Conditions of the skin appendages
Terms Definitions
What hair is:
Lanugo hair
What replaces lanugo hair?
vellus hair on body
terminal hair on scalp
What condition results when lanugo hair is not replaced by vellus hair?
Congenital hypertrichosis lanuginosa
What areas of the body are not affected in congenital hypertrichosis lanuginosa?
palms, soles, dorsal surface of digits and prepuce
Name the following dz:
Paraneoplastic disease
Lung, colon, prostate cancer
Can precede the diagnosis of cancer
May be associated with other paraneoplastic etiologies
Burning tongue, acanthosis nigricans, PPK, sign of Leser-Trelat
Occurs over short period
Acquired hypertrichosis lanuginosa
What are cancers are assoc with hypertrichosis?
Lung, Colon, Prostate
(may precede dx of cancer)
What are some other paraneoplastic signs?
Burning tongue, acanthosis nigricans, PPK, sign of Leser Trelat
Name Four Hereditary d/o characterized by congenital generalized hypertrichosis?
Congenital hypertrichosis lanuginosa
Universal hypertrichosis
Ambras syndrome
X-linked hypertrichosis
Describe Congenital hypertrichosis lanuginosa
fine, downy, silvery-gray to blond lanugo hair
may shed over the first year of life
occasional dental anomalies
Describe Universal hypertrichosis
Thicker, longer hair most prominent on the frontal, temporal and preauricular areas of the face, the back and the proximal extremities
Increases during infancy and tends to persist
Describe Ambras syndrome
AD, Chr 8
Fine, silky long hair (ex., >10cm) uniformly distrib on the face (incl nose), ears and shoulders
Persists for life
Minor facial dysmorphism, dental anomalies, supernumerary nipples
Describe X-linked hypertrichosis
Curly, shorter, dark hair, most prominent on the face and upper body
Anteverted nostrils, prognathism, occasional dental anomalies, deafness
Most common drugs assoc with acquired generalized hypertrichosis
Name the localized hypertrichosis syndromes
Generalized Becker's Nevi syndrome is aka?
Michelin Tire Baby syndrome
What is SNUB Syndrome?
Supernumerary Nipples, Uropathies and Becker's Nevus
What other dermal tumors/lesions can have associated hypertrichosis?
Blue nevus (plaque type)
Fibrous hamartoma of infancy
Dermal dendrocyte hamartoma
Eccrine angiomatous hamartoma
Tufted angioma
Difference between Primary and Secondary Nevoid Hypertrichosis
Nevoid hypertrichosis: uncommon congenital alteration of terminal hair growth in circumscribed area

1. Primary (skin normal)
No extracutaneous findings
Can follows Blaschko’s lines

2. Secondary (associated with lipodystrophy, scoliosis, hemihypertrophy, abnormalities of underlying vasculature)
What is spinal dysraphism?
Cutaneous findings that 'mark' an underlying defect.... ex. 'faun tail' in the lumbosacral region as a marker for spina bifida occulta
What is another name for Vaniqa? How does it work?
Vaniqa is 13.9% eflornithine cream:
Enzyme inhibits orthinine decarboxylase involved in polyamine synthesis and, if inhibited, has been shown to affect cell division/synthetic functions
(affects rate of hair growth)
Works approx 1/3rd of the time
Go back to normal after cessation of therapy

