Nodular primary localized cutaneous amyloidosis: presentation of 2 case reports and literature review

Abstract

The nodular primary localized cutaneous amyloidosis is rare and characterized by skin deposition of amyloid substance. There are cases of progression to invasive disease. The type of amyloid substance is not specific to this disease, also being found in primary systemic amyloidosis or systemic amyloidosis associated with multiple myeloma. The authors describe two cases of nodular primary localized cutaneous amyloidosis. A 70-year-old man underwent excision of papule on the mouth left commissure, and a 39-year-old woman with a vulva's labia majora swelling that was excised, in which the histology revealed a nodular amyloidosis, both without systemic involvement. Although the prognosis is benign, the follow-up of the patient is required for early detection of a possible evolution to systemic disease, or exclusion of an underlying systemic amyloidosis.

Primary localized cutaneous nodular amyloidosis presenting as lymphatic malformation: A case report

“…PLCNA mainly occurs between the ages of 40 and 60, with the same proportion in men and women [ 11 ]. It is characterized by focal cutaneous predominant accumulation of light-chain immunoglobulins, secondary to a localized monoclonal proliferation of the plasma cells [ 12 ,13, 14 ]. In this case, amyloid originated from the immunoglobulin light chain and was associated with dermal plasma cell infiltration, which may penetrate the subcutaneous tissues and blood vessels and might develop into a systemic disease.…”

Primary cutaneous nodular amyloidosis: A rare disease

“…The amyloid protein in the nodular form is of AL type, which consists of monoclonal immunoglobulin light chain. [ 1 2 ] It is thought that AL amyloid in the cutaneous nodular form is produced by local plasma cells infiltrating the dermis. Some authors have reported a 7–50% risk of progression from localized nodular cutaneous amyloidosis to systemic forms on long-term follow-up.…”