Download Diseases Of The Skin Appendages

Diseases of the Skin
Appendages
Alopecia areata
 Characterized by rapid and complete
loss of hair in one , or more often
several round or oval patches
 Usually on the scalp, bearded areas,
eyebrows and eyelashes
 1-5 cm
 May progress to total loss of scalp hair
– alopecia totalis, or hair loss involving
the entire body – alopecia universalis
Ophiasis/sisaipho
Alopecia areata
Alopecia totalis
 Usually occurs
without associated
disease
 However, there is a
higher incidence in
patients with atopic
dermatitis, Down
syndrome, LP, LE,
thyroiditis, MG and
vitiligo
 Nails may develop
uniform pits that
may form transverse
or longitudinal lines
– 10 %
etiology
 Cause unknown
 Most evidence points toward its being an
autoimmune disease mediated by the cellular
arm and modified by genetic factors
 25% report family history
 Patients with early onset, severe, familial
clustering alopecia areata have a unique and
highly significant association with HLA
antigens DR4, DR11, and DQ7
 histology
 In early disease there is a helper T-cell
lymphocytic infiltrate in the peribulbar area
of anagen or early catagen follicles
 DDX
 Tinea capitis, early lupus erythematosis,
syphilis, congenital triangular alopecia,
alopecia neoplastica and trichotillomania
TX
 Intralesional injections of corticosteroid
suspensions, q 6 weeks
 High-strength topical steroid creams
 Anthralin
 Minoxidil alone or combined therapy
 Spontaneous recovery is extremely common
 Tendency is for spontaneous recovery in
patients who are post pubertal at onset
 Predictors of poor prognosis include the
presence of atopic dermatitis, childhood onset,
widespread involvement, ophiasis, duration of
longer than 5 years, and onychodystrophy
Telogen effluvium
 Early and excessive loss of normal club
hairs from normal resting follicles in the
scalp
 Loss results from traumatization of the
normal hair by some stimulus, such as
surgery, parturition, fever, drugs, or
traction which precipitates the anagen
phase into catagen and telogen phases
 Follicle is not diseased and
inflammation is absent
Telogen effluvium
 “lots of hairs coming out by the roots”
 Loss is diffuse and only infrequently
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causing clinically perceptible thinning of
hair
Normal telogen count is below 10%
Can be estimated by the pull test
Grasping 40 hairs firmly between thumb
and forefinger, followed by a slow pull
Greater than 4-6 club hairs is abnormal
 100 – 150 hairs lost daily
 150 – 400 lost in telogen effluvium
Postpartum telogen effluvium
 Between 2-6 months postpartum
 Diffuse pattern, may be first noted over
anterior scalp
 May continue for another 2-6 months
Drug induced telogen effluvium
 Amphetamines, aminosalicyclic acid,
bromocriptine, captopril, coumarin,
carbamazepine, cimetidine, danazol,
enalapril, lithium carbonate, levodopa,
metyrapone, metoprolol, propranolol,
pyridostigimine and trimethadione
TX
 No specific therapy
 In the majority of cases it will stop
spontaneously within a few months and
the hair will regrow
 Chronic form may occur
 5% minoxidil solution
 education
Anagen effluvium
 Seen frequently following the administration of
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cancer chemotherapeutic agents, such as
antimetabolites, alkylating agents, and mitotic
inhibitors
Only anagen hairs are involved
With cessation of the drug the follicle resumes
normal activity within a few weeks
Process being entirely reversible
Topical minoxidil may decrease the period of
baldness by an average of 50 days
Anagen effluvium
Androgenetic alopecia
male-pattern baldness
 Shows itself during the twenties of early
thirties by gradual loss of hair, chiefly from
the vertex and frontotemporal regions
 Several patterns of this type occur
 Most frequent is the biparietal recession
with loss of hair on the vertex
 Rate varies
 The exact mechanisms responsible for
androgenetic alopecia are still unknown,
however, there is no doubt that inherited
factors and the effect of androgens on the
hair follicle are most responsible
 Pathogenesis involves lengthening of
telogen phase and shortening of the
