Systemic amyloidosis and the skin: a review with special emphasis on clinical features and therapy

This report is a review of the literature on systemic amyloidosis and concentrates largely on primary and plasma cell dyscrasia-related disease. Recent developments in the fields of amyloid structure and pathogenesis arc discussed and patterns of clinical presentation are described. It is emphasized that a combination of the carpal tunnel syndrome, macroglossia and specific mucocutaneous lesions necessitates an intensive search for an underlying plasma cell dyscrasia, and that immunodectrophoresis of serum and concentrated urine is essential to exclude a paraproteinaemia. Other diagnostic measures, including cutaneous histopathology, are considered. Despite the advances in our understanding of the pathogenesis of amyloid, there has been little progress in terms of the success of therapy, and the ultimate prognosis of patients with systemic amyloidosis remains poor.