A Young Boy with Generalized Hyperpigmentation

Abstract

Addison’s disease is rare endocrinal disease of adrenal insufficiency. The most common cause is autoimmune destruction of afrenal glands, but in developing countries adrenal tuberculosis is the leading cause of Addison’s disease. Cutaneous and mucosal hyperpigmentation is common in Addison’s disease and can present early in the disease process. Morning (8 a.m.) serum cortisol level below 83 nmol/L confirms the diagnosis of Addison’s disease. Other key findings are hyponatremia, low chloride and hyperkalemia due to low/absent of aldosterone. The mainstay of the treatment is adequate replacement of corticosteroids with/without mineralocorticoids. Adrenal crisis is a life threatening complication and needs to be suspected early and treated aggressively with intravenous hydrocortisone and supportive measures.