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Spontaneous bilateral peri-orbital purpura: an important clinical sign of primary systemic amyloidosis
  1. Paula Evelyn Beatty1,
  2. Lisa Killion1,
  3. Johnny Mc Hugh2 and
  4. Ann-Marie Tobin1
  1. 1 Department of Dermatology, Tallaght University Hospital, Dublin, Ireland
  2. 2 Department of Haematology, Tallaght University Hospital, Dublin, Ireland
  1. Correspondence to Dr Paula Evelyn Beatty; p.beatty0906{at}gmail.com

Abstract

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.

  • dermatology
  • haematology (incl blood transfusion)

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Footnotes

  • Contributors PEB devised the concept for the article, reviewed the literature and compiled the case report. She is the guarantor. LK reviewed and edited the content of the case report. JMH and A-MT reviewed and edited the case report.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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