anagen phase
TX
 Minoxidil
 2% and 5%
 Indefinite treatment to maintain a
response
 1/3 cases grow cosmetically useful hair
 Finasteride
 Effective in preventing further hair loss and
in increasing the hair counts
 Hair transplantation
Androgenetic alopecia in
women
 Generally diffuse hair loss throughout
the midscalp, sparing the frontal hairline
except for slight recession
 The midline part is an important clinical
clue to the diagnosis, revealing this
central thinning by the appearance of
the “Christmas tree pattern”
 The cause is now believed to be a
genetic predisposition in combination
with an excessive androgen response
TX
 Topical minoxidil 2%
Androgenetic alopecia in
women
Other forms of alopecia
trichotillomania
 A neurotic practice of plucking or breaking hair
from the scalp or eyelashes
 Usually localized but may be widespread
 Areas of alopecia characteristically contain hairs
of various lengths
 Seen mostly in girls under 10, may also be seen
in boys and adults
 It has been suggested that one ask the child not
if but rather how the removal is done
 Shave 3 X 3 cm area and watch the hair regrow
normally. Hairs in this ‘skin window” will be too
short for plucking
 Biopsy – high number of catagen hairs,
pigmentary defects and casts, trichomalacia and
heorrhage
trichotillomania
 May be a
manifestation of
obsessivecompulsive disorder
 May be assoc with
depression or
anxiety
 TX – psychotherapy,
behavioral therapy
or an appropriate
psychopharmacologi
c medication
 fluoxetine
trichotillomania
Hot comb alopecia
 Develops characteristically on the crown
and spreads peripherally to form a large
oval area of partial hair loss
 Initially reported in black women who
straightened their hair with hot combs
 The hot petrolatum used with the iron
causes thermal damage to the hair follicle
Pseudopelade of Brocq
(alopecia cicatrisata)
 A rare form of scarring alopecia
 Destruction of the hair follicle produces
multiple, round, oval, or irregularly shaped,
hairless, cicatricial patches of varying
sizes
 Coin sized and white or slightly pink
colored with a smooth, shiny, marble like
or ivory, atrophic, “onion skin” surface
 Inflammation is completely absent
 No pustules, crusts, or broken off hairs
 Insidious onset
 Permanent and slowly progressive
 Topical and intralesional steroids, longterm TCN in antiinflammatory doses- not
often successful
pseudopelade
Traction alopecia
 Occurs from prolonged tension on the hair
 Either from the hair tightly braided or in a
ponytail
Tractional alopecia
Pressure alopecia
Pressure alopecia
 Occurs frequently on the occipital areas of
babies lying on their backs
 In adults it is seen most often after
prolonged pressure on the scalp during
general anesthesia, with the head fixed in
one position
 Most likely due to pressure induced
ischemia
Loose anagen syndrome
Loose anagen syndrome
 Disorder in which anagen hairs may be
pulled from the scalp with little effort
 Occurs mostly in blonde girls
 Improves with age
Alopecia syphilitica
 May have a typical moth-eaten
appearance on the occipital scalp, a
generalized thinning, or a combination of
the two
 Eyebrows and lashes, and other body hair
may be involved
 May be the first sign of a syphilis infection
Alopecia syphilitica
Alopecia syphilitica
Follicular mucinosis
(alopecia mucinosa)
 Most commonly occurs on the scalp or beard
area
 Manifest as deposition of mucin in the outer
root sheath and sebaceous glands
 Inflammatory reaction produces alopecia, and
at times hypopigmentation
 Primary cases occur either as localized
lesions of the head or neck typically resloving
within a year
Follicular mucinosis
(alopecia mucinosa)
 More generalized lesion have a longer
course
 Young people are primarily affected
 A secondary type – associated with
cutaneous T-cell lymphoma, usually more
widespread and in older persons
Follicular mucinosis
Inflammatory alopecia
 May be seen in lichen simplex chronicus
and various eczematous changes on the
scalp, including kerion
 DLE, lichen planopilaris, sarcoidosis, and
folliculitis decalvans are the commonest
inflammatory causes of cicatricial alopecia
Vascular or neurologic alopecia
