Juvenile Dermatomyositis (JDM) is a chronic inflammatory disease probably of an autoimmune nature.^1,2 Hereditary angioneurotic oedema (HANE) is an enzyme deficiency that results in the loss of inhibition of the classical complement pathway. This results in the consumption of classical pathway factors particularly C4. It is associated with some autoimmune disorders, particularly SLE. We report for the first time the occurrence of JDM in a child with HANE.

A 6 year …