Excessive Hair Growth in Adolescent

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					EXCESSIVE HAIR
GROWTH IN
ADOLESCENT
    Dr. D’Pankar Banerji
     Consulting Gynecologist
        Infertility Specialist
  Ideal Fertility :IVF and Genetic
                Center
          Jabalpur, India
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     EXCESSIVE HAIR
        GROWTH
IT MAY BE EITHER

 HIRSUTISM
 VIRILIZATION




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        DEFINITION
HIRSUTISM : APPEARANCE OF
 EXCESSIVE COARSE
 (TERMINAL)HAIR IN A PATTERN NOT
 NORMAL IN THE FEMALE
 Definition highlights the abnormal
 distribution of excess hair growth ,such
 as facial ,chest,or upper abdominal hair


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        DEFINITION
HYPERTRICHOSIS : GROWTH OF HAIR
 IN EXCESS OF THE NORMAL WHILE
 LIMITED TO A NORMAL PATTERN OF
 DISTRIBUTION
 It is frequently associated with the use
 of medication such as antiepileptics



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       DEFINITION
VIRILIZATION : REFERS TO CONCURRENT
  PRESENTATION OF HIRSUTISM WITH A
  BROAD RANGE OF SIGNS SUGGESTIVE
  OF ANDROGEN EXCESS,SUCH AS
  ACNE,
  FRONTOTEMPORAL BALDING,
  DEPPENING OF THE VOICE ,
  A DECREASE IN BREAT SIZE
  CLITORAL HYPERTROPHY

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          DEFINITION
INCREASED MUSCLE MASS
AMENORREA / OLIGOMENORRHEA
Virilization is seen less frequently than
hirsutism and may reflect a severe underlying
pathologic condition ,such as malignancy
Hirsutism and virilization are closely linked
and hirsutism may actually be the first
manifestation of a condition that ultimately will
lead to virilization in left untreated

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 BASIC FACTS ABOUT
        HAIR
Hair grows from a individual hair follicle that
are part of a pilosebaceous gland unit
Number of hair follicles is set from birth
Main difference between sexes is the degree
of differentiation of the hair
Human hair growth is continuous
Hair grows in a mosaic pattern(in a given
area ,hair are in different stages of
development)

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BASIC FACTS ABOUT
       HAIR
Some condition may cause a high level
of synchrony between the growth cycles
of hair ,leading to the appearance of
either massive hair loss (alopecia)or
excess hair for a limited period of time




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   BASIC FACTS ABOUT
          HAIR
Growth cycle of the Hair: ACT
  Anagen : Growth phase,85- 90 % of the life
  cycle
  Catagen : rapid involution Phase
  Telogen : Quiescent phase
The growth phase or the anagen phase is
  primarily influenced by disorders that
  stimulate hair growth as well as therapeutic
  midalities.

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   BASIC FACTS ABOUT
          HAIR
Three types of Hair :
  Lanugo : Body hair seen in the fetus and
  newborn
  Vellus : Fine adult hair covering the body
  Terminal hair : Thick pigmented hair of scalp
  and pubic area
Thickness of the terminal hair varies form one
  individual to other depending upon genetic,
  and possibly nutritional

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  BASIC FACTS ABOUT
         HAIR
 Androgen sensitive hair : depend upon
 androgen input for hair growth.
Face,neck,chest,abdomen,axillary,upper
 arms ,inner thighs and pubic hair,+ part
 of the scalp hair.
 Less Androgen independent :
Forearms ,hands .lower legs

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BASIC FACTS ABOUT
       HAIR
                      PITUITARY
              PITUITARY

       ACTH
                 DHEAS
                DHEAS


                DHEA              OVARY
  ADRENAL
    ADRENAL                               OVARY
                 AND,STEN,ONE

                PERIPHERAL
                CONVERSION

               TESTOSTERONE
                 HAIR FOLLICLE
               DIHYDROTESTERONE
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     PRESENTATION
Most of the cases of virilization seen
clinically are acute and striking in nature
and seldom go unrecognized and
usually prompt immediate medical
intervention
Hirsutism may present in variety of
ways


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    PRESENTATION OF
       HIRSUTISM
HIRSUTISM ALONE
HIRSUTISM AND ASSOCIATED
PILOSEBACEOUS UNIT
OVERACTIVITY (ACNE)
HIRSUTISM AND OVULATORY
DISORDERS
HIRSUTISM AND SIGNS OF
VIRILIZATION

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      PRESENTATION OF
         HIRSUTISM
Hirsutism alone is the greatest
challenge,patients usually go to dermatologist
Hirsutism wIth acne is frequently in teenage
girls
Hirsutism with ovulatory disorders comes
mostly to gynecologist
Hirsutism with virilization requires immediate
work-up


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CAUSES OF HIRSUTISM
Excess androgen production
Relative circulating androgen excess
and low binding globulins
Excess end organ response
Patient perception



