Catalog

Primary systemic amyloidosis affecting skin or oral cavity

No MMS code

Link to WHO Definition

Primary systemic amyloidosis affecting skin or oral cavity – http://id.who.int/icd/entity/848611604

Primary systemic amyloid, due to abnormal secretion of immunoglobulin light chain fragments as a result of plasma cell dyscrasia or multiple myeloma, may present in the skin. The commonest findings are spontaneous petechial haemorrhages, purpura and bruising. Such haemorrhage has a predilection for skin with clinically apparent amyloid infiltration: smooth, shiny, waxy, translucent papules and plaques develop, particularly on the eyelids, the neck and in flexural areas. Macroglossia occurs in 10 per cent of cases.