原发性系统性淀粉样变病1例

报告1例原发性系统性淀粉样变病。患者男，39岁。1年前肩关节突然出现酸痛，症状渐渐加重，双手掌皮肤发硬，肢体上抬及下蹲困难，舌不能外伸，吞咽时有梗阻感。曾经患有胸椎椎体骨髓瘤。皮肤科检查示面颈部、双侧腋下、腹部、阴茎、双大腿内侧泛发对称性的蜡样半球形丘疹，有融合，呈肤色或琥珀色。口腔黏膜肥厚，舌弥漫性浸润、质硬，呈巨舌。皮损组织病理检查示表皮萎缩，真皮与皮下组织淀粉样物质沉积，甲基紫染色阳性，诊断为原发性系统性淀粉样变病。给予泼尼松和雷公藤治疗，症状明显改善。

Primary systemic amyloidosis:a case report

A case of primary systemic amyloidosis(PSA)is reported.A male,39years old,suddenly suffered from joint pain1year ago and the symptoms became progressively worsened.Palm skin became stiff,and he had difficulty in lifting and bending limbs,and extruding the tongue.The patient had a history of myeloma of thoracic vertebra.The cutaneous eruption began as smooth,firm,flat-topped papules of waxy color,the tongue became progressively enlarged with translucent papules manifested as macroglossia.Skin biopsies showed atrophy of the epidermis,amyloid substance deposited at the dermis and hy-podermic tissue.Methyl violet staining test was positive.The diagnosis of primary systemic amyloidosis was made and symp-toms markedly improved after the administration of prednisone and Tripterygium Wilfordii Hook.