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     DISORDERS OF EXCESS
    ANDROGEN PRODUCTION

 Source of androgen :
Exogenous
Endogenous (most common)
 Two primary endogenous sources :
Adrenal glands
Ovaries


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     DISORDERS OF EXCESS
    ANDROGEN PRODUCTION

ADRENAL ANDROGEN EXCESS
May be linked to genetically determined
 steroid synthesis enzyme deficiency
Malignant adrenal neoplastic process
Other conditions like Cushing’s syndrome



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      DISORDERS OF EXCESS
     ANDROGEN PRODUCTION
ADRENAL ANDROGEN EXCESS
Three recognised adrenal enzyme deficiencies :
21 alpha Hydroxylase defieiency
11-beta-Hydroxylase deficiency
3-beta-ol-dehydrogenase deficiency
Classical forms are usually presented in
  prenatal or neonatal period as ambiguous
  genitalia in female
Nonclassic forms are linked with hirsutism

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      DISORDERS OF EXCESS
     ANDROGEN PRODUCTION
21-alpha-Hydroxylase deficiency:
  Most common ,<1% to >10%
  Prevalence depends on ethnic
  origin(common in slavs,1/50 Hispanics 1/40,
  ashkenazi jews 1/27
Cushing’s syndrome :Hirsutism with weight gain
  and growth retardation as the primary
  manifestation,with acne and other cutaneous
  problems

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    DISORDERS OF EXCESS
   ANDROGEN PRODUCTION

OVARIAN ORIGIN
 Most common cause is
POLYCYSTIC OVARIAN SYMDROME
Other
Neoplastic ovarian disease



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     Lab.Evaluation of
        Hirsutism
Three basic hormonal evaluation
1. Total testosterone
2. DHEAS
3. AM 17-hydroxyprogesterone




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Total Testosterone
  Normal Value (0.2 –0.9 ng/ml)




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DHEAS   (600 –2800 ng/ml)




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        AM 17 –
hydroxyprogesterone(0.1 –0.8
            ng/ml )




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RELATIVE ANDROGEN
 EXCESS AND SHBG
<3 % TESTOSTERONE IS FREE
Mostly bound to Sex hormone binding
globuline(SHBG)
Dcrease in SHBG leads to Excess free
Testosterone
Causes of Reduced SHBG :
PCOS(Chronic anovulation) and
Obesity

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 EXCESS REPONSIVITY
    TO ANDROGEN
TESTOSTERONE                     TARGET CELLS
5-ALPHA -REDUCTASE

DIHIDROTESTOSTERONE               RECEPTOR
  Excessive response of the receptor to DHT(may be
  due to mutation of the highly polymorphic region in
  gene of the receptor located on X Chromosome
  Over activity of the 5-alpha-reductase (Type –1 and
  Type 2,type –1 is involved in hirsutism )


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 BASIC APPROACH TO
  THE DIAGNOSIS OF
   HIRSUTISM AND
    VIRILIZATION
SYMPTOMS AND HISTORY
SIGNS
PHYSICAL EXAMINATION
INVESTIGATION

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       APPROACH TO
        DIAGNOSIS
It should be methodical.
First step : True nature of presentation
Patient may present with ovulatory problems
and hirsutism may not be reported
There may be normal hair pattern but patient
complains about hirsutism
Evident virilization should investigated at
once

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      APPROACH TO
       DIAGNOSIS
Careful history regarding the timing of
onset and chronological progression
Precocious puberty with androgen
excess suggests adrenal enzyme defect
Family history : androgen excess
disorders



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        APPROACH TO
         DIAGNOSIS
Physical examination
 Establish presence of hirsutism and
 quantifying it
 Presence of acne and virilization and
 rule out hypertrichosis
 Skin hyperpigmentation,acanthosis
 nigricans suggests insulin
 resistance.Often associated with PCO

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       APPROACH TO
        DIAGNOSIS
Measurement of weight and height and
blood pressure: defects relates to
adrenal enzyme defects
Galactorrhoea
Tanner staging : Hirsutism before
Tanner stage 3 to 4 is alarming and
suggests a serious pathology
Visual genital examination for virilization

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     APPROACH TO
      DIAGNOSIS
Semiobjective scoring system :
Ferriman and Gallwey system ,between
6-12 is the lower limit.




