原发性结节性皮肤淀粉样变研究进展

原发性结节性皮肤淀粉样变是原发性皮肤淀粉样变的最少见亚型,其特征为蜡样光泽的黄棕色结节或斑块,组织病理改变为在真皮、皮下及血管壁弥漫性淀粉样蛋白沉积。研究发现,结节性淀粉样变可能与创伤、IL-6家族细胞因子受体基因突变、免疫功能异常等有关。手术切除、电干燥法、刮除术、冷冻术、局部糖皮质激素注射和皮肤磨削术为结节性淀粉样变的主要治疗手段。近年来研究发现局部注射淀粉样蛋白抗体的免疫疗法有望成为该病新的有效的治疗手段。

Advances in primary nodular cutaneous amyloidosis

Primary nodular cutaneous amyloidosis is a rare variant of primary cutaneous amyloidosis characterized by waxy yellowish-brown nodules or plaques. Histopathologic changes include diffuse deposits of amyloid in the dermis, subcutaneous tissue and blood vessel walls. It has been found that the nodular amyloidosis may be related with trauma, IL-6 family cytokine receptor gene mutations and immune dysfunction. Surgical excision, electrodesication, curettage, cryotherapy, intralesional steroid injection and dermabrasion are the main methods for the treatment of nodular myloidosis. Recently, immunotherapy which means subcutaneous injection of antibodies is expected to be a new effective therapy for the disease.