HAIR-AN syndrome

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Classification according to ICD-10
E28.2 Polycystic Ovarian Syndrome
ICD-10 online (WHO version 2019)

The HAIR-AN syndrome , acronym for H yper A ndrogenämie , I nsulin R esistenz and A canthosis N igricans , is a very rare congenital disease with the eponymous principal features.

The syndrome can be viewed as a subtype of polycystic ovary syndrome in which there is pronounced insulin resistance.

distribution

The disease affects (almost) only women. About 5–10% of women with hyperandrogenemia have HAIR-AN syndrome, up to 40% of young women with menstrual disorders can have this syndrome.

Sometimes there is an association with autoimmune diseases such as Hashimoto's thyroiditis and Graves' disease .

root cause

The disease can be caused by two different changes:

The increased insulin level leads to an increase in androgen levels .

Clinical manifestations

Clinical criteria are:

  • Hyperandrogenemia with hirsutism , acne , seborrhea , menstrual disorder
  • Insulin resistance
  • Acanthosis nigricans

diagnosis

The diagnosis is based on the clinical findings and the changed laboratory values ​​in the blood serum.

Differential diagnosis

Must be distinguished are the PCOS and insulin resistance syndrome type A .

therapy

Treatment can include antiandrogens and weight loss measures .

literature

  • M. Peigné, A. Villers-Capelle, G. Robin, D. Dewailly: Hyperandrogénie féminine. In: Presse medicale. Vol. 42, No. 11, November 2013, pp. 1487-1499, doi: 10.1016 / j.lpm.2013.07.016 , PMID 24184282 (review).
  • R. Azziz, E. Carmina, D. Dewailly, E. Diamanti-Kandarakis, HF Escobar-Morreale, W. Futterweit, OE Janssen, RS Legro, RJ Norman, AE Taylor, SF Witchel: The Androgen Excess and PCOS Society criteria for the polycystic ovary syndrome: the complete task force report. In: Fertility and sterility. Vol. 91, No. 2, February 2009, pp. 456-488, doi: 10.1016 / j.fertnstert.2008.06.035 , PMID 18950759 (review).
  • KK McClanahan, HA Omar: Navigating adolescence with a chronic health condition: a perspective on the psychological effects of HAIR-AN syndrome on adolescent girls. In: TheScientificWorldJournal. Vol. 6, October 2006, pp. 1350-1358, doi: 10.1100 / tsw.2006.242 , PMID 17072487 (review).
  • HA Omar, S. Logsdon, J. Richards: Clinical profiles, occurrence, and management of adolescent patients with HAIR-AN syndrome. In: TheScientificWorldJournal. Vol. 4, July 2004, pp. 507-511, doi: 10.1100 / tsw.2004.106 , PMID 15258677 .

Individual evidence

  1. ↑ Encyclopedia Dermatology
  2. ↑ a b c d A. H. Dédjan, A. Chadli u. a .: Hyperandrogenism-Insulin Resistance-Acanthosis Nigricans Syndrome. In: Case Reports in Endocrinology. 2015, 2015, p. 1, doi: 10.1155 / 2015/193097 .
  3. ^ KM Rager, HA Omar: Androgen excess disorders in women: the severe insulin-resistant hyperandrogenic syndrome, HAIR-AN. In: TheScientificWorldJournal. Vol. 6, January 2006, pp. 116-121, doi: 10.1100 / tsw.2006.23 , PMID 16435040 (review).
  4. ↑ a b K. B. Elmer, RM George: HAIR-AN syndrome: a multisystem challenge. In: American family physician. Vol. 63, No. 12, June 2001, pp. 2385-2390, PMID 11430453 .
  5. ↑ M. Manco, M. Castagneto, G. Nanni, C. Guidone, V. Tondolo, AV Greco, G. Gasbarrini, G. Mingrone: Biliopancreatic diversion as a novel approach to the HAIR-AN syndrome. In: Obesity surgery. Vol. 15, No. 2, February 2005, pp. 286-289, doi: 10.1381 / 0960892053268435 , PMID 15802076 .

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