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DIAGNOSTIC APPROACH TO A
CASE 0F HIRSUTISM
Dr. RANJAN DASH
THE FORBIDDEN AREA
INTRODUCTION
 Hirsutism is a common clinical condition affecting 5-10% of the women across
the globe
 Hirsutism has a huge psychosocial impact, especially in the young females
 Excess body hair outside of cultural norms can be very distressing
 Distinguishing normal variations of hair growth from hypertrichosis and true
hirsutism is important
 The cause is mainly hyperandrogenism , which may be ovarian or adrenal. It may
be part of a rare metabolic syndrome, drug induced, or just idiopathic
Pattern of Normal Hair Distribution
SITES OF HAIR
Non sexual Ambi-sexual Male sexual
Sites Lower parts of the
scalp, eye brow,
lashes, fore-arms,
lower legs.
Temporal & vertical
parts of the scalp,
axilla, lower pubic
hair.
Ears, nasal tip, chin,
sternum, upper pubic
triangle, back.
Depend on Growth hormone
from pituitary.
Androgen in low
concentration from
the adrenals &
ovaries in females &
adrenals in males.
Androgen in high
concentration.
HIRSUTISM and SINISTERS
Hirsutism
 presence of excessive terminal hair in androgen-dependent areas (male pattern)
in a woman’s body
a sign of increased androgen action on hair follicle (endogenous or exogenous)
 increased sensitivity of hair follicles to normal levels of circulating androgens
Hypertrichosis
 Androgen independent excess hair growth
 May be generalised or localised
 May consist of lanugo, vellus or terminal hair
 Frequently associated with the use of medication such as antiepileptics
Masculinization : the process of becoming more manlike and developing a male hair
pattern, increased pectoral musculature, and huskiness of the voice
Virilization : masculinization associated with a more complete voice change,
changes in libido, and clitoromegaly
SIGNS SUGGESTIVE OF ANDROGEN EXCESS
 Acne
 Frontotemporal balding
 Deepening of the voice
 Decrease in breast size
 Clitoral hypertrophy
 Increased muscle mass
 Amenorrea / oligomenorrhea
NEED FOR AN APPROACH
 Although treatment for hirsutism is sought for cosmetic reasons , hirsutism
may signal more serious internal pathology
 Unattended, this can lead to serious psychosocial morbidity,especially in young
females
 Therefore , a proper diagnostic approach entails a thorough history , clinical
and biochemical evaluation
Evaluation of History
Age of onset :congenital/early onset and late onset
Menstrual history : regular or irregular
Drug intake : drugs causing hirsutism and drugs causing hypertrichosis
AGE of ONSET
Congenital /Early onset H. Late onset H.
CAH (Congenital Adrenal Hyperplasia) Idiopathic hirsutism
Pituitary adenoma PCOS (Polycystic ovarian syndrome)
Acromegaly Late onset CAH
MENSTRUAL HISTORY
Normal Abnormal
Hair pattern Hair pattern
Vellus Terminal Vellus Terminal
Ethnic variation Misunderstanding of normal hair pattern Medications Medications
Medication Hirsutism by proxy
Delusional fixation
Plasma Testosterone
>2ng < 2ng
Nonclassic CAH Insulin Resistance
DRUG INTAKE
Positive Negative
Cyclosporine Consider Paraneoplastic syndrome
Diazoxide
Dexamethasone
Penicillamine
Phenytoin
Interferon
Minoxidil
Medications That May Cause
Hirsutism and/or Hypertrichosis
Hirsutism
 Anabolic steroids
 Danazol (Danocrine)
 Metoclopramide (Reglan)
 Methyldopa (Aldomet)
 Phenothiazines
 Progestins
 Reserpine (Serpasil)
 Testosterone
Clinical Evaluation
1. Vellus vs terminal hair
2. Genital ambiguity
3. BMI
4. ENDOCRINE
5. Neoplastic
VELLUS VS TERMINAL HAIR
Vellus Terminal
Medication History Menstrual History
Positive Negative Normal Abnormal
Modified Ferriman-Gallwey score : Nine body regions are evaluated for their degree of hair growth from 0–4.
A total score >8 is a sign for hirsutism.
