Category: Pituitary Disorders/Neuroendocrinology

Monitor: 13

13 - GLUCORTICOID RESISTANCE IN A PATIENT WITH TREATMENT RESISTANT ALOPECIA AREATA: A CASE REPORT

Friday, Apr 26
12:00 PM – 12:30 PM

Objective : Alopecia areata (AA) is a common disorder thought to be the result of an autoimmune attack on hair follicles (HF). The exact pathogenesis of the disease is unknown, however, it is postulated that genetic, environmental, autoimmunity and stress factors play a role. Components of the HPA axis, such as CRH, are involved in the local stress response in the HF. Treatment typically involves topical and intralesional glucocorticoid (GC) therapy. We report a case of AA, resistant to GC treatment, with asymptomatic elevation in ACTH and cortisol levels likely consistent with glucocorticoid resistance (GR).


Methods :

n/a


Results : n/a


Discussion :

A 39 year old male presented with hair loss and was diagnosed with AA. His condition progressed despite topical treatments and injections with eventual loss of 50% of his scalp hair in addition to hair loss on his legs and eyebrows. All treatments were subsequently discontinued. Two years later, the patient was referred to a dermatologist because of “tanned skin”. An ACTH level was sent and was found to be elevated at 116 pg/mL (9-42 pg/mL). This prompted an endocrinology referral. He denied any symptoms of cortisol excess aside from a 10 lbs weight gain over 1 year. On examination, BMI was 28 with a muscular build, blood pressure was within normal limits, patchy areas of alopecia were noted over his scalp and he had tanned skin. Features of cortisol excess such as moon facies, supraclavicular fat pad deposition, truncal obesity or violaceous striae, were absent.

Workup revealed 2 consecutive midnight salivary cortisol levels of 182 and 169 ng/dL ((was 121.4 µg/day(µg/dL (3.4-26.9 µg/dL) and 97 pg/mL respectively. Cortisol binding globulin was 3.2mg/dL (1.9-7.5 mg/dL). A 1-mg overnight dexamethasone suppression test (DST) revealed a cortisol of 6.3 µg/dL (3.4-26.9 µg/dL), ACTH of 80.7 pg/mL (9-46 pg/mL) with a dexamethasone level of 282 ng/dL (180-550ng/dL). 8mg DST revealed a cortisol of 6.5 µg/dL , ACTH of 57.1 pg/mL . Evaluation of remainder of pituitary axis was unremarkable. MRI of the pituitary was unremarkable.


Conclusion : The treatment for AA is GC. In patients with hypercortisolemia, AA lesions are expected to improve. We report a case of treatment-resistant AA in a patient with complete absence of physical signs of Cushing’s syndrome in association with elevated ACTH, elevated salivary and urinary free cortisol levels, and failure to suppress on dexamethasone treatment all of which suggest a diagnosis of GR.

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Alexandra Mikhael

Endocrinology Fellow
Department of Endocrinology, Diabetes and Metabolism, Cleveland Clinic
Cleveland, Ohio

Endocrinology Fellow, Department of Endocrinology, Diabetes and Metabolism, Cleveland Clinic

Laurence Kennedy

Chairman, Department of Endocrinology, Diabetes and Metabolism
Cleveland Clinic, Ohio

Chairman, Department of Endocrinology, Diabetes and Metabolism. Cleveland Clinic

Mary VOUYIOUKLIS.. Kellis

Associate Staff
Cleveland Clinic, Department of Endocrinology, Diabetes and Metabolism, Ohio

I am currently Associate Staff at the Cleveland Clinic, Department of Endocrinology, Diabetes and Metabolism. I graduated Magna Cum Laude from Columbia University. I went to Albert Einstein College of Medicine in the Bronx, New York and trained at NYU Langone Medical center in Internal medicine. I was selected Chief Resident there and also completed a fellowship in Endocrinology at NYU. I am currently Staff at the Cleveland Clinic and my focus of interest is thyroid, diabetes and adrenal disorders.

Alexandra Mikhael

Endocrinology Fellow
Department of Endocrinology, Diabetes and Metabolism' Cleveland Clinic
Beachwood, Ohio