 Most often of the lower extremities
 May be seen in diabetes mellitus or
atherosclerosis
Endocrinologic alopecia
 May occur in various endocrinologic
disorders
 Hypothyroidism – hair becomes dry,
brittle, coarse and sparse
 Hyperthyroidism – hair becomes
extremely fine and sparse
 Some women develop telogen effluvium
2-4 months after discontinuing
anovulatory agents
Tumor alopecia
 Refers to hair loss in the immediate
vicinity of either benign or malignant
tumors of the scalp
 Syringomas, nerve sheath myxomas,
and steatocytoma multiplex
 Alopecia neoplastica – hair loss from
metastatic tumors, most commonly
breast carcinoma
Congenital alopecia
 Occurs either as total or partial hair loss, or
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lack of initial growth
Accompanied usually by other ectodermal
defects of the nails, teeth and bone
Hair is light and sparse, and grows slowly
Congenital triangular alopecia and aplasia
cutis congenita are examples of congenital
localized absence of hair
Hidrotic ectodermal dysplasia - diffuse
Syndromes that include
abnormalities of the hair
Albright’s disease
(polyostotic fibrous dysplasia)
 May present as slowly progressive lifelong
unilateral hair loss
 Scalp, pubic, axillary, and palpebral
 Sickle cell disease is often characterized
by scantiness of body and facial hair
 Cronkhite-Canada syndrome
characterized by alopecia, skin
pigmentation, onychodystrophy,
malabsorption, and generalized
gastrointestinal polyposis
Marinesco-Sjorgren syndrome
 Cerebellar ataxia, mental retardation,
congenital cataracts, inability to chew
food, thin brittle fingernails, and sparse
hair
 Autosomal recessive inheritance
trichothiodystrophy
 Brittle hair with
markedly reduced
sulfur content
 Sulfur reduced 50%
of normal value
 Distinctive features
under polarized light
and scanning electron
microscopy
trichothiodystrophy
 With light microscopy
trichoschisis (clean
fractures) may be
seen
 With polarized
microscopy the hair
shows alternating
bright and dark
regions
trichothiodystrophy
trichothiodystrophy
 Hair findings present in all cases
 Other variable features include short
stature, mental deficiency, ichthyosis, nail
dystrophy, ocular dysplasia,
photosensitivity and infertility
 Various names associated with different
combinations Brown’s syndrome, BIDS,
IBIDS, PIBIDS and Tay’s syndrome
trichothiodystrophy
 Different mutations in the ERCC2 DNA
repair gene may lead to different
phenotypic expressions of the disease
Generalized hair follicle
hamartoma syndrome
 Generalized trichoepitheliomas, alopecia
and myasthenia gravis
 Localized case report
POEMS syndrome
(Crow-Fukase syndrome)
 Polyneuropathy
 Organomegaly
 Endocrinopathy
 M-protein
 Skin changes
 Diffuse hyperpigmentation, dependent
edema, skin thickening, hyperhidrosis
and hypertrichosis
POEMS syndrome
(Crow-Fukase syndrome)
POEMS
syndrome
Cartilage-hair hypoplasia
 Short limbed dwarfism and abnormally fine
and sparse hair in children
 Especially susceptible to viral infections
and recurrent respiratory infections
AIDS patients
 Many black patient with AIDS have
experienced softening, straightening,
lightening and thinning of their hair
 HIV patients may also experience
elongated eyelashes and telogen effluvium
Tricho-rhino-phalangeal
syndrome
 Genetic disorder consisting of fine and
sparse scalp hair, thin nails, pear-shaped
broad nose, and cone shaped epiphyses
of the middle phalanges of some fingers
and toes
 Autosomal dominant and recessive types
Lipedematous alopecia
 Consists of shortened hairs, with
thickening of the scalp associated with an
increase in subcutaneous fat
 Scalp may be as much as 15 mm thick
 Primarily affects persons of color
Hallerman-Streiff syndrome
 Rare condition
 Bird-like facies with a pronounced
beaklike nose, microphthalmia,
micrognathia, congenital cataracts, and
hypotrichosis
 Hair is diffusely sparse and brittle
 Sutural alopecia is characteristic of this
syndrome – hair loss following the lines
of the cranial sutures
Hallerman-Streiff syndrome
 Small face with
disproportionately
large head
Progeria
(Hutchinson-Gilford syndrome)
 Characterized by the appearance of
