Photo Quiz

Hyperpigmentation of the Tongue and Systemic Symptoms

 

Am Fam Physician. 2020 Apr 15;101(8):499-501.

A 25-year-old woman presented with a one-month history of rapid heartbeat, lightheadedness, feeling faint with standing, and nausea. She had lost 13 lb (6 kg). She also reported darkening of her skin, tongue, and lips over the previous two weeks. The patient had a history of Hashimoto thyroiditis, which was diagnosed at 12 years of age. The patient was not taking any medications.

On physical examination, her systolic blood pressure was 100 mm Hg, and she was mildly tachycardic. There were multiple small, dark macules on her tongue and hyperpigmentation on her lip mucosa and the dorsal surfaces of the distal and proximal interphalangeal joints on both hands (Figure 1 and Figure 2).

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FIGURE 1


FIGURE 1

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FIGURE 2


FIGURE 2

Question

Based on the patient's history and physical examination findings, which one of the following is the most likely diagnosis?

A. Drug-induced hyperpigmentation.

B. Lingua villosa nigra (black hairy tongue).

C. Peutz-Jeghers syndrome.

D. Pigmented fungiform papillae of the tongue.

E. Primary adrenal insufficiency (Addison disease).

Discussion

The answer is E: primary adrenal insufficiency (Addison disease). Hyperpigmentation is present in almost all patients with primary adrenal insufficiency. The patchy hyperpigmentation is generalized but most often notable in areas exposed to light, chronic friction, or pressure.1 In this patient, a history of an autoimmune endocrine condition and the fatigue, hyperpigmentation, and vital signs prompted concern for adrenal insufficiency. An early morning (4 to 8 a.m.) serum cortisol measurement was 0.6 mcg per dL (16 nmol per L), which is significantly lower than the normal range of less than 10 to 20 mcg per dL (276 to 552 nmol per L). An adrenocorticotropic hormone stimulation test was performed, confirming the diagnosis of Addison disease.

Cortisol deficiency causes an increased production of pro-opiomelanocortin, a prohormone that is cleaved into

Address correspondence to Olivia M. Seecof, MD, at olivia.seecof@jefferson.edu. Reprints are not available from the authors.

Author disclosure: No relevant financial affiliations.

References

show all references

1. Irvine WJ, Barnes EW. Adrenocortical insufficiency. Clin Endocrinol Metab. 1972;1(2):549–594....

2. Dunlop D. Eighty-six cases of Addison's Disease. Br Med J. 1963;2(5,362):887–891.

3. Thompson DF, Kessler TL. Drug-induced black hairy tongue. Pharmacotherapy. 2010;30(6):585–593.

4. Mangold AR, Torgerson RR, Rogers RS III. Diseases of the tongue. Clin Dermatol. 2016;34(4):458–469.

5. Latchford A, Cohen S, Auth M, et al. Management of Peutz-Jeghers syndrome in children and adolescents: a position paper from the ESPGHAN Polyposis Working Group. J Pediatr Gastroenterol Nutr. 2019;68(3):442–452.

6. Holzwanger JM, Rudolph RI, Heaton CL. Pigmented fungiform papillae of the tongue: a common variant of oral pigmentation. Int J Dermatol. 1974;13(6):403–408.

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

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