Treatment of Hirsutism in Nonclassic Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency
Women presenting with hirsutism in the context of nonclassic congenital adrenal hyperplasia (NCCAH) due to 21-hydroxylase deficiency are managed through a structured hormonal treatment approach. This protocol targets measurable hair-growth reduction over the course of therapy.
This protocol applies to hirsutism occurring alongside nonclassic congenital adrenal hyperplasia due to 21-hydroxylase deficiency. The therapeutic approach in these women follows the same framework used for androgen-excess-related hirsutism more broadly.
Oral contraceptive-based hormonal therapy is central to this protocol. Whether an alternative hormonal strategy applies depends on clinical circumstances covered in detail in the full regimen — including options available when pregnancy is not being pursued.
The target is a meaningful reduction in the Ferriman–Gallwey hirsutism score. Given the hair growth cycle, improvement becomes detectable at approximately 6 months of therapy.
References
Our approach to treating hirsutism in women with NCCAH is the same as for women with PCOS.
We suggest starting with an OC and adding an antiandrogen after 6 months if necessary.
Clinicians can administer an antiandrogen as initial therapy if the woman is not pursuing pregnancy and has a reliable form of contraception.
Due to the long hair growth cycle, it takes ;6 months to detect the effects of hormonal therapy and ;9 months for these effects to become maximal.
DOI: 10.1210/jc.2018-00241
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