A 55-year old woman was seen at the outpatient Dermatology department with orange-red, soft-elastic, asymptomatic papules on her abdomen since five years. A biopsy was positive for Congo-red staining and showed apple-green under bipolarized light. Laboratory work-up showed no systematic amyloidosis. We diagnosed it as primary nodular cutaneous amyloidosis.

Nodular cutaneous amyloidosis represents the rarest variant of primary localized cutaneous amyloidosis. The proposed management ranges from topical or systemic agents to surgical treatment. Complete surgical excision is advisable due to its potential progression to systemic amyloidosis due to dermis and subcutaneous tissue infiltration. However, in particular locations, the risk of functional complications is high, so an alternative treatment option should be considered. We report a case of a large primary nodular cutaneous amyloidosis of the leg involving the joint capsule which was successfully treated by incomplete surgical removal, without recurrences at 7-year follow-up.