of note: eflornithine is also used to treat sleeping sickness from trypanosome brucei gambiense
What is HAIRAN syndrome?
Insulin Resistance
Acanthosis Nigricans
What % of hirsute pts have SAHA?
What % of women have PCOS? Of those, what % are hirsuite?
5% of women in reproductive age have PCOS
90% are hirsute
What are the clinical findings in PCOS?
large cystic ovaries
secondary amenorrhea
mesnstrual alterations
What are some findings in:
Ovarian hyperthecosis
PCOS but with more testosterone
Normal LH, FSH
Elevated estrone
What are some findings in:
Ovearian hirsutism
Mild hirsutism with virilization in older, postmenopausal women
What are some findings in:
Pituitary hirsutism
ACTH and prolactin secretion (anterior pituitary)
Amenorrhea-galactorrhea syndrome, infertility
What are some findings in:
Iatrogenic hirsutism
Lateral aspects of face and back
Anabolic steroids (danazol)
OCP’s of the non-steroidal progestogen type
Resolution with drug cessation
If hair is found localized to the areola or lateral neck/face -- what hormonal source would you consider?
Suspect ovarian (vs adrenal)
If hair is increased centrally, what hormonal source would you consider?
Suspect adrenal
How does Cyproterone acetate work?
interferes with DHT binding to androgen receptor and by inhibiting FSH and LH secretion due to its progestogen action
How does spironolactone work?
anti-androgen activity, decreases levels of total testosterone
How does flutamide work?
pure non-steroidal antiandrogen, used to tx prostatic hyperplasia
How does finasteride (propecia) work?
inhibits 5-alpha-reductase isoenzyme 2, blocking conversion of testosterone to DHT
How does Drosperinone work?
17-alpha-spironolactone derivative with progestonic, anti-androgenic and anti-aldosterone activity
Name subtypes of Telogen Effluvium
Immediate anagen release
Delayed anagen release
Short anagen
Delayed telogen release
Immediate telogen release
Chronic telogen effluvium
Which HLA type is a susceptibility marker for all forms of AA? severe alopecia totalis/universalis?
1. HLA-DQ3
2. HLA DR4 & DQ7
Proposed Classification of Alopecia
Group 1: Lymphocytic
Group 2: Neutrophilic
Group 3: Mixed
Group 4: Other
Name the Lymphocytic Alopcias
Chronic cutaneous LE
Lichen planopilaris (Classic, Frontal fibrosing alopecia, Graham Little Syndrome)
Classic Pseudopelade (Brocq's alopecia)
Central centrifugal cicatricial alopecia
Alopecia mucinosa
Keratosis follicularis spinulosa decalvans
Name the Neutrophilic Alopecias
Folliculitis decalvans
Dissecting cellulitis
Name the Mixed Alopecias
Acne keloidalis
Acne necrotica
Erosive pustular dermatosis
what % with other findings of LP?
Condition more common in women
Keratosis follicularis spinulosa decalvans
Clinical: cictricial alopecia, follicular papules on face, trunk and extremities, atopy, PPK, photophobia, corneal abnmls
Hair abnmls assoc w/ increased fragility
Trichorrhexis invaginata (bamboo hair)
Pili torti
Trichorrhexis nodosa
Hair abnmls NOT assoc w/ increased hair fragility
Acquired progressive kinking of the hair
Loose anagen hair
Pili annulati and pseudo pili annulati
Pili bifurcati
Pili multigemini
Spun-glass hair (uncombable hair)
Woolly hair
What Dz assoc are seen with Pili Torti?
"B/C" My Cousin is a BRAT:
B-Bazek’s follicular atrophoderma
Longitudinal shaft splitting
aka 'split ends'
What hormone is linked with male-pattern baldness? Enzyme? Different forms of the enzyme? locations on the body?
Expression related to dihydrotestosterone (DHT)
5a-reductase converts testosterone to DHT
Both DHT & 5a-reductase increased
Type I: scalp follicle, liver, sebaceous gland
Type II: scalp follicle, liver, chest & beard hair, prostate
Genetic absence of Type II 5a-reductase is protective from the development of male androgenetic alopecia
Where on hair follicle are the 5a-reductase enzymes located?
Treatment options for male pattern alopecia?
Minoxidil 5% (Rogaine)
1 ml AAA bid
Finasteride (Propecia)
1mg po qd
Hamilton type III, IV
Stops hair loss in 90% men for at least 5 years
Regrows hair in 65% men
Continued use necessary to sustain regrowth & stopping medication associated with resumption of hair loss
Hair transplant
Bald men have higher risk of CAD, especially younger patients with early onset, severe disease
Classification for Male baldness? female baldness?
Male: Hamilton & Norwood
Female: Ludwig
Treatment for female pattern baldness?
Minoxidil 2-5% topical solution …plus
Iron supplement to maintain ferritin levels
Biotin supplementation…2.5mg/day
“Re-Activate” shampoo – proprietary name for my new ketoconazole otc shampoo product. $80/bottle.
Postmenopausal females: consider anti-androgen therapy…spironolactone 200mg/day. Or send to Tijuana for Diane-35.
What is Leukonychia
Normal nail plate surface with loss of transparency:
Distal nail matrix damage --> parakeratotic cells within ventral portion makes nail look white
What part of the matrix produces which part of the nail?
Proximal matrix --> dorsal nail plate
Distal Matrix (lunula) --> ventral nail plate
What is the growth rate of nails?
fingernails: 3mm / month
toenails: 1mm / month
What are some manifestations of damage to the proximal nail matrix?
Beau's lines
Longitudinal ridging
Longitudinal fissuring
What are some manifestations of damage to the distal nail matrix?
True Leukonychia
What are some manifestations of damage to both the proximal and distal nail matrix?
nail thinning
What are some manifestations of nail bed damage?
subungual hyperkeratosis
apparent leukonychia
splinter hemorrhages
What is onychomadesis?
Proximal detachment of the nail
What is onychorrhexis?
longitudinal ridging
longitudinal fissuring
-- diffuse nail matrix damage by LP
What is trachyonychia?
twenty nail dystrophy
nail roughness -- caused by proximal nail damage
What is true leukonychia?
distal nail matrix damage; white opaque discoloration
What is onycholysis? photoonycholysis?
detachment of the nail plate, usu due to psoriasis or onychomycosis; photoonycholysis -- occurs with TCN use, and sun exposure.
What is onychauxis?
nail thickening, subungual hyperkeratosis
Another name for Racquet thumbs
Brachyonychia (broad and short thumbnail)
Common extracutaneous problem for pts with nail-patella syndrome
40% have nephropathy leading to renal insufficiency in 8%
what is a nail finding seen with indinavir use?
paronychia with pyogenic granulomas (also seen with retinoids and EGFRI)
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