Endocrine Abstracts

Introduction: The HAIR-AN syndrome, which consists of hyperandrogenism (HA), insulin resistance (IR) and acanthosis nigricans (AN), is an underdiagnosed endocrinopathy.

Observation: We report the case of Miss A.M., 24 years old, admitted for exploration of hyperandrogenism. Anamnesis: the patient reported polyuropolydipsic syndrome and the notion of weight gain without eating disorders, making a total of 28 kg in 6 months. This period is also marked by the onset of amenorrhea, increased body and facial hair, increased pigmentation of skin, increased sweating and alopecia. Upon presentation to the Endocrine clinic, examination of the patient revealed - Severe hirsutism scored at 34 according to the Ferriman and Gallowey score, associated with acne and seborrhea - Homogeneous obesity without male musculature - Acanthosis nigricans was positive at the flexor surfaces, neck, axillae, cubital fossae the base of the neck. - buffalo hump

Investigations showed: follicle stimulating hormone (FSH), luteinizing hormone (LH), and prolactin (PRL) are within normal limits. She had normal estradiol level, and testosterone level of 0.5 mg/l, 4 androstene-dione and 17 OHP within the standards. low-dose dexamethasone suppression test returned to negative at 0.35 mg/dl, 2 normal 24-hour urinary free cortisol (UFC), UFC the first at 31.85 mg/24 h, the second at 42 mg/24 h and salivary cortisol was normal level of 0.6 ng/ml. Ultrasound of the pelvis revealed that the left ovary measured 18.8 cm³ with several anechoic cystic formations, number> 10 of infracentimetric size, The right ovary measured 13.7 cm³ in volume, seat of several anechoic cystic formations number> 10 of infracentimetric size concluding to an aspect in favor of a bilateral ovarian dystrophy. The metabolic impact assessment showed hypertriglyceridemia level of 2.02 g/l and diabetes with an HBA1c 6.8%. From a therapeutic, the patient was started hygiene and dietary measures with metformin for her blood sugar, and to help in enhancing the peripheral insulin action., cyproterone acetate associated with ethinyl estradiol.

Discussion: The HAIR-AN syndrome is one of the most underdiagnosed clinical entities in endocrinology. The reason for this is that women with hyperandrogenism are not usually screened for insulin resistance and acanthosis nigricans. It probably accounts for 1%-3% of women with hyperandrogenism. Barbieri and Ryan have described the salient features of this syndrome.

Conclusion: The HAIR-AN syndrome can cause profound psychological distress manifested by morbidity, depression and a sense of worthlessness which may require long-term psychological support.