AMBIGUOUS GENITALIA FLOW CHART
External Genitalia
Ambiguous Not Ambiguous
Measure Plasma Hair Type
17-hydroxyprogesterone
11-deoxycortisol
17-hydroxy pregnenolone Vellus Terminal
Medication History Menstrual History
Normal Abnormal
Medical genetics consultation Congenital Adrenal Hyperplasia Positive Negative Positive Negative
Treat with glucocorticoids
BMI FLOW CHART
BMI
Increased Normal
Insulin Resistance- likely Gonadotrophin suppression with GnRH Superagonist
Treat with weight loss if possible
Cosmetic treatments also very helpful
Positive Negative
Gonadotrophin dependent Gonadotrophin independent
Ovarian Hyperandrogenism Ovarian Hyperandrogenism
ENDOCRINE OVARIAN VS OTHERS
Endocrine
Ovarian Others
Neoplasms Acromegaly
Insulin resistance Cushing’s syndrome
Familial ovarian hyperandrogenism Hypo or hyperthyroidism
Hyperthecosis Adrenal tumors
Persistent corpus luteum of pregnancy
HPA vs HPO Axis
NEOPLASTIC OVARIAN VS OTHERS
Neoplastic
Ovarian Others
Arrhenoblastoma Virilizing adrenal adenoma
Leydig cell tumor Virilizing adrenal carcinoma
Hilus cell tumor Pituitary adenoma
BIOCHEMICAL EVALUATION
• Testosterone : normal /increased and definitely raised (>200ng/dl)
• Dehydroepiandrosterone sulfate (DHEAS) : Raised DHEAS (>700 μg/dl)
always indicates an adrenal cause, benign or malignant.
• 17 Hydroxy progesterone : Levels < 200 ng/dl excludes the disease
Post-stimulation values (>1,000 ng/dl) constitute a positive test
• Twenty four hour urine free cortisol in women with signs and symptoms of
Cushing's syndrome.
Prolactin : raised in hyperprolactinemia due to hypothalamic disease or a pituitary tumor.
Serum TSH: Hypophyseal hypothyroidism can act as a cofactor in hirsutism causing raised TSH
LH/FSH greater than 3 is indicative of PCOS.
TESTOSTERONE FLOW CHART
Plasma Testosterone
>200 ng/dl <200 ng/dl
Probably neoplastic ACTH Stimulation Test
MRI Adrenal Glands 17-OH progesterone
Abnormal Normal >1000 ng/dl <1000 ng/dl
Must rule out adrenal Ovarian Source
adenoma or carcinoma Non classical CAH BMI
MRI and/or ultrasound
of pelvic organs
Insulin Normal
Resistance
Pelvic USG MRI/CT Abdomen
PCOS Adrenal tumor
ovarian neoplasm CAH
IMAGING
Key Message
 Hirsutism is very common; the causes are usually benign
but the psychological impact can be severe
 Few investigations are needed in most cases of hirsutism
 Effective treatment usually requires a combination of physical hair
removal and endocrine treatment
Diagnostic approach to a case of Hirsutism

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Diagnostic approach to a case of Hirsutism

  • 1. DIAGNOSTIC APPROACH TO A CASE 0F HIRSUTISM Dr. RANJAN DASH
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  • 4. INTRODUCTION  Hirsutism is a common clinical condition affecting 5-10% of the women across the globe  Hirsutism has a huge psychosocial impact, especially in the young females  Excess body hair outside of cultural norms can be very distressing  Distinguishing normal variations of hair growth from hypertrichosis and true hirsutism is important  The cause is mainly hyperandrogenism , which may be ovarian or adrenal. It may be part of a rare metabolic syndrome, drug induced, or just idiopathic
  • 5. Pattern of Normal Hair Distribution
  • 6. SITES OF HAIR Non sexual Ambi-sexual Male sexual Sites Lower parts of the scalp, eye brow, lashes, fore-arms, lower legs. Temporal & vertical parts of the scalp, axilla, lower pubic hair. Ears, nasal tip, chin, sternum, upper pubic triangle, back. Depend on Growth hormone from pituitary. Androgen in low concentration from the adrenals & ovaries in females & adrenals in males. Androgen in high concentration.
  • 7.
