Severe Hirsutism in Non Classic Congenital Adrenal Hyperplasia: A Case Report and Literature Review

Authors

  • Merita Emini Faculty of Medicine, University of Prishtina, Prishtina, Kosovo; Clinic of Endocrinology, University Clinical Center of Kosovo, Prishtina, Kosovo
  • Blertina Olldashi Clinic of Endocrinology, University Clinical Center of Albania, Tirana, Republic of Albania
  • Blerina Qovanaj Faculty of Medicine, University of Prishtina, Prishtina, Kosovo

DOI:

https://doi.org/10.3889/oamjms.2020.3423

Keywords:

Congenital adrenal hyperplasia, Hirsutism, Hair loss

Abstract

 

 BACKGROUND: Non-classic congenital adrenal hyperplasia (NCAH) is a relatively common disorder, but very often, it remains undiagnosed. Clinical appearance of NCAH can be asymptomatic at birth. Symptoms are presented more often in late childhood, adolescence, or later in life caused by excessive androgen secretion.

CASE REPORT: We report the case of 27-year-old girl with 2 years history of high-grade of hirsutism, hair loss on the front of scalp with female pattern hair loss, (grade II according to Ludwig scale), breast volume reduction, weight loss (15 kg), depression, but without menstrual disorders, normal blood pressure, and usage of oral contraceptives for more than 1 year without any improvement. We did blood tests for hormonal protocol for hyperandrogenemia and we found these values high 17 (OH) progesterone and all other androgen hormones. After these examinations, the patient was diagnosed with a non-classic form of CAH.

CONCLUSION: Treatment with corticosteroids and other necessary treatment improved clinical and hormonal features.

 

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References

Krone N, Dhir V, Ivison HE, Arlt W. Congenital adrenal hyperplasia and P450 oxidoreductase deficiency.Clin Endocrino (Oxf). 2007;66(2):162-72. https://doi. org/10.1111/j.1365-2265.2006.02740.x PMid:17223983

White PC, Speiser PW. Congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Endocr Rev. 2000;21(5):245-91. https://doi.org/10.1210/edrv.21.3.0398 PMid:10857554

New MI. Extensive clinical experience: Nonclassical 21-hydroxylase deficiency. J Clin Endocrinol Metab. 2006;91(11):4205-14. PMid:16912124

Speiser PW. Nonclassic adrenal hyperplasia. Rev Endocr Metab Disord. 2009;10(1):77-82. https://doi.org/10.1007/ s11154-008-9097-x PMid:18690539

Pang S, Shook MK. Current status of neonatal screening for congenital adrenal hyperplasia. Curr Opin Pediatr. 1997;9(4):419-23. PMid:9300201

Therrell BL. Newborn screening for congenital adrenal hyperplasia. Endocrinol Metab Clin North Am. 2001;30(1):15-30. PMid:11344933

van der Kamp HJ, Wit JM. Neonatal screening for congenital adrenal hyperplasia. Eur J Endocrinol. 2004;151 Suppl 3:U71-5. https://doi.org/10.1530/eje.0.151u071 PMid:15554889

Unluhizarci K, Kula M, Dundar M, Tanriverdi F, Israel S, Colak R, et al. The prevalence of non-classic adrenal hyperplasia among Turkish women with hyperandrogenism. Gynecol Endocrinol. 2010;26(2):139-43. https://doi. org/10.3109/09513590903215466 PMid:19718570

Witchel SF. Nonclassic congenital adrenal hyperplasia. Curr Opin Endocrinol Diabetes Obes. 2012;19(3):151-8. PMid:22499220

Lekarev O, New MI. Adrenal disease in pregnancy. Best Pract Res Clin Endocrinol Metab. 2011;25(6):959-73. PMid:22115169

Pinkas H, Fuchs S, Klipper-Aurbach Y, Zvulunov A, Raanani H, Mimouni G, et al. Non-classical 21-hydroxylase deficiency: Prevalence in males with unexplained abnormal sperm analysis. Fertil Steril. 2010;93(6):1887-91. https://doi.org/10.1016/j. fertnstert.2008.12.037 PMid:19200987

Merke DP, Bornstein SR. Congenital adrenal hyperplasia. Lancet. 2005;365(9477):2125-36. PMid:15964450

Moran C, Azziz R, Carmina E, Dewailly D, Fruzzetti F, Ibañez L, et al. 21-Hydroxylase-deficient nonclassic adrenal hyperplasia is a progressive disorder: A multicenter study. Am J Obstet Gynecol. 2000;183(6):1468-74. https://doi.org/10.1067/ mob.2000.108020 PMid:11120512

Witchel SF, Azziz R. Nonclassic congenital adrenal hyperplasia. Int J Pediatr Endocrinol. 2010;2010:625105. https://doi. org/10.1186/1687-9856-2010-625105 PMid:20671993

Burger HG. Androgen production in women. Fertil Steril. 2002;77 Suppl 4:S3-5. PMid:12007895

Bidet M, Bellanné-Chantelot C, Galand-Portier MB, Tardy V, Billaud L, Laborde K, et al. Clinical and molecular characterization of a cohort of 161 unrelated women with nonclassical congenital adrenal hyperplasia due to 21-hydroxylase deficiency and 330 family members. J Clin Endocrinol Metab. 2009;94(5):1570-8. https://doi.org/10.1210/jc.2008-1582 PMid:19208730

Nordenstrom A, Falhammar H. Management of endocrine disease: Diagnosis and management of the patient with non- classic CAH due to 21-hydroxylase deficiency. Eur J Endocrinol. 2019;180(3):R127-45. https://doi.org/10.1530/eje-18-0712

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Published

2020-02-05

How to Cite

1.
Emini M, Olldashi B, Qovanaj B. Severe Hirsutism in Non Classic Congenital Adrenal Hyperplasia: A Case Report and Literature Review. Open Access Maced J Med Sci [Internet]. 2020 Feb. 5 [cited 2024 Apr. 19];8(C):8-11. Available from: https://oamjms.eu/index.php/mjms/article/view/3423

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Case Report in Internal Medicine

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