Endocrine Abstracts

Introduction: Cushing’s syndrome (CS) is typically characterized by faciotronic obesity with signs of protein hypercatabolism, which may be iatrogenic or endogenous (adrenal or ATCH dependent). We report a case of CS of unusual presentation.

Case Report: We report the case of a 45-year-old patient, referred for etiological assessment and management of an atypical cushing syndrome.– Symptomatology dates back to 2007, by the appearance of abdominal pain with vomiting and asthenia treated as acute adrenal insufficiency.– The exploration was then completed by the realization of a biological assessment of the adrenal cortex (17 total urinary cetosteroids =3.95 mg/24 h (normal between 4.9 and 14)), and a radiological exploration made of thoraco-abdominal CT scan and pituitary MRI: without abnormalities.– In addition, the patient reports the appearance since 2007 of facial erythrosis, skin fragility, with progressive weight gain, with stretch marks and asthenia, evolving intermittently, this symptomatology complicated 3 months ago by the worsening of the intensity, significant asthenia, muscle weakness, myalgias, muscle cramps, spinal bone pain, hair loss, with erythematosquamous lesions and ecchymosis more evident at the level of the MI, no melanoderma, no acne, no seborrhea.– Urinary free cortisol was 50.7 µg/24 h, with an 8-hour cortisol level of 6.4 µg/dl.– No other signs of pituitary insufficiency or hypersecression werw reported.– For confirmation of cushing’s syndrome: Braking not done (because of IS), CLU not done (patient under hydrocortisone), cortisol cycle was performed (no disruption of the circadian cycle).– Etiological workup: Biological: hypophysogram:ACTH=2.96, hypogonadotropic hypogonadism. Radiological: adrenal CT scan(no abnormalities), octreoscan (no abnormal fixation in favor of ectopic cushing), brain MRI (arachnoidocele pushing back the pituitary gland).

Discussion: Diagnostic hypotheses: This is most likely a cushing’s disease with an apoplectic pituitary adenoma responsible for arachnoidocele, in view of the progressive onset of intermittent cushing’s syndrome, hypogonadism of central appearance, and the negativity of the rest of the workup although the ACTH is low that can be explained by braking of the corticotropic axis by CTC in the long term (equivalent of 7.5 mg of prednisone for 12 years).

Conclusion: This case illustrates an atypical and confusing presentation of CS highlighting the clinical polymorphism of this disease and the resulting diagnostic difficulties.