title: [Two cases of limited cutaneous nodular amyloidosis with primary Sjögren's syndrome].
abstract: 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.
authors.affiliation: Yokohama City University School of Medicine, First Department of Internal Medicine.
authors.forename: Akiko
authors.initials: A
authors.lastname: Aoki
authors.forename: Shigeru
authors.initials: S
authors.lastname: Ono
authors.forename: Atsuhisa
authors.initials: A
authors.lastname: Ueda
authors.forename: Eri
authors.initials: E
authors.lastname: Hagiwara
authors.forename: Tsuji
authors.initials: T
authors.lastname: Takashi
authors.forename: Haruko
authors.initials: H
authors.lastname: Ideguchi
authors.forename: Midori
authors.initials: M
authors.lastname: Misumi
authors.forename: Tetsuo
authors.initials: T
authors.lastname: Sasaki
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Ikezawa
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Inayama
authors.forename: Yuko
authors.initials: Y
authors.lastname: Inoue
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Ishigatsubo
mesh_terms: D000368:Aged; D000682:Amyloid / Q000378:metabolism; D000686:Amyloidosis / Q000209:etiology*; D005260:Female; D006801:Humans; D008875:Middle Aged; D012859:Sjogren's Syndrome / Q000150:complications*; D012875:Skin Diseases, Metabolic / Q000209:etiology* / Q000378:metabolism
chemical_list: D000682:Amyloid; C035222:amyloid protein AL