premature aging
 Marked by failure to develop normally in
growth after the first year of life
 Large bald head and lack of eyebrows and
eyelashes are distinctive
 Skin is wrinkled, pigmented and atrophic
Progeria
(Hutchinson-Gilford syndrome)
 Nails are thin and atrophic
 Most patient lack subcutaneous fat
 Intelligence remains intact
 Arteriosclerosis, anginal attacks, and
hemiplegia may occur, followed by death
from coronary heart disease at an early
age
Progeria
(Hutchinson-Gilford syndrome)
Papillon-Lefevre syndrome
 Hyperkeratosis palmaris et plantaris,
periodontosis, and sparsity of the hair
 Hyperhidrosis and other signs and
symptoms begin early in life
 Autosomal recessive
Klippel-Feil syndrome
 Consists of low posterior scalp hairline
extending onto the shoulders, with a
short neck, limiting movement of the
neck, and suggestive of webbing
 Cervical vertebrae are fused
 Strabismus, nystagmus, cleft palate,
bifid uvula, and high palate are other
features
 Occurs mostly in girls
Klippel-Feil syndrome
McCusick’s syndrome
 Includes short-limbed dwarfism and fine,
sparse, hypoplastic and dysmorphic hair
Turner’s syndrome
 Short stature, webbing of the neck, low
posterior hairline margin, increased
carrying angle of the elbows (cubitus
valgus), and infantile development of the
breasts, vagina and uterus
 Coarctation of the aorta is frequent
 Alopecia of the frontal scalp and cutis laxa
Turner’s syndrome
 Caused by ovarian dysgenesis
 Only 45 chromosomes present
 Sex chromosomes have an XO pattern
Noonan’s syndrome
 Short stature with atypical webbing of the neck,
low hairline in the back, prominent and low-set
ears, and cubitus valgus.
 Similar to Turner’s and frequently termed male
Turner’s syndrome
 Absence of coarctation of the aorta
 Valvular pulmonary stenosis is the typical heart
lesion
Noonan’s syndrome
Werner’s syndrome
 Shortness of stature, cataracts, skin changes,
premature graying and alopecia, atrophy of
the muscles and subcutaneous tissue, and
bone atrophy of the extremities to produce
spindly extremities
 Skin changes include poikiloderma,
scleroderma, atrophy, hyperkeratosis and leg
ulcers
 Skin has a dark gray or blackish diffuse
pigmentation
Werner’s syndrome
 A high-pitched voice and hypogonadism in
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both sexes are distinctive in this syndrome
Diabetes mellitus is frequently present
Because most of the signs are not fully
manifested before the age of 30, the
diagnosis is usually made in middle age
Autosomal recessive
DNA helicase mutation
Patients usually die before they are 50 from
malignant disease or vascular accident
Werner’s syndrome
Rothmund-Thomson syndrome
 Characterized by early onset poikiloderma,
short stature, sun sensitivity, bone defects,
and hypogonadism
 Sparseness of eyelashes, eyebrows, or
scalp hair has been reported in 60% of
cases
 Mutation in a DNA helicase gene
Rothmund-Thomson syndrome
Rothmund-Thomson
syndrome
HAIR COLOR
 Black and brown hair – eumalanin
 Blonde and red hair – pheomelanin
 Changes in hair color occur in various
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metabolic disorders
Phenylketonuria hair becomes blond
Bleaching effect in homocystinuria
Oasthouse disease
albinism
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Triparanol assoc. with hypopigmented hair
Minoxidil causes darkening
Diazoxide gives reddish tint
Chloroquine therapy may cause whitening
Kwashiorkor red-blonde color in infants
B12 deficiency and interferon therapy may
cause whitening
 Canities segmentata sideropenica –
alternating light and dark bands seen with
iron-deficiency anemia
 Gray hair (canities) melanogenic activity
is decreased as a result of fewer
melanocytes and melanosomes as well
as gradual loss of tyrosinease activity
 Genetically determined and may start at
any age
 Early graying (before age 20 in whites
30 in blacks) usually familial, may occur
in progeria, Rothmund-Thomson
syndrome, Book’s syndrome, and
Werner’s syndrome
poliosis
 Gray hair occurring in circumscribed patches
 Waardenburg’s syndrome, piebaldism, and
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tuberous sclerosis
