Nodular primary cutaneous amyloidosis. Isolation and characterization of amyloid fibrils

Characterization of the Amyloid Fibril from Primary Localized Cutaneous Nodular Amyloidosis Associated with Sjögren’s Syndrome

“…Various investigations for amyloidogenesis have shown that amyloid fibrils in PLCNA are also AL protein derived from immunoglobulin light chains [2,3]. Previous bio chemical studies have demonstrated that amyloid fibrils in PLCNA consisted of several types of polypeptides with apparent molecular weights of 10 or 14 kD or 17, 20 and 29 kD originating from either the X or k light chain [2][3][4]. However, the evidences that amyloid in PLCNA is not of monoclonal origin have been accumulated.…”

“…Fourteen cases of nodular cutaneous amyloidosis have been reported since 1971, and 5 out of 14 cases were asso ciated with Sjogren's syndrome [4,[10][11][12] (table 1), sug gesting a high incidence of association between primary localized amyloidosis and Sjogren's syndrome. These cases will not be secondary amyloidosis due to Sjogren's syn drome, because the amyloid deposit in the dermis showed positive reactivity with antibody for AL protein in 3 of 5 cases, unassociated with AA protein.…”

“…Nodular or tumefactive amyloidosis is a rare form of cutaneous amyloidosis, and its clinical and pathogenic fea tures are very similar to nodular lesions of systemic amyloi dosis, in which amyloid fibrils are derived from monoclo nal immunoglobulin light chains secreted by bone marrow plasma cells [1], Recent immunohistochemical studies have shown that amyloid fibrils in primary localized cutaneous nodular amyloidosis (PLCNA) also have their origin in immunoglobulin light chains [2,3], Several polypeptides with different molecular weights have been identified with biochemical analysis of amyloid fibrils extracted from some cases of PLCNA [2][3][4][5], We have isolated the amyloid fibrils biochemically from the patient skin to analyze the origin of amyloid fibrils and summarized the cases of PLCNA accompanied by Sjogren's syndrome. …”

Primary Localized Cutaneous Nodular Amyloidosis: Case Report and Biochemical Analysis of Amyloid

“…They suggested that X light chain was an inherent component of amyloid fibrils, whereas other chains and complement represented nonessential absorp tion on amyloid material, since other components except a. light chain could be washed out by pretreatment of sec tions with some detergents. Immunohistochemical stud ies on formalin-fixed biopsy material showed that amyloid deposits reacted with antisera against k light chain [2], amyloid protein A L ( k) [7] or AL(k) [4,5]. The amyloid deposits in our case retained the affinity to Congo red and green birefringence on polarization after the treatment with potassium permanganate, which clearly shows that they were not amyloid proteins A A [22], They were immunohistochemically shown to be amyloid proteins AL(k).…”

“…However, the origin of amyloid in primary cutaneous amyloidosis is still a matter of contro versy. In primary localized cutaneous nodular amyloid osis (PLCNA) few cases have been investigated immunohistochcmically [2,[4][5][6][7] and biochemically [5,8,9], which suggested that the amyloid may be of immunoglobulin light-chain origin. Wc had a case with large scalp lesions of the disease and purified and characterized amyloid fibrils.…”