case_images: PMC11439687_01
case_images: Mechanisms underlying the development of hyperpigmentation in primary adrenal insufficiency. Created with .
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case_images: PMC11439687_01_fendo-15-1411774-g001.jpg
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case_images: Thus, PAI patients present increased levels of a-MSH, which acts on melanocytes favoring the production of eumelanin with a consequent increase in skin pigmentation, which is more evident in areas exposed to the sun (face, neck, back of the hand, and knuckles), normally hyperpigmented (nipple, scrotum, and labia), or subjected to friction and microtrauma ( Figure 1 ).
case_images: The diagnostic approach in autoimmune and non-autoimmune primary adrenal insufficiency. 21-OH, 21-hydroxylase; autoabs, autoantibodies; X-ALD, X-linked adrenoleukodystrophy; AAD, autoimmune Addison's disease; CMC, chronic mucocutaneous candidiasis; CH, chronic hypoparathyroidism; IFN, interferon.
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case_images: PMC11439687_01_fendo-15-1411774-g002.jpg
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case_images: A stepwise diagnostic approach guided by age, the clinical presentation, and associated diseases is recommended ( Figure 2 ). For example, the association of AAD with autoimmune thyroiditis or type 1 diabetes is suggestive of APS-2, whereas the association with chronic mucocutaneous candidiasis or hypoparathyroidism suggests a diagnosis of APS-1, which needs be confirmed by an analysis of the AIRE gene ( Figure 2 ).