  • 8. HIRSUTISM and SINISTERS Hirsutism  presence of excessive terminal hair in androgen-dependent areas (male pattern) in a woman’s body a sign of increased androgen action on hair follicle (endogenous or exogenous)  increased sensitivity of hair follicles to normal levels of circulating androgens Hypertrichosis  Androgen independent excess hair growth  May be generalised or localised  May consist of lanugo, vellus or terminal hair  Frequently associated with the use of medication such as antiepileptics
  • 9. Masculinization : the process of becoming more manlike and developing a male hair pattern, increased pectoral musculature, and huskiness of the voice Virilization : masculinization associated with a more complete voice change, changes in libido, and clitoromegaly SIGNS SUGGESTIVE OF ANDROGEN EXCESS  Acne  Frontotemporal balding  Deepening of the voice  Decrease in breast size  Clitoral hypertrophy  Increased muscle mass  Amenorrea / oligomenorrhea
  • 10. NEED FOR AN APPROACH  Although treatment for hirsutism is sought for cosmetic reasons , hirsutism may signal more serious internal pathology  Unattended, this can lead to serious psychosocial morbidity,especially in young females  Therefore , a proper diagnostic approach entails a thorough history , clinical and biochemical evaluation
  • 11. Evaluation of History Age of onset :congenital/early onset and late onset Menstrual history : regular or irregular Drug intake : drugs causing hirsutism and drugs causing hypertrichosis
  • 12. AGE of ONSET Congenital /Early onset H. Late onset H. CAH (Congenital Adrenal Hyperplasia) Idiopathic hirsutism Pituitary adenoma PCOS (Polycystic ovarian syndrome) Acromegaly Late onset CAH
  • 13. MENSTRUAL HISTORY Normal Abnormal Hair pattern Hair pattern Vellus Terminal Vellus Terminal Ethnic variation Misunderstanding of normal hair pattern Medications Medications Medication Hirsutism by proxy Delusional fixation Plasma Testosterone >2ng < 2ng Nonclassic CAH Insulin Resistance
  • 14. DRUG INTAKE Positive Negative Cyclosporine Consider Paraneoplastic syndrome Diazoxide Dexamethasone Penicillamine Phenytoin Interferon Minoxidil
  • 15. Medications That May Cause Hirsutism and/or Hypertrichosis Hirsutism  Anabolic steroids  Danazol (Danocrine)  Metoclopramide (Reglan)  Methyldopa (Aldomet)  Phenothiazines  Progestins  Reserpine (Serpasil)  Testosterone
  • 16. Clinical Evaluation 1. Vellus vs terminal hair 2. Genital ambiguity 3. BMI 4. ENDOCRINE 5. Neoplastic
  • 17. VELLUS VS TERMINAL HAIR Vellus Terminal Medication History Menstrual History Positive Negative Normal Abnormal
  • 18.
  • 19. Modified Ferriman-Gallwey score : Nine body regions are evaluated for their degree of hair growth from 0–4. A total score >8 is a sign for hirsutism.
  • 20. AMBIGUOUS GENITALIA FLOW CHART External Genitalia Ambiguous Not Ambiguous Measure Plasma Hair Type 17-hydroxyprogesterone 11-deoxycortisol 17-hydroxy pregnenolone Vellus Terminal Medication History Menstrual History Normal Abnormal Medical genetics consultation Congenital Adrenal Hyperplasia Positive Negative Positive Negative Treat with glucocorticoids
  • 21. BMI FLOW CHART BMI Increased Normal Insulin Resistance- likely Gonadotrophin suppression with GnRH Superagonist Treat with weight loss if possible Cosmetic treatments also very helpful Positive Negative Gonadotrophin dependent Gonadotrophin independent Ovarian Hyperandrogenism Ovarian Hyperandrogenism
  • 22. ENDOCRINE OVARIAN VS OTHERS Endocrine Ovarian Others Neoplasms Acromegaly Insulin resistance Cushing’s syndrome Familial ovarian hyperandrogenism Hypo or hyperthyroidism Hyperthecosis Adrenal tumors Persistent corpus luteum of pregnancy
  • 23. HPA vs HPO Axis
  • 24.
  • 25.
  • 26. NEOPLASTIC OVARIAN VS OTHERS Neoplastic Ovarian Others Arrhenoblastoma Virilizing adrenal adenoma Leydig cell tumor Virilizing adrenal carcinoma Hilus cell tumor Pituitary adenoma
  • 27. BIOCHEMICAL EVALUATION • Testosterone : normal /increased and definitely raised (>200ng/dl) • Dehydroepiandrosterone sulfate (DHEAS) : Raised DHEAS (>700 μg/dl) always indicates an adrenal cause, benign or malignant. • 17 Hydroxy progesterone : Levels < 200 ng/dl excludes the disease Post-stimulation values (>1,000 ng/dl) constitute a positive test • Twenty four hour urine free cortisol in women with signs and symptoms of Cushing's syndrome.
  • 28. Prolactin : raised in hyperprolactinemia due to hypothalamic disease or a pituitary tumor. Serum TSH: Hypophyseal hypothyroidism can act as a cofactor in hirsutism causing raised TSH LH/FSH greater than 3 is indicative of PCOS.
  • 29. TESTOSTERONE FLOW CHART Plasma Testosterone >200 ng/dl <200 ng/dl Probably neoplastic ACTH Stimulation Test MRI Adrenal Glands 17-OH progesterone Abnormal Normal >1000 ng/dl <1000 ng/dl Must rule out adrenal Ovarian Source adenoma or carcinoma Non classical CAH BMI MRI and/or ultrasound of pelvic organs Insulin Normal Resistance
  • 30. Pelvic USG MRI/CT Abdomen PCOS Adrenal tumor ovarian neoplasm CAH IMAGING
  • 31.
  • 32.
  • 33. Key Message  Hirsutism is very common; the causes are usually benign but the psychological impact can be severe  Few investigations are needed in most cases of hirsutism  Effective treatment usually requires a combination of physical hair removal and endocrine treatment