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Year : 2015  |  Volume : 1  |  Issue : 1  |  Page : 66-67

Effect of steroid replacement on hyperpigmentation in Addison's disease

Department of Endocrinology and Metabolism, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India

Date of Web Publication9-Nov-2015

Correspondence Address:
Jaya Prakash Sahoo
Room No. 557, 4th Floor, SSB Block, Jawaharlal Institute of Postgraduate Medical Education and Research Campus, Puducherry - 605 006
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Source of Support: None, Conflict of Interest: None

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How to cite this article:
Sahoo JP, Kamalanathan S, Selviambigapathy J, Vivekanandan M. Effect of steroid replacement on hyperpigmentation in Addison's disease. J Curr Res Sci Med 2015;1:66-7

How to cite this URL:
Sahoo JP, Kamalanathan S, Selviambigapathy J, Vivekanandan M. Effect of steroid replacement on hyperpigmentation in Addison's disease. J Curr Res Sci Med [serial online] 2015 [cited 2021 Jul 30];1:66-7. Available from: https://www.jcrsmed.org/text.asp?2015/1/1/66/168932

Dear Sir,

A 26-year-old female presented to emergency with recurrent vomiting and abdominal pain for 1-week. She also had fever, myalgia, loss of appetite, and loss of weight for 6 months. General physical examination revealed her thin built (body mass index – 16 kg/m 2), hypotension (blood pressure – 90/70 mmHg), and skin hyperpigmentation [Figure 1]a. Routine serum biochemistry showed glucose – 50 mg/dl (60–100), sodium – 125 meq/L (135–145), potassium – 5.5 meq/L (3.5–5), and chloride – 115 meq/L (102–110) and bicarbonate – 12 meq/L (18–23). Evaluation for pyrexia of unknown origin including work up for tuberculosis and HIV were negative. Hormonal and radiological investigations were suggestive of Addison's disease: Basal serum cortisol – 1.35 mcg/dl (normal: 5–20), plasma adrenocorticotropic hormone (ACTH) −912 pg/ml (normal: 9–52) and bilateral adrenal atrophy on contrast enhanced computed tomography of abdomen. As the basal serum cortisol was <3 mcg/dl, ACTH stimulation test was not done. She was managed with intravenous fluids (dextrose normal saline) and hydrocortisone infusion. Then, she was discharged on 5 mg prednisolone and 100 mcg fludrocortisone daily with advice regarding stress doses of steroids. Following 6 months of steroid replacement, her pigmentation has decreased to the original status [Figure 1]b.
Figure 1: Patient's photo before (a) and 6 months after (b) steroid replacement

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Chronic primary adrenal insufficiency has an incidence of 4.7–6.2/million people in white populations.[1] Low serum cortisol (<3 µg/dL) with high plasma ACTH (>100 pg/ml) level and/or hyperpigmentation of skin/mucous membranes is considered as the diagnostic criteria for Addison's disease.[1] Pro-opiomelanocortin (POMC) is the precursor molecule of ACTH and melanocyte stimulating hormone (MSH).[2] Thus, an increase in the production of ACTH in Addison's disease also results in an increased production of MSH in pituitary. Three MSHs have been described: α-MSH, γ-MSH, and β-MSH. γ-MSH and β-MSH are produced in the normal processing of the POMC molecule in pituitary. α-MSH is produced mainly by ectopic ACTH-producing tumors. These MSHs can produce an increase in skin pigmentation by stimulating melanocortin-1 receptor on melanocytes.[3],[4] As both ACTH and MSH have same precursor molecule, ACTH in high levels (as in Addison's disease) also cause hyperpigmentation of skin and mucous membranes due to enhanced stimulation of melanocytes. This manifestation may appear months before acute adrenal crisis like in our case. Steroid replacement improves hyperpigmentation by decreasing serum level of both ACTH and MSH in these patients.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/ their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Brandão Neto RA, de Carvalho JF. Diagnosis and classification of Addison's disease (autoimmune adrenalitis). Autoimmun Rev 2014;13:408-11.  Back to cited text no. 1
Peter SA, Brignol YF, Razavi MH, Greeley N. Diffuse hyperpigmentation associated with acquired immunodeficiency syndrome. J Natl Med Assoc 1992;84:977-9.  Back to cited text no. 2
Lerner AB, Shizume K, Fitzpatrick TB. On the mechanism of melanin pigmentation in endocrine disorders. J Invest Dermatol 1953;21:337-8.  Back to cited text no. 3
Oelkers W. Adrenal insufficiency. N Engl J Med 1996;335:1206-12.  Back to cited text no. 4


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