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         APPROACH TO
          DIAGNOSIS
INVESTIGATION:
FOR VIRILIZATION :
Work-up focuses of the identification on the
  source of androgen excess
Rule out exogenous androgen
Evidence of endogenous androgen excess:
 Serum total testosterone
Serum dehydroepiandrosterone sulfate
  (DHEAS)

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         APPROACH TO
          DIAGNOSIS
INVESTIGATION:
FOR VIRILIZATION
Imaging studies:Pelvic sonography
               Adrenal imaging(USG,CT)
Specialized studies :
Selective venous catherization(adrenal or
  ovarian)
Radioisotope studies

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          APPROACH TO
           DIAGNOSIS
INVESTIGATION :
HIRSUTISM: Goal is to rule out serious
  potential life threatening conditions and gain
  information that helps in treatment
Evaluation of Androgen excess:
  Testosterone ,total preferred
  DHEAS
  In selected cases : 17-OHP(fasting morning
  sample)

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          APPROACH TO
           DIAGNOSIS
Evaluation of accompanying medical disorder
  Ovulation disorder :FSH,LH
  Thyroid dysfunction:TSH
  Hyperprolactinemia :PRL
Other investigations ( inselected cases)
  Androgen production :Androstenedione,
   3-alpha Androstenediol glucuronide
  Provocative tests : Corticotropin stimulation
  tests,Insulin resistance determination
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THERAPEUTIC OPTIONS
VIRILIZATION
  GOAL: Identify the underlying cause
  and correcting it
  Usually related to malignant process
  and requires surgical approach




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THERAPEUTIC OPTIONS
 HIRSUTISM
GOAL:
 The prevention of further stimulation of
 hair growth
 Cosmetic correction of the problem



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THERAPEUTIC OPTIONS
BASIC STEPS OF MANAGEMENT OF
 HIRSUTISM ARE:
 DEFINE THE PROBLEM
 QUANTIFY THE DEGREE OF HIRSUTISM
 INDENTIFY THE PATHOPHYSIOLOGY
 CORRECT THE PROBLEM,WHETHER
 ACUTE OR CHRONIC
 DEFINE SUCESSWITH THE PATIENT
 FOLLOW UP

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THERAPEUTIC OPTIONS
A key element of any therapeutic plan is
to define what will ultimately be viewed
and successful therapy
Regular follow up is indicated at
appropriate intervals,usually every 3- 6
months



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THERAPEUTIC OPTIONS
GENERAL MEASURES :
  Eliminating causative factors
  Optimizing weight
  Manage hair
Bleaching
Cutting or shaving
Electrolysis
Laser epilation

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THERAPEUTIC OPTIONS
Management of excess ovarian androgen
  production :
Standard therapy is :combined E+P,most
  commonly OCs
  It reduces ovarian androgen production
  It increases SHBG
  It induces competition at the cellular level for
  binding to the androgen receptor

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THERAPEUTIC OPTIONS
Choice of OC
 EE + Norgestimarte approved in USA
 Cyproterone acetate used as progesterone
 component in Ocs
OVARIAN SUPPRESSION BY LONG ACTING
 GnRH ANALOGUE
 Can be used for functional ovarian androgen
 overproduction and even for malignant
 condition
 But to be used for long with back-up
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THERAPEUTIC OPTIONS
 Long acting GnRH analogues used
 But there is doubt that this therapy will
 be beneficial over Ocs
 INSULIN SENSITIZING AGENTS:
For PCO with acanthosis nigicans
Commonly used agent is : Metformin and
 Troglitazone,Pioglitazone,Rosiglitazone

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THERAPEUTIC OPTIONS
MANAGEMENT OF EXCESS
ADRENAL ANDROGEN PRODUCTION
Metabolic correction of the
disorder,usually with exogenous
steroids
Dexamethasone,mostly used,But
LIMITED ROLE

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THERAPEUTIC OPTIONS
Management directed to the target organ
 and cells
 Competition with Androgen
 receptors:Spironolactone,Flutamide,
 Ketoconazole,Cyproterone acetate
 5-alpha reductase Inhibitors :Finasteride



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THERAPEUTIC OPTIONS
  androgen receptors
     competitors
SIPRONOLACTONE:
  Best studied and as Gold standard
  Mechanism :Androgen receptors blockade
  Suppression of Androgen biosynthesis
  Increased metabolic clearance of teststerone
  ( Testosterone  Estrogen )
  50-200 mg/day in two divided doses
  Spironolactone + OC is well established
  regimen
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THERAPEUTIC OPTIONS
  androgen receptors
     competitors
FLUTAMIDE :
  Blocks the androgen receptors
  Decreases androgen production
  May have therapeutic value in cases of
  PCOS
  Usually used with Ocs
KETOCONAZOLE:
  Equally effective but danger of liver toxicity

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THERAPEUTIC OPTIONS
SELECTING BEST THERAPY:
Correct underlying medical problem
Correct thyroid/hyperprolactinemia
PCO :oral contraceptives
Ocs + spironolactone is usually the choice
75 –80% patients shows response
Atleast 6 months is needed for evidence of
response

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THERAPEUTIC OPTIONS
If response is seen in 6 months then
treatment should be continued for
further 6 months and in most cases for
number of years




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posted:4/12/2008
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