Case Report of a Cutaneous Manifestation of Immunoglobulin Light Chain Amyloidosis Associated with Smouldering Myeloma

Systemic immunoglobulin light-chain (AL) amyloidosis associated with multiple myeloma is rather common because they both have the same cell of origin. A monoclonal plasma cell clone produces unstable light chains which form amyloid fibrils. These fibrils are then spread through the bloodstream and deposited in the tissue of multiple organs and the skin. Especially renal and cardiac manifestations lead to a very poor outcome for the patients because of the related tissue damage. Focussing on cutaneous manifestations, there are some symptoms like bilateral periorbital haematoma (also known as "raccoon eyes"), ecchymoses and makroglossia, that can be seen as pathognomonic for a systemic AL amyloidosis. Because early diagnosis is essential for the prognosis of the affected patients these signs should lead to a fast diagnostic work-up and treatment. We present a case of a patient with cutaneous manifestations of a systemic AL Amyloidosis associated with a lambda-light-chain smouldering myeloma.