POMC and primary adrenal insufficiency: The classic clinical presentation includes the following triad: progressive dysphagia of functional appearance, confirmed as achalasia by esophageal manometry; slowly progressive peripheral adrenal insufficiency (Addison’s disease), suggested by severe physical asthenia and orthostatic hypotension, confirmed by low cortisol, elevated ACTH, negative anti-21 hydroxylase antibodies, and morphologically normal adrenal glands on imaging; and alacrima, manifesting as ocular dryness that can be complicated by keratitis [10,11].