Also assoc with vitiligo and Vogt-Koyanagi
syndrome
May be seen in alopecia areata with new hair
growth
Tietze’s syndrome, Alezzandrini’s syndrome,
and NF
May signal a regressing area of a scalp
melanoma
poliosis in vitiligo
poliosis
 Green hair has been trace to copper in the
water of swimming pools
 Occurs only in blonde and light hair
 Premature whitening of the scalp hair is
usually caused by vitiligo, sometimes
without recognized, or actually without,
lesions of glabrous skin
HAIR STRUCTURE
DEFECTS
Hair casts
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Mimics nits closely
Affects women chiefly
Results from long term and frequent tractions
Many scalp hairs bear a white keratinous
sleeve about 3-5 mm long, which lies within
1-3 cm of the scalp surface
 Unlike a nit can be slid along the hair shaft
 Their bluish yellow fluorescence under
Wood’s light may cause them to be confused
with tinea capitis
Hair casts
 Formed by retention and desquamation of
segments of the root sheaths
 0.025% tretinoin lotion has been found to
be effective
Pili torti
(twisted hairs)
 A malformation of hair characterized by
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twisting of the hair shaft on its own axis
The hair shaft is segmentally thickened
and light and dark segments are seen
Scalp, eyebrows, and eyelashes
Hairs are brittle and easily broken
Typically occurs in childhood and improves
by puberty
Pili torti
(twisted hairs)
 May be associated with patchy alopecia
 Follows a dominant inheritance pattern
 May be assoc with Bjornstad’s syndrome,
citrullinemia, Menkes’ kinky hair syndrome,
Bazex’s follicular atrophoderma syndrome
trichothiodystrophy
 Assoc with etretinate and isotretinoin
Pili torti
Pili torti
Pili torti
(twisted hairs)
Menkes’ Kinky Hair Syndrome
(steely hair disease)
 Patients have deficiency of serum copper
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and copper-dependent enzymes
Pili torti, and often monilethrix and
trichorrhexis nodosa are common
Sex-linked recessive disorder
Seen only in boys
Characteristic ivory color of the hair
appears between 1 and 5 months
Menkes’ Kinky Hair Syndrome
(steely hair disease)
 Drowsiness, lethargy, convulsive seizures, and
severe neurologic deterioration, with periodic
hypothermia ensue
 Hairs become wiry, sparse, fragile, and twisted
about their long axis
 Skin is pale and the face is pudgy
 Upper lip has an exaggerated “cupid’s bow”
Menkes’ Kinky Hair Syndrome
(steely hair disease)
Menkes’ Kinky Hair Syndrome
(steely hair disease)
 A distinctive neurochemical pattern in the
plasma allows for early diagnosis
 Institution of copper histidine treatment
has shown promising results in some
infants
 The occipital horn syndrome is a milder
variant
Uncombable
hair syndrome
Uncombable hair syndrome
(pili triangulati et canaliculi)
 Noted in the first years of life as dry, blonde,
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shiny hair that stands straight out from the scalp
and cannot be combed
Longitudinal grooves make hair rigid
Autosomal dominant inheritance
Sporadic cases reported
Some cases improve spontaneously in
childhood
Uncombable
hair syndrome
Monilethrix
(beaded hairs)
 Characterized by dryness, fragility, and
sparseness of the scalp hair, with fusiform or
spindle-shaped swellings of the hair shaft
separated by narrow atrophic segments
 Hair tends to break at the delicate internodes
 Involvement of the hair may occur during
pregnancy and resolve after delivery
Monilethrix
(beaded hairs)
 Improvement may occur with age
 May be seasonal improvement during the
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summer
Autosomal dominant
Has been described in assoc with Menkes’
Disease of the hair cortex
No effective treatment
Monilethrix
(beaded hairs)
Monilethrix
(beaded hairs)
Trichorrhexis nodosa
 Affected hair shafts may have small white
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nodes arranged at irregular intervals
The nodes are the sites of fracture of the hair
cortex
Hairs soon break at the nodes
Found mostly on the scalp
Often in a small area or areas
Pubic area, axillae and chest may be involved
