Severe Hirsutism in Non Classic Congenital Adrenal Hyperplasia: A Case Report and Literature Review
DOI:
https://doi.org/10.3889/oamjms.2020.3423Keywords:
Congenital adrenal hyperplasia, Hirsutism, Hair lossAbstract
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.
Downloads
Metrics
Plum Analytics Artifact Widget Block
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
Downloads
Published
How to Cite
Issue
Section
Categories
License
Copyright (c) 2020 Merita Emini, Blertina Olldashi, Blerina Qovanaj (Author)
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
http://creativecommons.org/licenses/by-nc/4.0