Somatoform Disorders

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General Info

• vellus hair- nonpigmented, soft covers nearly
  the entire body
• terminal hair- pigmented, thick mostly on the
  scalp, axilla, pubic area, extremeties, back
• Hirsutism- ? in terminal hair on the face, chest,
  back, lower abdomen, and inner thighs of a woman
• virilization- (L. virilis, masculine) development
  of male features including clitoromegaly, breast
  atrophy, male body habitus, hair loss may
  require higher level of androgens or in utero
  exposure

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General Info

• prevalence of hirsutism is 5-10 in women of
  reproductive age
• often present with baldness and acne as the
  presentation of a serious endocrinopathy

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Mechanism

• Pilosebaceous unit (PSU)
• Differentiates from vellus hair
  follicle-immature sebaceous gland ? terminal
  hair follicle-immature sebaceous gland or
  vellus hair follicle-mature sebaceous gland
  under presence of dihydrotestosterone (DHT)
  responsible
• DHT blood level not correlated with severity

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From UpToDate
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Non-neoplastic Causes

• ? adrenal synthesis of androgens
• zona fasciculata and reticularis (but not the
  glomerulosa, the outermost layer) of adrenal
  cortex have 17a-hydroxylase and reticularis makes
  DHEA/DHEAS testosterone
• Lab Tip DHEAS almost exclusive to adrenal gland
• ? ovarian synthesis of testosterone
• theca interna cells make testosterone (LH), then
  granulosa cells convert to estrogen (FSH)
• Cholesterol ? 1. desmolase, 2. 17a-hydroxylase
  3. C17-20 lyase ? DHEA ? 1. dehydrogenase, 2.
  isomerase ? testosterone ? aromatase ? estrogen

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Non-neoplastic Causes PCOS

• Polycystic ovarian syndrome is the 1 cause of
  hirsutism with prevalence of 4 in women of
  reproductive age
• Virilization, anovulation, amenorrhea, insulin
  resistance with hyperglycemia, and obesity may be
  present
• ? LH/FSH ratio (31 is diagnostic) theca
  interna hyperplasia
• Cysts or enlarged ovaries on pelvic exam
• Tx
• OCPs
• Glucophage (metformin)

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Other Non-neoplastic Causes

• CAH
• usually diagnosed childhood
• 21-OHase deficiency
• Most common CAH
• ? cortisol, aldosterone, BP
• ? sex hormones female pseudohermaphrodite,
  virilization
• stromal hyperthecosis
• common in women aged 50-70
• ovaries uniformly enlarged
• ? 5a-reductase
• Assay performed when androgens WNL

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Neoplastic Causes

• 70 of primary ovarian neoplasms are of the
  serous cystadenoma/cystadenocarcinoma type and
  rarely cause hirsutism
• functional ovarian tumors
• Sex-cord stromal are a heterogenous group of
  ovarian tumors
• Sertoli-Leydig cell, theca cell, granulosa cell
• rare
• usually benign
• may produce androgens or estrogens
• Luteoma of pregnancy
• Ovarian enlargement in presence of ß-hCG
• Androgen production possible

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Neoplastic Causes

• nonfunctional ovarian tumors
• dont secrete androgens they induce surrounding
  stroma to proliferate
• Krukenberg tumor
• Classically arises in stomach
• signet ring cell adenocarcinoma, BL ovarian mets
• cystadenoma rarely

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Other Causes of Hirsutism

• drug-induced
• minoxidil
• cyclosporin
• phenytoin
• idiopathic
• perhaps mild PCOS
• 2nd only to PCOS in prevalence

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Diagnosis

• HP
• rapidity of onset
• age
• virilization
• blood pressure
• severity
• pelvic
• Vaginal USG

• Labs
• BMP (or AccuCheck)
• 17-OH-pregnenolone
• free testosterone 2 ng/dL (0.07 nmol/L)
• dehydroepiandrosterone sulfate (DHEA-S) 700
  mcg/dL (13.6 µmol/L)

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General Treatment Considerations

• Best approach to hirsute patient is to exclude
  serious causes of hirsutism, then treat
  symptomatically
• Rx
• Spironolactone
• Anti-androgen at the DHT receptor
• Propecia
• Also useful for men with testosterone-induced
  hair loss
• Glucocorticoids
• (-) feedback on hypothalamus and anterior
  pituitary to decrease fasciculata (cortisol) and
  reticularis (sex hormones)
• Not ideal for long-term Rx