Aoki Akiko, Ono Shigeru, Ueda Atsuhisa, Hagiwara Eri, Takashi Tsuji, Ideguchi Haruko, Misumi Midori, Sasaki Tetsuo, Ikezawa Yoshio, Inayama Yoshiaki, Inoue Yuko, Ishigatsubo Yoshiaki
Yokohama City University School of Medicine, First Department of Internal Medicine.
Nihon Rinsho Meneki Gakkai Kaishi. 2002 Apr;25(2):205-11. doi: 10.2177/jsci.25.205.
We described two female patients with primary Sjögren's syndrome associated with localized cutaneous nodular amyloidosis (LCNA), in which amyloid protein was derived from immunoglobulin light chain. Case 1; a 70-year-old female had complained with polyarthralgia, low-grade fever and parotid gland swelling. She was diagnosed as primary Sjögren's syndrome. Three years later she noticed brown color small tumor on the thigh and yellow to brown nodules on the bilateral calves of legs. Skin biopsy from the left thigh revealed amyloid L protein deposition, which was positive for anti-lambda light chain staining, in almost entire dermis. Infiltration of lymphocytes and plasma cells around the amyloid deposit were prominent. Case 2; a 51-year-old female had noticed increasing eruption on the hip. Skin biopsy revealed amyloid L protein deposition in the dermis, which was negative for anti-lambda nor kappa light chain staining. When she was refereed to our hospital, she complained of xerostomia and xerophthalmia. She was diagnosed as primary Sjögren's syndrome. In both cases, histological examination of a minor salivary gland biopsy revealed infiltration of lymphocytes and plasma cells but not amyloid deposit. Serum M protein and urine Bence-Jones protein were not detected. These cases represent localized amyloidosis without systemic involvement. It is widely recognized that Sjögren's syndrome is frequently accompanied by B cell lymphoproliferative disorders. In LCNA, infiltration of plasma cells around the amyloid deposits was frequently prominent. The relation between these two disorders is discussed.
我们描述了两名患有原发性干燥综合征并伴有局限性皮肤结节性淀粉样变（LCNA）的女性患者，其中淀粉样蛋白源自免疫球蛋白轻链。病例1：一名70岁女性，主诉多关节痛、低热和腮腺肿大。她被诊断为原发性干燥综合征。三年后，她注意到大腿上有棕色小肿瘤，双侧小腿有黄色至棕色结节。左大腿皮肤活检显示，几乎整个真皮层都有淀粉样L蛋白沉积，抗λ轻链染色呈阳性。淀粉样沉积物周围淋巴细胞和浆细胞浸润明显。病例2：一名51岁女性，注意到臀部皮疹增多。皮肤活检显示真皮层有淀粉样L蛋白沉积，抗λ和κ轻链染色均为阴性。当她转诊到我院时，主诉口干和眼干。她被诊断为原发性干燥综合征。在这两个病例中，小唾液腺活检的组织学检查显示有淋巴细胞和浆细胞浸润，但无淀粉样沉积物。未检测到血清M蛋白和尿本周氏蛋白。这些病例代表无全身受累的局限性淀粉样变。众所周知，干燥综合征常伴有B细胞淋巴增殖性疾病。在LCNA中，淀粉样沉积物周围浆细胞浸润常常很明显。本文讨论了这两种疾病之间的关系。
