Endocrine Abstracts

Introduction: Intermittent Cushing’s syndrome is characterized by alternating episodes of hypercorticism with periods of eucorticism. It is a rare but well-defined entity and should be known. It remains a diagnostic challenge sometimes particularly difficult in endocrinology, we report a case.

Case presentation: A 22-year-old diabetic patient on metformin, who consulted us because of a weight gain with the appearance of stretch marks for 2 months. The clinical examination revealed severe obesity with TTP, aconthosis nigricans, complete and flagrant clinical cushing syndrome. At this time, we could not explore the patient hormonally. The hypothalamic-pituitary MRI showed a pituitary micro-adenoma measuring 3.3*2.4mm. The workup for cushing’s syndrome was unremarkable except for diabetes with a HBa1C of 7%. After 5 months, the patient had regression of stretch marks with a significant weight loss of 9kg, and hypoglycemic malaise motivating the discontinuation of diabetes treatment. The diagnostic workup for cushing’s syndrome, including urine cortisol levels and a 1mg overnight dexamethasone suppression test, was normal. The diagnosis of cushing’s disease with cyclic hypercorticism was considered. The management consisted of a quarterly clinical and biological evaluation

Discussion: The diagnostic criteria for intermittent cushing’s syndrome include three episodes of hypercorticism and two episodes of eucorticism, although these criteria are valid for most patients, they can be difficult to achieve especially if the intercycle phase is large. Our patient presented with a clinically diagnosed episode of hypercorticism followed by an episode of spontaneous eucorticism. The fluctuating clinical presentation and discordant biochemical findings make this intermittent Cushing syndrome extremely difficult to diagnosis. Frequent measurements of urinary cortisol or better salivary cortisol level are reliable and practical diagnostic tools, and should be repeated, especially when clinical signs and symptoms reappear.

Conclusion: This observation illustrates the diagnostic challenge of intermittent Cushing’s syndrome, so clinicians should be aware that hypercorticism may occur periodically and actively search for it in all patients with suspected Cushing’s syndrome.