Clue in the tongue.

A 67-year-old male with recently diagnosed amyloidosis presented to our hospital with acute bilateral lower extremity deep vein thrombosis. He was diagnosed with amyloid light chain amyloidosis 3 months prior to presentation and had been treated with one cycle of cyclophosphamide–bortezomib–dexamethasone. On physical examination, he was noted to have mildly enlarged tongue with lateral scalloping (Fig. 1). He did not have any difficulty with speech or eating.

Fig. 1

Photomicrograph showing mildly enlarged tongue and scalloping at the sides.

Macroglossia is an abnormal enlargement of the tongue in comparison to the mouth and jaws. It commonly leads to a scalloped appearance on the lateral margins of the tongue caused by the enlarged tongue pushing against the teeth (1). Macroglossia and tongue scalloping is a non-specific sign which may be associated with a variety of infiltrative or inflammatory conditions like Down syndrome, hypothyroidism, tuberculosis, sarcoidosis, amyloidosis, multiple myeloma, neurofibromatosis, infection (e.g., syphilis), and angioedema (1). However, it is important to remember that tongue scalloping without macroglossia can be seen with obstructive sleep apnea and is a useful clinical predictor of sleep pathology (2).

Macroglossia with scalloping is seen in about 15% of cases of amyloid light chain amyloidosis (3). It may be a part of generalized amyloidosis or a localized oral amyloid deposition (4). Macroglossia usually resolves with the treatment of underlying amyloidosis, with rare cases with significant obstruction needing surgery (4).

In conclusion, it is important to consider macroglossia as a sign of some underlying disorder like amyloidosis. Focused diagnostic testing, including possible biopsy, should be considered (5).