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Generalized pigmentation due to Addison disease

  • Author(s): Kumar, Rajesh
  • Kumari, Sita
  • Ranabijuli, Pradeep Kumar
  • et al.
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Generalized pigmentation due to Addison disease
Rajesh Kumar, Sita Kumari, Pradeep Kumar Ranabijuli
Dermatology Online Journal 14 (2): 13

Jagjivan Ram Hospital, Mumbai Central, Mumbai, India. rkderm@gmail.com

Abstract

A 10-year-old boy presented with generalized hyperpigmentation, abdominal pain, change in behavior and, no weight gain for 2 years. On examination, he had hypotension and generalized hyperpigmentation that was more marked on gingival mucosa and frictional sites. Serum cortisol was markedly decreased and his CT scan abdomen showed adrenal atrophy. On the basis of clinical and laboratory findings, a diagnosis of Addison disease was made.


Primary adrenal insufficiency or Addison disease is a rare endocrine disorder characterized by generalized hyperpigmentation, fatigue, anorexia, weight loss, muscle weakness, hypotension, nausea, vomiting, and diffuse abdominal pain [1]. Although autoimmune adrenalitis is the most common etiology of Addison disease in developed countries, in our region tuberculosis is the most common cause [2, 3]. We report a case of primary adrenal insufficiency or Addison disease that we attribute to tuberculosis.


Clinical synopsis


Figure 1Figure 2
Figure 1. Gingival pigmentation
Figure 2. Pigmentation of feet at friction sites

Figure 3
CT scan showing bilateral adrenal atrophy

A 10 year-old-boy presented with progressive generalized pigmentation of the skin and failure to gain weight for 2 years. He also had behavioral changes and abdominal pain for 15 days. His history revealed that he had received anti-tubercular treatment for tubercular cervical lymphadenitis for 9 months at the age of 5 years. He appeared anxious and restless. His blood pressure was 100/60 mm. There was generalized hyperpigmentation of skin and mucous membranes; it was most prominent over the hands, feet, and gingival mucosa (Fig. 1). The pigmentation was more marked at sites of friction (Fig. 2). Hair and nails were normal. Systemic examination was normal. Serum cortisol level was markedly reduced (0.96 mg / dl, with a normal range: 9 -23 mg/dl). A CT scan of the abdomen showed bilateral moderate to severe adrenal gland atrophy (Fig 3). Serum electrolytes and thyroid function tests were within normal limit. Unfortunately, we could not perform serum ACTH estimation. On the basis of clinical findings and investigations a final diagnosis of Addison disease was made. Past history of cervical tubercular lymphadenitis suggests, tubercular infection of the adrenals could be the possible cause of adrenal gland atrophy in this case. The child was treated with tablet hydrocortisone and fludrocortisones and he showed significant improvement in clinical symptoms after 4 weeks.


Discussion

Primary adrenal insufficiency or Addison disease is a rare endocrine disorder first described by Thomas Addison in 1855. It has a prevalence of 1 in 100,000 with no particular sex predilection [1]. In contrast to developed countries where it most commonly results from autoimmune adrenalitis, in our region tuberculosis plays an important role in its etiology [2]. It is characterized by gradual onset of fatigue, anorexia, weight loss, muscle weakness, and hypotension. Nausea, vomiting, and diffuse abdominal pain when present are signs of impending addisonian crisis. Generalized hyperpigmentation is a hallmark and most important differentiating feature of primary adrenal insufficiency from secondary adrenal insufficiency. The hyperpigmentation is attributed to excessive secretions of pituitary hormones MSH (melanocyte stimulating hormones) and ACTH (adrenocorticotrophic stimulating hormones). The pigmentation is gradual in onset and often recognized by others. It is more marked in areas subject to friction such as the elbows, knees, and waistline. It is also more pronounced in sun exposed areas such as the face and dorsa of hands. Recently acquired scars during adrenal insufficiency may get hyperpigmented. The pigmentation may involve mucous membranes, tongue, and gums. The hair becomes more darkened. Nails may show melanonychia (longitudinal hyperpigmented bands). Similar pigmentation may be seen in Nelson syndrome (pituitary adenomas from bilateral adrenalectomy for Cushing syndrome) and in tumors causing ectopic ACTH secretion. These adenomas secrete high level of ACTH and MSH. It is often difficult to prove tuberculosis as a cause of adrenal insufficiency [3]. In a retrospective study, Lam and Lo were able to find 6.6 percent cases of tuberculosis as a cause after investigation whereas in 70 percent of cases it was confirmed after autopsy [4]. In India tuberculosis is an important cause of adrenal insufficiency [2].

References

1. Feldman SR,Jorizzo JL.Adrenal, androgen related, and pituitary disorders. In: Callen JP, Jorizzo JL eds. Dermatological signs of internal disease, 3rd edn. Philadelphia: Saunders, 2003:187-91

2. Agrawal G, Bhatia E, Pandey R,Jain SK. Clinical profile and prognosis of Addison's disease in India. Nat Med J India 2001; 14: 23-5

3. Haddara WM, van Uum SH.TB and adrenal insufficiency. CMAJ 2004; 171: 710

4. Lam KY, Lo CY. A critical examination of adrenal tuberculosis and a 28-year autopsy experience of active tuberculosis. Clin Endocrinol (Oxf.) 2001;54(5):633-9.

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