原发性系统性淀粉样变病

Primary systemic amyloidosis:a case report

报告1例原发性系统性淀粉样变病。患者男,68岁。双上肢麻木、舌大伴言语不清1年。皮肤科检查:舌弥漫性肿大,中央见裂纹,裂纹两侧见鹅卵石样排列丘疹,舌缘有齿痕;双手指屈曲畸形,双手大鱼际萎缩发硬;双侧上眼睑可见瘀斑及丘疹。皮损组织病理检查:真皮浅层、深层血管周围及血管内可见嗜伊红团块沉积;刚果红染色(+)。诊断:原发性系统性淀粉样变病。

A case of primary systemic amyloidosis is reported.A 68-year-old male presented with numbness in both upper limbs and enlarged tongue with slurred speech for 1 year.Physical examination showed macroglossia with central scalloping.There were cracks and pebble-like papules on the top and both sides of the tongue as well as teeth marks on the edge of the tongue.There was atrophy and hardening of the thenar of both hands.Several papules and one ecchymosis were seen on the both upper eyelids.Histopathological examination showed pale pink,hyaline material deposited in the dermis and around blood vessels.Congo red stain was positive.A diagnosis of primary systemic amyloidosis was made.