Cushingoid Facies
Specialty: Endocrine and metabolic.
Why it occurs
- Prolonged exogenous administration of systemic glucocorticoids (iatrogenic cause of Cushing's syndrome due to suppression of the adrenal axis)
- Cushing's disease (ACTH-producing pituitary micro or macroadenoma that bilaterally stimulates the adrenal cortex)
- Primary adrenal adenoma or carcinoma (autonomous secretion of cortisol independent of ACTH)
- Ectopic ACTH syndrome (paraneoplastic production of ACTH by tumors such as small cell lung carcinoma or bronchial carcinoid tumor)
Initial workup
24-hour urinary free cortisol (at least two basal determinations to demonstrate hypersecretion); nocturnal suppression test with 1 mg of dexamethasone orally at 11:00 p.m. measuring plasma cortisol at 8:00 a.m. the next day (cut-off values greater than 1.8 mcg/dL confirm the absence of negative feedback); nocturnal salivary cortisol measured at 11:00 p.m.; determination of plasma ACTH to differentiate dependent or independent origin; and subsequent sellar MRI or tomography of the adrenal glands.
red flags
Rapid establishment of facial roundness associated with profound and disabling proximal muscle weakness, severe arterial hypertension that is difficult to control refractory to three classes of antihypertensive drugs, refractory hypokalemia of unexplained origin, systemic opportunistic fungal infections or other signs of severe immunosuppression, or acute psychosis induced by glucocorticoids.
Standard management
- Ketoconazole — imidazole antifungal used at doses of 400-1200 mg/day for its potent capacity for reversible inhibition of adrenal cytochrome P450 enzymes, blocking the synthesis of cortisol
- Metyrapone — inhibitor of the adrenal 11-beta-hydroxylase enzyme, useful for the acute and rapid control of severe hypercortisolism
- Mifepristone — competitive antagonist of the type II glucocorticoid receptor, indicated to treat secondary hyperglycemia in patients with endogenous Cushing's
- Cabergoline — dopamine agonist that acts on the D2 receptors of pituitary tumor cells to inhibit ACTH secretion in selected cases
Educational guidance for study. It is NOT a prescription recommendation. The actual choice depends on the cause, the patient, and current guidelines.
- Area
- Endocrine and metabolic
- Listed causes
- 4
- Treatment options
- 4