Macroglossia and periorbital ecchymosis

Published:February 21, 2016DOI:
      A 70-year-old woman with past medical history of idiopathic adult hydrocephalus syndrome was admitted to our center due to malaise of one year of evolution without any other symptomatology. Macroglossia with indentation, spontaneous periorbital purpura and cervical-thoracic ecchymotic cutaneous lesions were notable at physical examination. (Fig. 1, Fig. 2, Fig. 3).
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      Fig. 1Shows macroglossia with indentation due to amyloid depositions.
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      Fig. 2Shows peri-orbital haemorrhage (raccoon eyes), one of the classic signs of AL amyloidosis.
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      Fig. 3Shows cervical-thoracic ecchymotic cutaneous lesions due to amyloid infiltration of vessel wall causing capillary wall fragility.
      Hemogram and urine analysis were within normal limits. M protein (IgG) in serum was identified as well free light chain Kappa and Lambda. A lytic lesion was observed in a cranial radiography (Fig. 4).
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      Fig. 4Shows a lytic lesion in cranial radiography which is typical of myeloma.
      What is the diagnosis?
      Due to M protein, mucocutaneous and radiographic findings, AL amyloidosis associated with multiple myeloma was suspected. Fibrillar amyloid and bone marrow clonal plasmocytosis (15%) were demonstrated confirming diagnosis.
      Amyloidosis coexists with multiple myeloma in approximately 10% of cases. Mucocutaneous involvement can be the first manifestation of amyloidosis. Macroglossia can occur in almost 20% of cases [
      • Hazenberg B.P.
      Amyloidosis: a clinical overview.
      ]. Amyloid purpura appears in a minority of patients with amyloidosis. The purpura typically occurs above the nipple line and is often seen in the webbing of the neck, the face, and the eyelids. Raccoon eyes are present in only a minority of patients, but are highly characteristic of AL amyloidosis [
      • Eder L.
      • Bitterman H.
      Amyloid purpura.
      Treatment has been delayed awaiting surgery due the idiopathic adult hydrocephalus syndrome, given that treatment involves high risk of neutropenial infections and increased bleeding from thrombocytopenia.

      Conflict of interest

      The authors state that they have no conflicts of interest.


        • Hazenberg B.P.
        Amyloidosis: a clinical overview.
        Rheum Dis Clin North Am. 2013; 39: 323-345
        • Eder L.
        • Bitterman H.
        Amyloid purpura.
        N Engl J Med. 2007; 356: 2406