Author: Dr Ian Coulson, Consultant Dermatologist and Editor-in-Chief, 2022.
Edited by the DermNet content department
This 83-year-old lady has had recurrent attacks of bruise-like lesions on the eyelids. They are sometimes provoked by bouts of coughing.
She has been feeling tired and out of sorts recently. Routine haematology, platelet count, and clotting studies are normal. She has had numbness and tingling at night affecting the thumbs and first two fingers of both hands.
Purpura can be seen affecting the upper and lower lids of both eyes. It is quite common for lid purpura to be precipitated after bouts of coughing.
In the absence of any clotting or platelet problems, systemic amyloidosis should be considered. The presence of amyloid in the upper dermis disrupts the integrity of the support around small blood vessels making them susceptible to rupturing, and this accounts for the physical signs.
Amyloid deposits under the flexor retinaculum of the wrists has produced carpal tunnel syndrome which is quite common in systemic amyloid.
Systemic AL amyloid is usually associated with a plasma cell dyscrasia, so serum electrophoresis, estimation of immunoglobulins, and detection of Bence Jones proteins should be undertaken. Skin biopsies from affected skin may show amorphous upper dermal accumulation of amyloid protein.
