Photo Quiz

Hyperpigmentation of the Tongue and Buccal Mucosa


Am Fam Physician. 2020 Aug 1;102(3):181-182.

A 45-year-old woman with a 10-year history of primary adrenal insufficiency was admitted to the hospital for treatment of pyelonephritis caused by Escherichia coli infection, for which she was given intravenous antibiotics and fluids.

On physical examination, her heart rate was 120 beats per minute, and her blood pressure was 90/58 mm Hg. Oral examination revealed hyperpigmentation of her tongue, the vermilion border of her lips, and the buccal mucosa (Figure 1).




Based on the patient's clinical findings and appropriate treatment of the related underlying diagnosis, which one of the following steps is correct?

A. Check adherence to glucocorticoid therapy.

B. Increase the dose of glucocorticoid therapy.

C. Increase the dose of fludrocortisone.

D. Observe.


The correct answer is A: check adherence to glucocorticoid therapy. Hyperpigmentation is a characteristic clinical finding of primary adrenal insufficiency. Successful treatment of adrenal insufficiency depends on taking the correct dosage of the glucocorticoid for the prescribed period.

Hyperpigmentation is caused by an increase in adrenocorticotropic hormone, which acts as an agonist of the melanocortin-1 receptor. This receptor is highly expressed on the surface of melanocytes.1 The hyperpigmentation is most prominent at flexures, sites of pressure and friction, palmar and plantar creases, and sun-exposed areas. However, it can also affect scars and buccal, vaginal, and anal mucous membranes.

Primary adrenal insufficiency hyperpigmentation usually resolves when the excess secretion of adrenocorticotropic hormone is blocked by replacement doses of glucocorticoid. Persistence of hyperpigmentation in a patient

Author disclosure: No relevant financial affiliations.

Address correspondence to H.U. Rehman, MBBS, at Reprints are not available from the authors.


show all references

1. Charmandari E, Nicolaides NC, Chrousos GP. Adrenal insufficiency. Lancet. 2014;383(9935):2152–2167....

2. Murray RD, Ekman B, Uddin S, et al.; EU-AIR Investigators. Management of glucocorticoid replacement in adrenal insufficiency shows notable heterogeneity. Clin Endocrinol (Oxf). 2017;86(3):340–346.

3. Bergthorsdottir R, Leonsson-Zachrisson M, Odén A, et al. Premature mortality in patients with Addison's disease: a population-based study. J Clin Endocrinol Metab. 2006;91(12):4849–4853.

4. Bensing S, Brandt L, Tabaroj F, et al. Increased death risk and altered cancer incidence pattern in patients with isolated or combined autoimmune primary adrenocortical insufficiency. Clin Endocrinol (Oxf). 2008;69(5):697–704.

5. Oprea A, Bonnet NCG, Pollé O, et al. Novel insights into glucocorticoid replacement therapy for pediatric and adult adrenal insufficiency. Ther Adv Endocrinol Metab. 2019;10:2042018818821294.

This series is coordinated by John E. Delzell Jr., MD, MSPH, associate medical editor.

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