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Hirsutism

Hirsutism is defined as the presence of excessive terminal hair in androgen-sensitive
 areas of the female body , it is one of the most common disorders in women during the reproductive age ( H.F Escober-Morreale et al 2012 )

How common –the prevalence of hirsutism is about 10 % in most populations a common reason for presentation in dermatology OP’s for cosmetic reasons Far-East Asian women suffer less frequently

Causes –Number of conditions which can lead to hirsutism is extensive , as a primary care clinician a knowledge of the main causes and primary underlying pathology would suffice . Many women who have hirsutism would be endocrinologically normal , our 
aim is to identify women who would benefit from an endocrinological evaluation , investigations and treatment . Uncomplicated cases are managed in primary care.

PCOS and IH together account for about 90 % of cases of hirsutism.

Polycystic Ovarian Syndrome –PCOS is the commonest cause of hirsutism and affects about 70-80 % of women it is thought that 60-80 % of women with hirsutism have androgen excess ie testosterone -this can come from the ovaries or the adrenals 
 
Ovarian causes – PCOS and ovarian tumours

Adrenal causes – Cushing’s syndrome , androgen producing tumours , congenital adrenal hyperplasia (CAH ) , 21-hydroxylase deficiency.

Idiopathic hirsutism –patients with hirsutism but with normal circulating androgens , normal ovulatory cycles and normal ovaries may be considered a separate group , termed idiopathic hirsutism ( Azziz et al 2000 ) 2nd most common reason after PCOS often familial and thought to be related to disorders in peripheral androgen activity Onset of IH happens shortly after puberty with slow progression always a diagnosis of exclusion.

Congenital adrenal hyperplasia-These are a spectrum of inherited disorders of adrenal steroidogenesis , with decreased cortisol production resulting in overproduction of androgenic steoids ( Deaton et al 1999 ). CAH accounts for 2-4 % of cases of hirsutism.

Other less common/ rare causes –Androgen secreting tumours ( ovarian or adrenal ) , Cushing’s , acromegaly , hyperprolactinemia and thyroid dysfunction , tamoxifen , minoxidil , cyclosporine , phenytoin , interferon.

Drugs –Oral contraceptives ( levonorgestrel , norethindrone and norgestrel ) , anabolic steroids , glucocorticoids , androgen therapy.

Pathophysiology-Androgens are the most important factor for hair growth modulation Andogens are necessary for terminal hair and sebaceous gland development Androgens are involved in keratinization , increased hair follicle size , hair fiber diameter and the time that th terminal hair spends in the anagen phase Hair growth also depends on local factors as (1) peripheral metabolism of androgens (2) end-organ sensitivity (3) other variables as insulin resistance Hirsutism is an androgen- dependent disorder resulting from the interaction between circulating androgen levels and the sensitivity of the hair follicle to androgen Hirsuitism indicates an increased androgen action on hair follicles due to an increased levels of androgens ( endogenous ) or increased sensitivity of the hair follicles to a normal circulating level of androgens.

History-Age of onset Ethnicity Speed of onset Sites involved Menstrual & reproductive history ( focus on PCOS ) Signs and symptoms of virilization (r/o adrenal or ovarian tumour )
for e.g acne , deepening of voice , infrequent menstruation , 
loss of breast tissue ,clitoromegaly ( clitoral diameter of 
more than 4 mm ) ,male pattern alopecia Family history ( 50 % have a + ve family hx ) Abdominal symptoms Breast discharge (hyperprolactinemia ) Skin changes ( e.g striae , acne ) Weight gain or diabetes Any treatment used for e.g hair removal methods Medications for e.g
anabolic steroids , danazol , metoclopramide , methyldopa , phenothiazies , progestins , reserpine , valporic acid Impact of the disease ( e.g is she distressed , low self esteem , depression & social difficulties )

Examination –distribution of hair- woman normally present with increased terminal hair at sides of face , upper lip , chin ,upper back , shoulders , sternum and upper abdomen
any hair loss examine the skin for acne , excessive sebum thickening or darkening of skin ( acanthosis nigricans- marker of insulin resistance ) palpate the abdomen for any ovarian mass look for signs of virilization breast examination for galactorrhoea ( if clinically suspected ) check BMI / BP

Ferriman and Gallwey devised a score for clinical quantification of hirsutism , this evaluates
 9 different body parts with scores ranging
 from 0 ( no excessive terminal hair growth ) t0 4 
( extensive hair growth visible ) Max score is 36 but >= 8 typically indicates hirsutism.

