Nodular cutaneous amyloidosis involving the vulva. Case report and literature review

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).…”

“…and the plasma cells in their case were also reactive with anti-K and -X light chain antibodies, while the amyloid reacted with anti amyloid AL(x) antibodies only. Plasma cells proliferated focally are thought to secrete light chains which form amy loid fibrils [2]. X light chains in the plasma cells may have epitopes for anti-amyloid AL(X) antibodies.…”

“…The nodular or tumefacient type is rare, and 46 cases only had been reported in the world by 1985 [2], The nature and variabil ity of amyloid in systemic amyloidosis have been studied intensively, and several types of amyloid proteins have been recognized [3]. However, the origin of amyloid in primary cutaneous amyloidosis is still a matter of contro versy.…”

“…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.…”

Localized amyloidosis of the vulva with and without vulvar intraepithelial neoplasia: report of a series

“…Amyloid deposits mimicking giant condyloma have been described [10,11]. Northcutt and Vanover [12] reported a case of vulvar amyloidosis that recurred after 6 years. The lesion was ulcerated and had brisk plasma cell infiltration with numerous Russell bodies.…”