Tongue Indentations Secondary to Light Chain Amyloidosis

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A 65-year-old-woman presented with tongue pain and difficulty chewing and swallowing that started 4 months ago. She also reported asthenia and weight loss of 6 lbs as well as paresthesia of hands and feet. Physical examination revealed severe macroglossia with marked tongue indentations (Figs. 1 and 2), shoulder infiltration, periorbital papular lesions and signs suggestive of carpal tunnel syndrome. Laboratory examinations were remarkable for anemia and hypercalcemia. Serum monoclonal paraprotein (lambda light chain) was detected by immunoelectrophoresis. A periumbilical biopsy specimen stained with Congo-red was positive for amyloidosis and bone marrow study confirmed the diagnosis of multiple myeloma. Unfortunately, the patient died because of respiratory tract infection during treatment.
Despite light chain amyloidosis can deposit on almost any organ, tongue infiltration is still an uncommon site of presentation, as well as the “shoulder pad sign”1 and “raccoon sign”.2 Tongue indentations are a strong clue for incipient macroglossia that must alert the clinician for possible infiltration secondary light chain amyloidosis.
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