Familial acanthosis nigricans. A longitudinal study.

The triad of hyperandrogenism, insulin resistance and acanthosis nigricans (HAIR-AN) is known to have a familial tendency. In order to better understand the natural history and possible treatment of this disorder, we studied the clinical course of two families in which all female progeny were affected. A rapid progression of the HAIR-AN syndrome was apparent during the peripubertal period and early reproductive years. Beyond this period, the progression of HAIR-AN appeared quite variable. Rapid weight gain was a major stimulant for increasing insulin resistance and hyperandrogenemia. In the one subject with a postmenarcheal onset of the syndrome, prolonged weight reduction was associated with an improvement in the dermatologic manifestation as well as in the hyperandrogenism and insulin resistance. Oral contraceptives appear to improve the dermatologic symptoms of the HAIR-AN syndrome.