Image IQ: Recurrent Eyelid Purpura

August 6, 2019

Eyelid bruising was observed on a 64-year-old woman during routine skin cancer follow-up. She denied trauma or pain. What does this look like to you? 

A 64-year-old woman with a history of basal cell carcinoma presents to the dermatologist for skin cancer surveillance. She is incidentally noted to have bruising on her upper eyelids (above). On questioning, she reports experiencing recurrent intermittent eyelid bruising for several years. She denies a history of trauma to her face, pain, or other associated symptoms.

Her medical history is significant for hyperlipidemia, hepatitis C (diagnosed >30 years earlier), osteoporosis, celiac disease, and idiopathic proteinuria for several years. Her surgical history includes appendectomy and carpal tunnel repair.

Pertinent family history includes her mother dying of multiple myeloma and her father of complications of diabetes.

Review of systems is significant for vague symptoms of gastrointestinal discomfort. On more directed questioning, she reports the eyelid purpura is temporally related to the Valsalva maneuver/ strained bowel movements. Her medications include atorvastatin, risedronate sodium, a daily multivitamin, calcium, vitamin D, and aspirin.

Her examination reveals no bruising elsewhere and no findings of interest on skin or mucous membranes.

The patient is showing signs of:

A. Dermatomyositis (Heliotrope rash)
11% (4 Votes)
B. Allergic contact dermatitis, purpuric type
22% (8 Votes)
C. Raccoon eyes/trauma
11% (4 Votes)
D. Primary systemic amyloidosis
54% (19 Votes)

Total Votes: 35

D. Primary systemic amyloidosis

Discussion

Primary systemic amyloidosis is a rare plasma cell dyscrasia characterized by immunoglobulin protein deposition. Skin signs of primary systemic amyloid include purpura (typically on eyelids, nasolabial folds, mouth, neck, axillae, umbilicus, and anogenital area) and macroglossia.

Flexural and facial papules, nodules, and plaques resembling xanthoma can be seen, sometimes becoming so indurated as to produce leonine facies. Carpal tunnel syndrome (CTS) often occurs in primary systemic amyloidosis-a result of compression by amyloid deposits of nerves that travel distally to the hand, causing parasthesias, peripheral neuropathy, and muscle weakness.

Primary systemic amyloidosis is more common in men and most frequently diagnosed after age 60 years. Systemic manifestation can include renal (proteinuria), cardiac (congestive heart failure) and gastrointestinal disease (disordered motility).

This patient had a history of proteinuria, recurrent eyelid purpura, and CTS that had gone unrecognized for years. She had a family history of multiple myeloma.

This patient’s workup included a negative skin biopsy. Urinalysis revealed proteinuria (1.75 g/24 hrs) and urine protein electrophoresis revealed an M-spike (.32 mg/dL) and Bence-Jones (immunoglobulin light chain) proteinuria. An abdominal fat pad biopsy detected amyloid on Congo red staining.

She underwent bone marrow transplant, and several years later, upon recurrence, underwent chemotherapy, but ultimately succumbed 8 years after diagnosis to complications from cardiac and renal infiltration.

Primary systemic amyloidosis is an uncommon disease which classically presents with recurrent purpuric skin lesions that should arouse the physicians suspicion of underlying hematologic malignancy.