Primary Localized Cutaneous Nodular Amyloidosis Following Local Trauma

Abstract

Primary localized cutaneous nodular amyloidosis (nodular amyloidosis) is a rare and distinct type of amyloidosis, in which amyloid L deposition is limited to the skin and typically manifested as a tumefactive nodule on the acral sites. However, the definite cause of nodular amyloidosis is still unknown. Although it is relatively well known that the amyloid deposits in nodular amyloidosis originate from immunoglobulin light chains secreted by local plasma cells, traumatic injury to the skin has rarely been recognized as a triggering factor of nodular amyloidosis. Herein, we present a case of a 50-year-old male patient with primary localized cutaneous nodular amyloidosis, which occurred after local trauma, and discuss the relationship between traumatic damage and dermal amyloid L deposition.

Curious facial plaque diagnosed as nodular primary localised cutaneous amyloidosis

“…The causal relationship between the cat scratch and NPLCA in our patient remains unclear. While trauma-induced amyloidosis has been recognised in papular and macular amyloid, a few case reports indicate a similar association with nodular amyloidosis 9–11. In our case, lack of inflammation or infection at the time of the cat scratch makes Bartonella henselae and cowpox infections unlikely.…”

“…Quoted progression rates of NPLCA to systemic amyloidosis vary significantly. Earlier studies suggest a rate of 50%,9 while more recent studies suggest closer to 7% 3. Although there is no consensus on screening and long-term follow-up of NPLCA for systemic involvement, imaging studies including CT scanning and Serum amyloid P component (SAP) scintigraphy have been suggested.…”

Primary cutaneous nodular amyloidosis associated with psoriasis

“…Although local trauma has rarely been reported to precede development of PCNA, it is not clear if any of these few cases had coexistent psoriasis. It seems possible that foreign material persisting in the soft tissues from the original injury could act as an antigenic target for B‐cell antibody production.…”

Bilateral recurrent facial primary cutaneous nodular amyloidosis (PCNA)

“…PCNA is considered the rarest form of cutaneous amyloidosis compared with the other 2 types, macular amyloidosis and lichen amyloidosis. 2 , 3 , 4 The fibrils of amyloid in PCNA are composed of immunoglobulin light chains of either kappa and/or lambda type, called “protein AL” and do not differ from those seen in primary systemic amyloidosis and in multiple myeloma. 5 , 6 Unlike in the more common forms such as macular and lichen amyloidosis where the amyloid is formed by degenerating keratinocytes, monoclonal plasma cells in PCNA produce and secrete the immunoglobulin kappa and lambda light chains through an unknown mechanism.…”