Female pattern hair loss in complete androgen insensitivity syndrome

Abstract

Female pattern hair loss, also known as female androgenetic alopecia, is generally regarded as an androgen-dependent disorder representing the female counterpart of male balding. We describe female pattern hair loss occurring in a patient with complete androgen insensitivity syndrome suggesting that mechanisms other than direct androgen action contribute to this common form of hair loss in women.Women with female pattern hair loss (FPHL) typically present with a history of gradual thinning of scalp hair, often over a period of several years. On examination they show widening of the central parting with a diffuse reduction in hair density mainly affecting the frontal scalp and crown. The frontal hairline is usually retained. FPHL is common, affecting about 5% of women by the age of 50 years increasing to over 30% by age 70. 1,2 The underlying change in hair follicle physiology is progressive miniaturization in a proportion of hair follicles, shortening of the growth phase of the hair cycle (anagen) and prolongation of the resting phase (telogen). 3,4 FPHL has long been thought to be the female counterpart of male balding and is often referred to as female androgenetic alopecia. However, the role of androgens in FPHL is less clear-cut than in male balding. We report a case of FPHL that reinforces this uncertainty and implicates androgen-independent mechanisms in the pathogenesis. Case reportA 52-year-old woman presented with a 10-year history of gradual thinning of her scalp hair. On examination she had a diffuse reduction in hair density affecting the mid and frontal regions of the scalp with retention of the frontal hair line (Fig. 1). The scalp appeared normal with no evidence of scarring. The clinical features were typical of FPHL of Ludwig grade I. Several years previously she had been diagnosed with complete androgen insensitivity syndrome (CAIS) following investigation of primary amenorrhoea. Her sister has the same condition but without hair loss. On general examination she had normal female external genitalia and breast development with scanty pubic hair but no axillary hair or beard growth. Chromosome analysis showed a 46XY karyotype. Intra-abdominal testes were found on ultrasound examination. She had high circulating levels of testosterone (28AE1 nmol L )1 , normal male range 8-28) and dihydrotestosterone (3AE9 nmol L )1 , normal male range 0AE9-2AE9). Thyroid-stimulating hormone and free thyroxine levels were normal. Her serum estradiol was in the lower normal range for a woman of reproductive age (142 pmol L )1 ). She continues to be monitored by the gynaecologists with regular ultrasound scans and tumour marker evaluation as she has refused orchiectomy. Androgen receptor gene analysis was also declined by the patient.Prior to referral she had tried topical minoxidil and oestrogen replacement therapy, both for 12 months, with no improvement in hair density. Oral finasteride (dose range 1AE25-2AE5 mg daily) and spironolactone (maximum dose 300 mg daily) were also ineffective. Some improvement was notic...

Controlled clinical trial for evaluation of hair growth with low dose cyclical nutrition therapy in men and women without the use of finasteride

“…Pattern hair loss can occur with normal androgen levels [12,13] , pattern hair loss can occur before puberty [13][14][15] , it has been reported in hypogonadism [16] and also in a case of complete androgen insensitivity syndrome [17] . Instead of insisting on anti-androgens in management of hair loss patients, we should focus on strengthening the hair follicle and promoting better hair growth to ensure more hair on the head.…”

Selected variants of the steroid‐5‐alpha‐reductase isoforms SRD5A1 and SRD5A2 and the sex steroid hormone receptors ESR1, ESR2 and PGR: No association with female pattern hair loss identified

“…Several studies have reported elevated serum androgen levels in affected women (9)(10)(11)(12)(13). In contrast, other studies of FPHL have found normal androgen levels, complete androgen insensitivity syndrome and or the absence of circulating androgens in affected women (3,(14)(15)(16)(17). The role in FPHL of the AR ⁄ EDA2R locus [which contains the androgen receptor (AR)] and the ectodysplasin A2 receptor (EDA2R) [which has been identified as the major susceptibility locus for male AGA (18)(19)(20)] remains unclear, with no replicated association finding having been reported to date (21).…”

“…Body regions rich in sebocytes are hyperpigmented and exhibit the highest HMel density, for example, face, axillae, and genitalia (12). Also, clinical observations suggest that sebocytes influence HMel, leading, for example, to postacne hyperpigmentation; naevus sebaceous appears dark and sebaceous hyperplasia of the vulva is associated with overlying hyperpigmentation and melanocyte hyperplasia (13,14).…”

Hair: What is New in Diagnosis and Management?

“…Pattern hair loss has been described in cases with complete androgen insensitivity syndromes, suggesting that mechanisms other than androgens may be involved. 11 Although FPHL can be associated with hyperandrogenic states, the circulating testosterone levels do not differ between patients with FPHL and normal controls. 7 Many women with FPHL have low levels of circulating sex hormone binding globulin (SHBG), which may increase the available free testosterone at the level of the hair follicle.…”

“…7 Many women with FPHL have low levels of circulating sex hormone binding globulin (SHBG), which may increase the available free testosterone at the level of the hair follicle. 11 Although it has been postulated that there is an increased peripheral sensitivity to androgens in FPHL, the response to treatment with 5a reductase inhibitors is unpredictable. Also, AR polymorphisms have not been uniformly confirmed and cannot completely explain the mechanism of FPHL.…”