Case Report Of Amyloid Light-Chain Amyloidosis With Periocular Cutaneous Involvement

Amyloid light-chain amyloidosis is a rare disease with diverse signs and symptoms according to the affected organs. The signs are often aspecific which can lead to delayed diagnosis. Considering the characteristic cutaneous manifesta-tions of the disease, dermatologists have an important role in early identification. Additionally, histopathological ex-amination is required for diagnosis. Here we present a rare case of light-chain amyloidosis in association with multiple myeloma. The patient presented with characteristic periocular, axillar and inguinal petechiae, purpurae, ecchymoses, suffusions, yellowish-brown waxy papules and plaques besides cardiovascular and renal involvement. In this case, the amyloidogenic proteins are the lambda-chains of monoclonal immunoglobulins secreted by the clonally expanded plasma cells of the bone marrow. The applied cyclophosphamide, bortezomib and dexamethason treatment induced complete morphological remission in the bone marrow and the patient currently awaits autologous stem cell trans-plantation which yields the longest possible survival.