AL）蛋白淀粉样变性，3例为血清淀粉样蛋白A（AA）蛋白淀粉样变性。组织学上，淀粉样物质可存在于乳头层真皮、网状真皮、皮下组织、附属器周围以及血管内或血管周围。在我们的病例系列中，所有20例患者至少有一次皮肤活检呈阳性。31份标本中有30份（97%）发现了淀粉样物质，这一比例高于大多数病例系列。皮肤活检是一种简单、安全且阳性率高的检查方法，可用于获得系统性淀粉样变性的确切诊断。
