A TONGUE TWISTER – MACROGLOSSIA OR ANGIOEDEMA OF THE TONGUE?

Abstract

Introduction Macroglossia is “unusual enlargement of the tongue”. It may be difficult to distinguish from angioedema affecting the tongue. Case Description A 69-year-old male was referred to the allergy-immunology clinic for suspected tongue angioedema impacting his quality of life. The patient described sudden enlargement of his tongue that began spontaneously with no waxing and waning over two months. There was no associated urticaria, lip swelling or systemic symptoms. Past medical history was notable for a heart transplant secondary to ischemic cardiomyopathy, for which the patient was taking tacrolimus. Physical examination was remarkable for a homogeneously enlarged tongue with no swelling or evidence of masses or ulcerations but loss of papillae. The fixed nature and the presence of surface changes excluded angioedema. The patient was diagnosed with acquired macroglossia. A broad differential diagnosis including hypothyroidism, amyloidosis, cheilitis granulomatosa, IgG4 disease, malignancy including Kaposi Sarcoma, and giant cell arteritis were considered. TSH, ESR and ANCA were within normal limits. A tongue biopsy was performed. Histochemistry for Congo red stain was negative, arguing against amyloidosis. Fibrosis typical of IgG4 disease was absent. Tongue biopsy revealed squamous epithelium with mild parakeratosis and ballooned cells, suggestive of oral hairy leukoplakia. Discussion This case illustrates the importance of accurately distinguishing between macroglossia as opposed to tongue angioedema as management differs greatly. Physicians should also be cognizant of the fact that immunosuppressive therapy predisposes patients to develop malignancies such as oral hairy leukoplakia. This requires appropriate monitoring and possibly reduction of immunosuppression therapy.