I recently saw an elderly woman in clinic. She had a background history of migraine headaches and had been investigated over the past 18 months for recurrent syncopal episodes. She had at least 5 episodes that began with palpitations usually on minimal exertion. Apart from these episodes, she was fit and active. Her ECHO showed only mild LV hypertrophy and mild pulmonary hypertension. EKGs were unrevealing.Two years ago she had been seen by a dermatologist for sun-related skin lesions and had mentioned that she had recurrent bruising around her eyelids. She had taken a photo of these lesions and they were noted to be purpuric. They would come and go and usually last for a couple of months when they were there.
She was referred to my clinic for investigation of mild albuminuria. She had ~500mg albumin for approximately 5 years with no significant change. Her renal function had always been otherwise normal apart from one single increase in creatinine was unexplained and returned to normal in a few days. She had no significant risk factors for kidney disease apart from long term NSAID use for her migraines.
As part of her investigations, she had an SPEP and free light chains. The results showed that she has an IgG lambda paraprotein likely related to an underlying multiple myeloma. Re-review of her ECHO images showed that they were consistent with infiltrative disease and she is currently undergoing work-up for AL-amyloidosis.
Periorbital purpura is a rare finding in patients with amyloidosis occurring in about 15% of patients. It is thought to be a result of Factor X deficiency due to binding of Factor X by the amyloid fibrils. It is generally atraumatic and tends to recur without treatment of the underlying amyloid. It is almost pathognomic of amyloid and its presence should prompt testing for AL amyloid.
Image from NEJM
We used to call it Post Proctoscopic Palpebral Purpura -- when proctoscopes were the thing.
ReplyDelete"almost pathognomonic" is right. I was moonlighting in an ER one night when a guy came in with P4 (see above); onset of periorbital purpura after a sigmoidoscopy. I told him that he probably had amyloidosis. He had no proteinuria. Sent him back to his primary care and of course there was no evidence of amyloid, myeloma, he had normal CBC, basically a normal healthy guy with a vigorous valsalva maneuver! Disappointing for me, good for him.
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