Endocrine Abstracts

Introduction: HAIR-AN syndrome is a rare condition, which can affect up to 5% of women with hirsutism. It is formed by the initials of the following clinical entities: HyperAndrogenism (hyperandrogenism), Insulino-Resistance (insulin resistance) and Acanthosis Nigricans. It is therefore a clinical triad that is considered as a severe subtype of PCOS, characterized by insulin resistance that appears early, constant and major. We present the case of a patient with Hair-an syndrome.

Observation : This is a 19-year-old adolescent girl presenting a primary amenorrhea with a progressive hirsutism at 13 years old. This hirsutism as scored 36 in ferriman and gallwey associated with acne as well as Acanthosis nigricans, without abnormal pubertal development. Hormonal assessment showed severe hyperandrogenism with a testosterone level of 3.32 ng/ml and an SDHEA level of around 525 µg/dl (N) as well as a delta 4 androstenedione (2.41 ng/ml) and 17OHP (1.44 ng/ml) within the standards. E2 at 203 pmol/l, FSH: 4.36 ui/l, LH: 6.8 ui/l are normal. Biologically: hyperinsulinism at 97.51 µ IU/ml was objectified, as well as diabetes discovered during an OGTT. Morphologically: The pelvic ultrasound shows pubescent-looking female OGI with a large dystrophic ovary 2-10 g, without tumor process, as well as an abdomino-pelvic CT scan that returned without abnormality. Given the above features, the diagnosis of hyperandrogenism-insulin resistance-acanthosis nigricans syndrome (hairan syndrome) is made.

Conclusion: HAIR-AN syndrome is an extreme form of PCOS. It is necessary to know how to evoke it in front of a clinical picture of severe hirsutism in the presence of acanthosis nigrigans.