Abstract:Amyloidosis is characterized by the deposition of a group of unrelated proteins leading to changes in tissue architecture and function. The nodular variant is the rarest form of the cutaneous amyloidoses. We report a patient with localized nodular amyloidosis without systemic amyloid involvement or paraproteinaemia after 6 years of follow-up. The unusual aspects of our case were a plaque presentation rather than nodular, and the disseminated pattern observed.
“…Three other patients had "multiple skin lesions" without a clear description of the locations [1]. There are three case reports, which describe disseminated lesions or new lesions spreading to non-contiguous locations over two to 6 years without monoclonal proteins [6,7,8 ].…”
“…Three other patients had "multiple skin lesions" without a clear description of the locations [1]. There are three case reports, which describe disseminated lesions or new lesions spreading to non-contiguous locations over two to 6 years without monoclonal proteins [6,7,8 ].…”
“…4,7 On histopathological examination, the amyloid material is found within the superficial papillary dermis. 2,4,8,9 Rarely, it may be atrophic or bullous in appearance. Nodular (tumefactive) amyloidosis may be localized or systemic and is derived from immunoglobulin light chains (AL).…”
Primary, localized cutaneous amyloidosis includes macular, lichen, and nodular (tumefactive) types in which the amyloid deposits are limited to the dermis without systemic involvement. The material in lichen and macular amyloidosis is derived from epidermal keratinocytes [keratinocyte-derived amyloid (AK)], whereas that in nodular amyloidosis is derived from immunoglobulin light-chains amyloid (AL). Primary, localized cutaneous nodular amyloidosis (PLCNA) is a form of primary, localized cutaneous amyloidosis that has been associated with a risk of progression to systemic amyloidosis. We report an unusual case of nodular AK-type amyloid deposited in the dermis of the feet. The patient is a 60-year-old woman with asymptomatic verrucoid-like lesions present around the medial and lateral aspects of the bilateral heels for 1-2 years. A biopsy showed massive deposition of eosinophilic amorphous material in the papillary and reticular dermis. The material stained positive for Congo red with apple-green birefringence on polarized light. It was also positive for pan-cytokeratin and negative for kappa and lambda light-chain immunostains. An extensive workup was negative for systemic involvement. Lipid chromatography tandem mass spectrometry confirmed that the deposition was AK-type amyloid. We believe that this is the first case of PLCNA with AK deposition. This entity should be included in the differential diagnosis of PLCNA so that an extensive systemic workup may be avoided.
“…Single or multiple nodules are the usual lesions seen, and large plaques as in this case, are exceptional. 2 Initial reports suggested that there was a 50% progression of NTA to systemic disease, 3 whereas recent studies were more encouraging, with only 7% progression on prolonged follow-up. 4 However, one case in this latter series showed systemic progression only after 20 years of surveillance.…”
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