限局性皮膚結節性アミロイドーシスを合併した原発性シェーグレン症候群の2例 [in Japanese] Two cases of limited cutaneous nodular amyloidosis with primary Sjogren's syndrome [in Japanese]
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アミロイドL蛋白が限局性に沈着した皮膚結節性アミロイドーシス(LCNA)を合併した原発性シェーグレン症候群の女性2例を報告する.<br> 症例1: 70歳,女性.関節痛,発熱,耳下腺腫脹のため当科受診.原発性シェーグレン症候群と診断した. 3年後右大腿に褐色小結節,両下腿に黄褐色小結節が出現.左下腿の皮膚生検にて真皮全層に抗L鎖(λ)抗体陽性アミロイドの沈着とその周囲にリンパ球と形質細胞の浸潤を認めた.症例2:51歳,女性.腰部皮疹の生検にて真皮にアミロイド沈着が認められ当科紹介.口腔および眼乾燥感あり,原発性シェーグレン症候群と診断した. 2例とも小唾液腺生検でリンパ球と形質細胞の浸潤著明であったが,アミロイドの沈着は認めなかった.また血清M蛋白陰性,尿中B-J蛋白陰性.全身性アミロイドーシスは否定的でLCNAと診断した.<br> 原発性シェーグレン症候群ではB細胞系のリンパ増殖性疾患を合併することが多いが,アミロイドーシスでもアミロイド沈着周囲に形質細胞の増殖が見られることがあり,シェーグレン症候群とLCNAの関連を示唆する症例と考え報告した.
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.<br> 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 deposi-tion, which was positive for anti-λ light chain staining, in almost entire dermis. Infiltration of lymphocytes and plasma cells around the amyloid deposit were prominent.<br> 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-λ nor κ light chain staining. When she was refereed to our hospital, she complained of xerostomia and xerophthalmia. She was diagnosed as primary Sjögren's syndrome.<br> In both cases, histological examination of a minor salivary gland biopsy revealed infiltra-tion 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 with-out systemic involvement.<br> 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.
- Japanese Journal of Clinical Immunology
Japanese Journal of Clinical Immunology 25(2), 205-211, 2002-04-30
The Japan Society for Clinical Immunology