Mild hirsutism and normal periods –Many experts suggest that in such cases extensive laboratory workup is usually not required- consider staring empiric therapy-No response or progression, proceed to investigations

Moderate or severe cases –Tests and interpretation of hyperandrogenism is controversial and not straightforward. Consider the following as part of initial screen-Many path labs would offer this option -start with that ( LH , FSH ). Testosterone level-This can be normal to increased based on pathology , a markedly elevated level > 200 ng/ ml indicates a virilizing tumour. Thyroid function tests-hypophyseal hypothyroidism can act as a co-factor in hirsutism causing a raised TSF.Pelvic US to detect an ovarian neoplasm or PCOS. Testing for less common causes of hyperandrogenism include
 DHEAS Free androgen index 17 Hydroxy progesterone Prolactin 24 hr urinary cortisol abdominal CT

Request and interpretation of these tests should be left to specialist endocrinologists.

Refer urgently as suspected cancer –Are we dealing with a potential malignancy ? 
These patient with potential androgen secreting
 tumors of the ovary or adrenal manifest as
 sudden onset rapid progression of hirsutism severe virilization defeminization usully accompany hirsutism a pelvic or abdominal mass.

Hypertrichosis –Hypertrichosis is the most important differential when dealing with patients with hirsutism
 it is a diffuse growth in villous hair growth this is not androgen dependent distribution is generalized or localized in a non-sexual pattern causes of hypertrichosis include

Congenital -e.g Hurler’s syndrome, trisomy 18 syndrome , fetal alcohol syndrome

Associated conditions -e.g hypothyroidis , porphyrias , epidermolysis bullosa , anorexia nervosa , malnutrition ,dermatomyositis or following head injury

Medications as acetazolamide , phenytoin , latanoprost , streptomycin , psoralen , minoxidil , cyclosporine and diazoxide.

Referral-Consider referral if hirsutism is particularly severe if an underlying endocrine condition is suspected that requires secondary care management Refer if raised testosterone level or raised elevated 17-hydroxyprogersterone level Primary care treatment fails for e.g COCP does not work after 6 months Oral contraceptive is contraindicated.

Lifestyle –Advice lifestyle advice like
 physical exercise dietary advise weight management smoking cessation

advice that this will not ameliorate hirsuitism but address the possible metabolic and cardiovascular dysfunction associated with milder hyperandrogenic disorders.

Cosmetic –Traditional methods include bleaching , shaving , plucking , waxing , chemical treatment and electrolysis
 Shaving – cheap and effective but needs to be done frequently & leads to stubble
 Electrolysis -
- leads to long term hair damage.
- is effective but costly and time consuming and larger areas as chest or upper back are difficult to treat.
- a thin needle is inserted into the hair follicle and electrical current is passed
 Laser
- has become increasingly popular
- cause selective photothermolysis
- as it only destroys hairs in the anagen phase several sessions may be needed
- several variants exist as ruby , alexandrite , pulsed diode and YAG
- can be used in combination for e.g with pharmacotherapy
- risk hyperpigmentation ( hence more suitable in women with light skin color ) and paradoxical hypertrichosis 
 ( women of Mediterranean and Middle-East origin.

Combined oral contraceptive –Androgen suppression- oral contraceptive agents are considered to be 1st line in pre-menopausal women. They work by
 suppression of LH secretion – inhibition of ovarian androgen biosynthesis stimulation of SHBG production which effectively decreases the serum free androgen concentrations mild reduction in adrenal androgen 
synthesis


a 13 % eflornithine cream is licenced for facial hirsutism it is a biological modifier of hair follicular growth continuous application reversibly slows facial hair growth in up to 70 % of patients treated costly and not licensed for use elsewhere results in about 8 weeks and hair growth resumes after discontinuation

Others –Various other agents are available for specialist use which include anti-androgen therapy ( spironolactone , cyproterone acetate , finasteride ) GnRH agonists , glucocorticoids, antifungal agents , metformin – discussing them is beyond the scope of this review.

