Alihan Oral1, Aysu Akın1, Fatih Türker1, Pelin Doğa Üstüner2, Aydın Tunçkale1

1Department of Internal Medicine, Demiroglu Bilim University, Faculty of Medicine, Istanbul, Turkey
2Department of Dermatology, Demiroglu Bilim University, Faculty of Medicine, Istanbul, Turkey

A 68-year-old man with known rheumatoid arthritis, hypertension, chronic renal disease, chronic ischemic heart disease, and chronic obstructive pulmonary disease, admitted to our clinic with shortness of breath, dizziness, and dysarthria. He was taking amlodipine 10 mg/day, furosemide 40 mg/day, prednisolone 10 mg/day, asetil-salisilik asit (ASA) 100 mg/day, pantoprazole 40 mg/day, and inhalant salmoterol 3 puff/day and fluticasone 3 puff/day. He had no known allergies. Family history of the patient was unremarkable. On examination, blood pressure was 135/78 mmHg, heart rate was 90 beats/min, and respiratory rate was 22 breaths/min. Pretibial pitting edema was observed. Diffused rhonchus was heard in lung examination. Neurological examination and cranial CT were normal. On his third day of hospitalization, the patient’s tongue was abnormally swollen (Figure 1). His macroglossia was causing speech impediment and dysphagia. Tongue examination revealed a mild nodular appearance on its surface, with obvious indentations along the bilateral margins, pink color, firmness, and hypertrophy. Swollen submental region and protrusion of the tongue were present, causing an inability to close his mouth. Magnetic resonance imaging revealed oropharyngeal narrowing caused by increased tongue volume. Tongue biopsy results were consistent with glossitis and muscular hypertrophy. The patient’s renal function gradually worsened and finally hemodialysis was commenced. The patient was administered IV methylprednisolone 40 mg and nystatin oral suspension. His complaints completely regressed. We thought that there might be an allergy to any medicine or food that we were unable to find the cause of. The patient now has complete resolution of his symptom without any recurrence after sixmonths of follow-up.

Although macroglossia is often caused by vascular malformations and muscular hypertrophy, it may also result from various congenital and acquired conditions.

When the cause is a primary disorder of the tongue tissue, it is named true macroglossia, and when it occurs secondary to another disorder, such as by amyloidosis, it is termed relative macroglossia. Macroglossia may be encountered in certain inherited or congenital disorders, including: Down syndrome and Beckwith- Wiedemann syndrome, Behmel or Laband syndromes, hemangioma, hamartomas, lingual thyroid, myxedema, mucopolysaccharidosis, and neurofibromatosis.[1] Acquired causes may include metabolic or endocrine conditions such as hypothyroidism, amyloidosis, and acromegaly; inflammatory/infectious diseases, such as pemphigus vulgaris, diphtheria, actinomycosis, candidiasis, tuberculosis, sarcoidosis; angioedema; and trauma.[2] Moreover, neoplastic disorders, such as lymphangioma or various malignancies, may also cause macroglossia.

Macroglossia is a clinical diagnosis. Therefore, clinical history, examination, and basic imaging techniques will be sufficient in identifying its etiology. Nasopharyngeal endoscopy may be necessary to assess whether the upper airway is obstructed. Genetic and chromosomal studies may be required to establish the diagnosis. Magnetic resonance imaging is the best method of choice for identifying the dimensions as well as the margins of the tumor. In certain cases, biopsy may be necessary in order to identify the cause, such as in lingual thyroid and amyloidosis.[3]

Cite this article as: Oral A, Akın A, Türker F, Doğa Üstüner P, Tunçkale A. Macroglossia and angioedema. D J Med Sci 2020;6(2):103-104.

Conflict of Interest

The authors declared no conflicts of interest with respect to the authorship and/or publication of this article.

Financial Disclosure

The authors received no financial support for the research and/or authorship of this article.

References

  1. Neville BW, Damm DD, Allen A, Chi A, editors. Oral and Maxillofacial Pathology, Amsterdam: Elsevier Health Sciences; 2015.
  2. Kovach TA, Kang DR, Triplett RG. Massive macroglossia secondary to angioedema: a review and presentation of a case. J Oral Maxillofac Surg 2015;73:905-17.
  3. Prada CE, Zarate YA, Hopkin RJ. Genetic causes of macroglossia: diagnostic approach. Pediatrics 2012;129:e431-7.