II. Definition

  1. Increased sexual Hair Growth
  2. Women develop male-type body hair distribution
    1. Face
      1. Mustache
      2. Beard
      3. Sideburns
    2. Body
      1. Chest
      2. Circum-areolar
      3. Linea alba
      4. Abdominal trigone
      5. Inner Thighs

III. Pathophysiology

  1. See Hair Growth
  2. Vellus Hair
    1. Small, straight, fair hairs
  3. Terminal Hair
    1. Large, curly, dark hair
    2. Develop from Vellus Hair in sex-specific regions in response to androgens
  4. Hyperandrogenism in women results in Terminal Hair development from Vellus Hairs

IV. Epidemiology

  1. Excessive upper lip hair in a third of women ages 14-45
  2. Unwanted chin and sideburn hair in 6-9% of women

V. Causes: Common

  1. See Hyperandrogenism Causes
  2. See Medication Causes of Hirsutism
  3. Polycystic Ovary Syndrome (72-82% of cases)
    1. Metrorrhagia and Infertility
    2. Hyperandrogenemia
    3. Insulin Resistance and Acanthosis Nigricans
    4. Central Obesity
  4. Idiopathic Hyperandrogenemia (6-15% of cases)
    1. Normal Menstrual Cycles
    2. Hyperandrogenemia without obvious cause
  5. Idiopathic Hirsutism (5-15% of cases)
    1. Normal androgen levels and no obvious cause of Hirsutism

VI. Causes: Uncommon or Rare

  1. Adrenal hyperplasia (2-4% of cases)
    1. Classic adrenal hyperplasia
      1. Ambiguous Genitalia leads to diagnosis at birth
    2. Non-classic adrenal hyperplasia
      1. Annovulation leads to diagnosis at Puberty
  2. Androgen-secreting tumors - Adrenal or ovarian (0.2% of cases)
    1. Consider for rapid onset Hirsutism, Virilization or palpable abdominal or pelvic mass
    2. Androgen levels will be significantly above normal range
  3. Acromegaly
  4. Cushing Syndrome
  5. Hyperprolactinemia
  6. Hypothyroidism

VII. History

  1. See Ferriman-Gallway Scale
  2. Hirsutism onset
    1. Rapid onset?
    2. Pubertal onset?
  3. Gynecologic history
    1. Metrorrhagia
    2. Infertility
  4. Family History
    1. Hair Growth patterns in women family members (idiopathic Hirsutism)
  5. Hyperandrogenemia signs
    1. Acne Vulgaris
    2. Acanthosis Nigricans
  6. Virilization signs
    1. Deepening voice
    2. Increased muscle mass
    3. Clitoromegaly
    4. Female body contour lost
  7. Other findings
    1. Striae (Cushing Syndrome)
    2. Galactorrhea (Hyperprolactinemia)

VIII. Signs

  1. Hirsutism
    1. See Ferriman-Gallway Scale
    2. Excessive Terminal Hairs in women in sex-specific regions (male distribution)
  2. Other signs of Hyperandrogenism
    1. See Hyperandrogenism
    2. Acne Vulgaris
    3. Alopecia

IX. Signs: Red Flags suggestive of adrenal hyperplasia or androgen secreting tumor

  1. Onset of Hirsutism after Puberty
  2. Rapid progression of Virilization or Hirsutism
  3. Irregular Menses
  4. Exam suggesting Hyperandrogenism or Virilization
  5. Family History does not suggest familial cause

X. Labs: Evaluation of secondary cause (indicated for moderate to severe Hirsutism or red flags above)

  1. Total Testosterone
    1. Total Testosterone >200 ng/dl should prompt complete endocrine workup with Abdomen and Pelvis imaging
    2. Avoid Dehydroepiandrosterone sulfate level (DHEAS) for screening
      1. Mild elevations are common and non-diagnostic with a normal Testerosterone level
  2. 17-Hydroxyprogesterone level
    1. Obtain Corticotropin Stimulation Test (ACTH Stimulation Test) if 17-Hydroxyprogesterone >200 ng/dl
  3. Thyroid Stimulating Hormone
  4. Serum Prolactin level
    1. See Hyperprolactinemia for evaluation
    2. Consider MRI imaging of pituitary
  5. Consider urine free cortisol level
    1. Indicated if Cushing Syndrome suspected

XI. Imaging (as indicated)

  1. Pelvic Ultrasound
    1. May demonstrate Polycystic Ovaries
  2. CT Abdomen and Pelvis
    1. Indicated for rapid Virilization and evaluation for adrenal or ovarian secreting tumor
  3. MRI Brain (or CT Brain)
    1. Indicated for Hyperprolactinemia and evaluation of sella turcica

XII. Evaluation

  1. Step 1: Initial
    1. History including Ferriman-Gallway Scale
    2. Exam including Thyroid exam, skin exam, Breast Exam and abdominal and pelvic exam
  2. Step 2: Consider evaluation for androgen secreting tumor
    1. Indications
      1. Rapid onset Virilization or Hirsutism or abdominal/pelvic mass
      2. If not indicated, go to Step 3
    2. Tests
      1. See labs above
      2. See Imaging above
  3. Step 3: Moderate Hirsutism (Ferriman-Gallwey Score 8-15) or PCOS suspected
    1. If more mild Hirsutism, go to step 4
    2. Tests
      1. See labs above
  4. Step 4: Mild Hirsutism (Ferriman-Gallwey Score 8-15)
    1. Treat Hirsutism (see below)

