Summary

Primary cutaneous amyloidosis is a rare disease, characterized by the deposition of amyloid in the dermis, in the absence of systemic involvement. It has been linked especially to autoimmune diseases, such as systemic lupus erythematosus, systemic sclerosis, rheumatoid arthritis, primitive biliary cholangitis. We present a case of a patient with primitive biliary cholangitis who developed lichen amyloidosis. This association has been rarely reported in the literature.