PATIENT INFORMATION

British Association of Dermatologists leaflet on Hirsutism http://www.bad.org.uk/shared/get-file.ashx?id=89&itemtype=document

Hormone Health Network on Hirsutism https://www.hormone.org/diseases-and-conditions/hirsutism

A very detailed information booklet for the patient who is extremely motivated from Reproductive Facts Org https://www.reproductivefacts.org/news-and-publications/patient-fact-sheets-and-booklets/documents/fact-sheets-and-info-booklets/hirsutism-and-polycystic-ovary-syndrome-booket/

University of Rochester Medical Center page on hirsutism https://www.urmc.rochester.edu/encyclopedia/content.aspx?contenttypeid=85&contentid=P00327 

Monash University page on hirsutism https://www.urmc.rochester.edu/encyclopedia/content.aspx?contenttypeid=85&contentid=P00327

A charity for patients with congenital adrenal hyperplasia https://www.caresfoundation.org/what-is-cah/

INFORMATION FOR CLINICIANS

Eflorinithine prescribing tips from Dorset CCG https://www.dorsetccg.nhs.uk/Downloads/aboutus/medicines-management/Other%20Guidelines/2018%20Updated%20Guideline%20for%20Eflornithine.pdf

 

References

  1. 
 Evaluation and Treatment of Women with Hirsutism MELISSA H.HUNTER,M.D.,and PETER J.CAREK,M.D. Medical University of South Carolina,Charleston,South Carolina https://hscj.ufl.edu/obgyn/docs/hirsutism.pdf
  2. Epidemiology, diagnosis and management of hirsutism: a consensus statement by the Androgen Excess and Polycystic Ovary Syndrome Society
    H.F. Escobar-Morreale1,*, E. Carmina2, D. Dewailly3, A. Gambineri4, F. Kelestimur5, P. Moghetti6, M. Pugeat7, J. Qiao8, C.N. Wijeyaratne9, S.F. Witchel10, and R.J. Norman11 http://www.ae-society.org/pdf/guidelines/hirsutism.pdf
  3. Evaluation and Treatment of Hirsutism in Premenopausal Women: An Endocrine Society* Clinical Practice Guideline Kathryn A Martin, R Rox Anderson, R Jeffrey Chang, David A Ehrmann, Rogerio A Lobo, M Hassan Murad, Michel M Pugeat, Robert L Rosenfield The Journal of Clinical Endocrinology & Metabolism, Volume 103, Issue 4, April 2018, Pages 1233–1257, https://doi.org/10.1210/jc.2018-00241
  4. Sachdeva S. Hirsutism: evaluation and treatment. Indian J Dermatol. 2010;55(1):3–7. doi:10.4103/0019-5154.60342
  5. CKS NHS Hirsutism https://cks.nice.org.uk/hirsutism#!scenario
  6. Management of hirsutism Olympia Koulouri, Gerard S Conway BMJ 2009 ;338:b847 An Approach to the Patient with Hirsutism D. Lynn Loriaux Department of Medicine, Oregon Health & Science University, Portland, Oregon 97239
  7. Hirsutism: Diagnosis and Treatment Gokalp Oner* Department of Obstetric and Gynecology, Van Baskale State Hospital, Van, Turkey
  8. NCBI Bookshelf Hirsuitism Hafsi W et al https://www.ncbi.nlm.nih.gov/books/NBK470417/
  9. Hirsutism: diagnosis and treatment Hirsutismo: diagnóstico e tratamento Alexandre Hohl1, Marcelo Fernando Ronsoni1, Mônica de Olivei http://www.scielo.br/pdf/abem/v58n2/0004-2730-abem-58-2-0097.pdf

 

 

 

 

 

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