XIII. Differential Diagnosis

XIV. Management: Hair Removal

XV. Management: Anti-androgen management

  1. Hirsutism related to excess androgen from Anovulation
  2. Mechanisms directed at reducing DHT and androgens
    1. Inhibit ovary and adrenal androgen secretion
    2. Alter Sex Hormone Binding Globulin (SHBG) binding
    3. Impair peripheral androgen precursor conversion
    4. Inhibit androgen action at target tissue
  3. General Measures
    1. Weight loss if Obesity present (lowers androgens)
    2. See Hair Removal Techniques
  4. Medications: First line
    1. Oral Contraceptives
      1. Lowers Serum LH: Decreases Testosterone production
      2. Increase Serum SHBG: Increases Testosterone binding
      3. Decreases Free Testosterone (unbound) levels
      4. Lowest Progestin Androgenic Activity
        1. Norgestimate (Ortho Tricyclen, Ortho Cyclen)
        2. Desogestrel (Ortho-Cept, Desogen)
        3. Norethindrone (Modicon)
        4. Ethynodiol (Demulen 1/35)
    2. Spironolactone 100 to 200 mg PO divided bid to tid
      1. Category D medication in pregnancy
    3. Eflornithine (Vaniqa) 13.9% cream
      1. FDA approved only for unwanted facial hair
      2. May be an adjunct to other hair removal methods, but effects are only temporary
  5. Medications: Second-Line for specific indications
    1. Metformin (Glucophage): Polycystic Ovary Syndrome
      1. Not indicated for Hirsutism without Polycystic Ovary Syndrome
  6. Medications: Third line due to potential toxicity
    1. Indicated only in severe, refractory cases
    2. Most of these agents are Teratogenic and require reliable Contraception
    3. Antiandrogen
      1. Flutamide (Eulexin) 250 mg bid to tid
        1. Endocrine Society discourages Flutamide use due to liver failure risk
      2. Finasteride 5 mg orally daily
        1. Category X medication in pregnancy (must use reliable Contraception)
        2. Hepatotoxicity risk
    4. Glucocorticoid: Dexamethasone 0.5 mg PO qHS
      1. May be indicated in non-classic Congenital Adrenal Hyperplasia
    5. GnRH agonist: Leuprolide (Lupron Depot)
      1. Dose: 3.75 mg to 7.5 mg IM qMonth for 6 months
      2. Depot dose: 11.25 mg q3 months
      3. Category X medication in pregnancy
      4. Causes menopausal symptoms (consider add-back hormones)
    6. Ketoconazole
      1. Not recommended due to Ketoconazole hepatotoxicity

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Ontology: Hirsutism (C0019572)

Definition (NCI_CTCAE) A disorder characterized by the presence of excess hair growth in women in anatomic sites where growth is considered to be a secondary male characteristic and under androgen control (beard, moustache, chest, abdomen).
Definition (NCI) Male-pattern hair growth on a female.(NICHD)
Definition (MSH) A condition observed in WOMEN and CHILDREN when there is excess coarse body hair of an adult male distribution pattern, such as facial and chest areas. It is the result of elevated ANDROGENS from the OVARIES, the ADRENAL GLANDS, or exogenous sources. The concept does not include HYPERTRICHOSIS, which is an androgen-independent excessive hair growth.
Definition (CSP) excess hair in females and children with an adult male pattern of distribution.
Concepts Finding (T033)
MSH D006628
ICD9 704.1
ICD10 L68.0
SnomedCT 40090008, 399939002
English Hirsutism, HIRSUTISM, Excessive growth of hair, Excessive hair growth, Hairiness, hirsutism, excessive facial or body hair (symptom), hirsutism (diagnosis), excessive facial or body hair (hirsutism), excessive facial or body hair, Hirsutism [Disease/Finding], hypertrichosis, polytrichia, excessive hairiness, excessive growth hair, hairiness, excessive growth of hair, excessive hair growth, pilosis, hirsuitism, hirsute, Hirsuitism, Hirsutism (disorder), Hirsuties, Pilosis, Hirsutes, Hirsutism, NOS
French HIRSUTISME, Pilosité, Hirsutisme
Portuguese HIRSUTISMO, Pilosidade, Hirsutismo
Spanish HIRSUTISMO, Vellosidad, crecimiento excesivo del pelo (trastorno), crecimiento excesivo del pelo, pilosis, hirsutismo (trastorno), hirsutismo, Hirsutismo
German HIRSUTISMUS, Hirsuitismus, Behaartheit, Hirsutismus
Dutch behaardheid, hirsutisme, Hirsutisme
Italian Villosità, Pelosità anormale, Irsutismo
Japanese 男性型多毛症, ダンセイガタタモウショウ
Swedish Hårighet, patologisk
Czech hirzutismus, Hirsutismus, Zvýšené ochlupení, nadměrné ochlupení, hirsutizmus
Finnish Hirsutismi
Korean 털과다증
Polish Hirsutyzm
Hungarian Szőrösség, Hirsutismus, túlzott szőrnövekedés
Norwegian Unormal hårvekst, Hirsutisme, Hårvekst, unormal