Konopinski Jonathan C, Seyfer Sarah J, Robbins Kerri L, Hsu Sylvia
Baylor College of Medicine, Houston, Texas.
Dermatol Online J. 2013 Apr 15;19(4):10.
Nodular cutaneous amyloidosis (NCA) is the rarest form of primary cutaneous amyloidosis. The amyloid fibrils of NCA are not unique to NCA but are also the prevailing amyloid component in primary systemic amyloidosis (PSA) and myeloma-associated systemic amyloidosis. Age of presentation in NCA has ranged from 20 to 87 years without a clear gender predilection. Progression from NCA to primary systemic amyloidosis has been reported, with an estimated lifetime risk of approximately 7 percent, prompting the need for appropriate follow up to evaluate for the presence of systemic amyloidosis. We report a case of nodular cutaneous amyloidosis in an otherwise healthy 62-year-old woman and we review the literature.
结节性皮肤淀粉样变性（NCA）是原发性皮肤淀粉样变性最罕见的形式。NCA的淀粉样原纤维并非NCA所特有，也是原发性系统性淀粉样变性（PSA）和骨髓瘤相关性系统性淀粉样变性中主要的淀粉样成分。NCA的发病年龄在20至87岁之间，无明显性别倾向。已有报道称NCA可进展为原发性系统性淀粉样变性，估计终生风险约为7%，因此需要进行适当的随访以评估是否存在系统性淀粉样变性。我们报告一例62岁健康女性的结节性皮肤淀粉样变性病例，并对相关文献进行综述。
