Nodular Amyloidosis Derived From Keratinocytes

Abstract

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.

Three cases of localized cutaneous nodular amyloidosis in patients with limited systemic sclerosis and a brief literature review

“…Amyloid L is the same type of amyloid that is involved in primary systemic and multiple-myeloma-associated systemic amyloidosis (Summers and Kendrick 2008). However, there has been report of keratinocyte-derived amyloid (AK) that is implicated in LCNA (Cornejo et al 2015). Cutaneous macular and lichen amyloidoses are composed of amyloid K (Shiman et al 2010).…”

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“…1 Less commonly, it can present as nodular amyloidosis where the amyloid protein is immunoglobulin-derived from plasma cells, serum amyloidassociated protein-derived, amyloid protein P-derived or in rare case reports, also keratinocyte-derived. [1][2][3][4] The involvement of the glans penis by primary cutaneous amyloidosis is a rare condition with less than 20 reported cases in literature to date. 2,3,5 These patients typically present with an asymptomatic, single, tan/yellow, waxy nodule, or plaque on the glans.…”

Localized Cutaneous Nodular Amyloidosis: A Specific Cutaneous Manifestation of Sjögren’s Syndrome

“…Unlike the other two forms of primary localized amyloidosis, the amyloid in PLCNA is derived from immunoglobulin light chains (amyloid AL) (either κ or λ) and, thus, from plasma cells. However, an isolated case of PLCNA has been described in which the amyloid substance was found to be of the K-type rather than the AL-type [ 9 ]. Although, in systemic amyloidosis, the amyloid substance is also derived from immunoglobulin light chains, whereas in PLCNA, the plasma cells are found only in the skin and not in the bone marrow since it is not associated with an underlying hematologic dyscrasia.…”