A 64 y.o. male comes to the ED 5 days after being released from prison with tongue lesions.

what do you think is going on?

he has a large ulcer on the L side of the tongue and nodules anteriorly. The whole tongue is hard

Our patient had AL amyloidosis ( amyloidosis caused by overproduction of immunoglobulins).   His tongue was infiltrated with amyloid because he had unrecognized multiple myeloma with lytic lesions thoughout the body.  Because the tongue was so large he developed ulcers from where his teeth caused abrasions.   The term amyloid was first used in 1853 by Virchow who observed the close similarity to starch after dyeing these deposits in iodine.

Macroglossiarefers to painless enlargement of the tongue.It occurs when the resting tongue protrudes beyond the alveolar ridge.There are multiple causes of macroglossia which can be either relative macroglossia or true macroglossia.Relative macroglossiacan be caused by Down’s syndrome or hypothyroidism where the tongue can appear enlarged due to hypotonia or Pierre Robin syndrome

Macroglossia on imaging.

 CAUSES OF TRUE MACROGLOSSIA

Infiltative disease- Amyloid(AL, primary amyloid caused by a malignancy or AA amyloid caused by chronic  inflammation) , mucopolysaccharodoses, polymyositis, sarcoidosis

Malignancy- squamous cell, lymphoma, eocinophilic granuloma

Structural abnormalities-lymphangioma, hemangioma

Medications –ritinovir

Flexion of the neck for a prolonged period as in posterior cranial fossa surgery- the compression of arteries and veins during surgery is felt to lead to  swelling of the tongue and may cause airway obstruction in 1% of cases.

Infectious causes- syphilis, tuberculosis

Endocrine- acromegaly  can contribute to enlargement of the lingual tonsil which contribute to the volume of the tongue.

pseudomacroglossia can occur with vitamin deficiencies

Amyloidosis can be caused by neoplasms, infectious disease and non infectious disease.   Excess protein from malignant overproduction occurs in multiple myeloma.  Amyloid can also occur in chronic inflammation.  In diseases like rheumatoid arthritis, joint synovitis leads to serum amyloid A protein is synthesize in the liver as a result of proinflammatory cytokines. In most of the world however,  chronic infection is the cause of amyloidosis. Tuberculosis, chronic osteromyelitis, leprosy,bronchitis, and chronic pyelonephritishave all been implemented in amyloidosis. 

Often amyloidosis presents as proteinuria and if suspicion is high: abdominal fat aspiration can confirm the diagnosis.

Our patient was admitted and transferred to heme-onc.  He elected to go home on hospice without treatment.

Bergesio F, Ciciani Am, Santostefano M, et al. Immunopatology Group, Italian Society of Nephrology renal involvement in systemic anyloidosi-an Italian retrospective study on epidemiological and clinical data at diagnosis. Nephrol Dial Transplant 2007;22(6):41-45.

 Sridharan K, Rokkam V,  Macroglossia, StatPearls.  Aug 11,21

Vriitsali E, Kolokotronis A, Valagouti D, et al. Acquired macroglossia due to lopinavir/ritinovir treatment. J Oral Pathol Med 2005 Juan;34(1_:56-8.

Ardran G, Kemp F.  The tongue and mouth in acromegaly Clinical Rodiology 1971 Volume 23, issue 4: 434-444.

Real de Asua D, Costa R, Galvan J, et al. Systemic AA amyloisosis: epidemiology, diagnosis, and management. Clin Epidemiol 2014, 6:369-377.