Abstract
Addisonian pigmentation is defined as hyperpigmentation of both exposed and unexposed parts of the body and it typically occurs in primary adrenal insufficiency. We hereby report a case of a 15 year old boy who presented with progressive hyperpigmentation of the face, trunk, chest, abdomen and extremities which was more accentuated over the sun exposed areas. He also had` pigmentation of nails, oral and genital mucosa, palms and soles with accentuation over palmar creases. Routine blood investigations were found to be normal. His serum electrolytes showed hyponatremia and hyperkalemia with evidence of hypocortisolemia and` increased serum ACTH. Chest x-ray was suggestive of pulmonary tuberculosis, sputum AFB was positive and Mantoux reaction was strongly positive. CT scan showed bilateral adrenal calcification. All these investigations confirmed primary adrenal insufficiency due to tuberculosis and ATT was initiated along with steroid replacement therapy. Keywords: Addisonian pigmentation, ACTH, hyperpigmentation.
Highlights
Generalised skin and mucosal hyperpigmentation is a tell-tale sign of Primary adrenal insufficiency which occurs in 95% of cases, differentiating it from secondary and tertiary hypoadrenalism
When ACTH is cleaved from the prohormone, MSH is concurrently released and it stimulates the enzyme tyrosinase in melanocytes resulting in pigmentation
Generalised hyperpigmentation is a hallmark and it is most pronounced on the sun exposed areas, areas subjected to repeated frictions, palmar and plantar creases, the normally pigmented sites, recently acquired scars and nevi, mucous membranes[3]
Summary
Generalised skin and mucosal hyperpigmentation is a tell-tale sign of Primary adrenal insufficiency which occurs in 95% of cases, differentiating it from secondary and tertiary hypoadrenalism.It often precedes other manifestations by months to years and it occurs when 90% of the adrenal gland is destroyed.[1]. Introduction Generalised skin and mucosal hyperpigmentation is a tell-tale sign of Primary adrenal insufficiency which occurs in 95% of cases, differentiating it from secondary and tertiary hypoadrenalism. It often precedes other manifestations by months to years and it occurs when 90% of the adrenal gland is destroyed.[1] Most common cause of adrenal insufficiency in developed countries is auto immune adrenalitis whereas in developing countries it is Mycobacterium tuberculosis.
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