Spontaneous bilateral peri-orbital purpura: an important clinical sign of primary systemic amyloidosis

A 69-year-old woman presented with an 18-month history of recurrent bruising of the eyelids. She was otherwise asymptomatic and systems review was unremarkable. On examination, she had peri-orbital purpura and waxy papules at the inner canthus of both eyes. Macroglossia was also noted. Subcutaneous abdominal biopsy identified amorphous material in the dermis that stained positive for Congo red, with apple-green birefringence seen under polarised microscopy. Immunohistochemistry demonstrated antibodies against lambda light chains. Bone marrow biopsy identified further deposits of immunoglobulin light chain amyloid and a clonal infiltrate with 10%-20%plasma cells, confirming amyloidosis secondary to multiple myeloma. Iodine-123-labelled serum amyloid protein scintigraphy showed no abnormal uptake, thereby excluding significant amyloid deposits in the liver, spleen or kidneys. Cardiac MRI was consistent with early amyloid infiltration. We highlight the importance of dermatological manifestations in amyloidosis, to allow for early diagnosis, potentially limiting end organ involvement.

Automated summary

A 69-year-old woman presented with an 18-month history of recurrent eyelid bruising, leading to a diagnosis of amyloidosis secondary to multiple myeloma. Highlighted the importance of dermatological manifestations in amyloidosis for early diagnosis and potential organ involvement limitation.