What physical findings are common in patients with systemic AL amyloidosis?
- • Edema (most common).
- • Palpable liver (34%).
- • Macroglossia (10%–20%; pathognomonic when present).
- • Purpura (16%).
- • CTS (10%–20%): can cause claw hand due to clumping of tendons together.
- • Painful sensory polyneuropathy (10%–20%).
Edema may occur as a result of nephrotic syndrome, congestive heart failure, and rarely, protein-losing enteropathy. Hepatomegaly is usually of only a modest degree. Macroglossia and purpura should particularly raise suspicion of systemic AL amyloidosis; both may be a source of patient complaints and are easily overlooked. Increased firmness of the tongue and dental indentations are helpful in determining the presence of macroglossia. Cutaneous purpura is generally localized to the upper chest, neck, and face. Purpura of the eyelids is a clue that is seen only when the patient’s eyes are closed. Gentle pinching of the eyelids may cause bruising (pinch purpura) due to vascular fragility caused by amyloid deposition in the blood vessels. Purpura around the eyes is called the “raccoon eyes” sign. Other findings include arthropathy (“shoulder pad” sign), CTS, nail dystrophy, adenopathy, and submandibular enlargement.
