This post is an answer to the Case – Periorbital Purpura
Approximately 3 years earlier, the patient had received a diagnosis of multiple myeloma and acquired monoclonal immunoglobulin light-chain amyloidosis, for which she had not required any treatment.
Therapy with cyclophosphamide and dexamethasone was initiated. The skin lesions waxed and waned repeatedly, with no apparent response to therapy. The patient died from pneumonia 6 months after the lesions first appeared.
Amyloid purpura appears in a minority of patients with amyloidosis. The purpura typically occurs above the nipple line and is often seen in the webbing of the neck, the face, and the eyelids. Factor X deficiency, resulting from the binding of factor X to amyloid fibrils, is thought to be one cause of the bleeding diathesis that may occur in patients with amyloidosis.