Trichorrhexis nodosa
 Has been described in assoc with
arginosuccinicaciduria, Menkes’ kinky hair
syndrome, Netherton’s syndrome,
hypothyroidism
Trichorrhexis nodosa
 Proximal
 Distal (split ends)
 Acquired localized
 Treatment
 Avoid traumatization
to the hair
Trichorrhexis
nodosa
Trichorrhexis invaginata
(bamboo hair)
 Caused by intussusception of the hair shaft at
the zone where keratinization begins
 The invagination is caused by the softness of
the cortex in the keratogenous zone
 patient has nodosa ball and socket
deformities
 Associated with Netherton’s syndrome with
ichthyosiform erythroderma or ichthyosis
linearis circumflexa
Trichorrhexis invaginata
Trichorrhexis invaginata
(bamboo hair)
Trichorrhexis invaginata
(bamboo hair)
 Occ only the proximal half of the abnormality
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is seen – golf tee hairs
Seen on scalp
Also eyebrows, eyelashes, and rarely in other
hairy areas
Hair sparsity is noted all over the body
Bamboo hairs may become normal in a few
years
Autosomal recessive mode of inheritance
Netherton’s syndrome
Netherton’s
syndrome
Pili annulati
(Ringed Hair)
 The hair seems banded by alternating
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segments of light and dark color when seen
in reflected light
Light bands are caused by clusters of
abnormal air-filled cavities which scatter light
Hair growth is normal
No other assoc abnormalities
Autosomal dominant
Begins in infancy and requires no treatment
Pili annulati
(Ringed Hair)
Pili pseudoannulati
 Mimics pili annulati
 Bright segments are caused by reflection
and refraction of light by flattened, twisted
surfaces of hair
 Variant of normal hair
Kinked hair
 Acquired progressive kinking of the hair
 Has a structural abnormality of kinking
and twisting of the hair shaft at irregular
intervals
 Begins in men in the late teens and
early twenties
 Begins on the frontotemporal or vertex
regions and progresses to both the
parietal and frontal areas
Kinked hair
 Usually straight, light brown hair becomes
curly, frizzy, and lusterless
 May be precursor to male-pattern hair loss
 May be induced by drugs, notably
retinoids
 May occur in AIDS patients
Woolly hair
 Present at birth and usually most severe
during childhood
 Variable amelioration in adulthood
 Four subgroups
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Hereditary woolly hair (AD)
Familial woolly hair (AR)
Woolly hair nevus
Naxos disease
Woolly hair
 Tend to unite into
locks
 No assoc cutaneous
or systemic diseases
Woolly hair
Woolly hair
NOT
Plica neuropathica
 Curling, looping, intertwisting, and felting
or matting of the hair in localized areas of
the scalp
 Predisposing factors include kinky hairs
and neurotic mental state
Pseudofolliculitis barbae
 Hairs that appear at the surface and curve
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back and pierce the skin as ingrowing hairs
Results in inflammatory papules and pustules
May scar
Chief cause is close shaving of curly hair
Seen in more than 50% of black men
Whites uncommonly affected
Pseudofolliculitis barbae
Pseudofolliculitis barbae
 Treatment
 Laser hair removal
 stop shaving
 Deforming keloids may result
Pili multigemini
 Rare malformation of the pilary apparatus
 Characterized by the presence of
bifurcated or multiple divided hair matrices
and papillae, giving rise to the formation of
multiple hair shafts within the individual
follicles
 No treatment
Pili bifurcati
 Described in a 3 year old seen for hair loss
 Bifurcation was found in short segments
along the shafts of several hairs
 Anomaly was transient
Trichostasis spinulosa
 A common disorder of the hair follicles that
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clinically gives the impression of follicular
keratosis
Follicles are filled with funnel-shaped horny
plugs within which are bundles of vellus hairs
Hairs are round at their proximal ends and
are shredded distally
Occurs on the nose and forehead of elderly
Shoulders and back
Trichostasis spinulosa
 Results from retention of telogen hairs
 Treatment with keratolytics after using a
wax depilatory
 Tretinoin 0.05% solution
Trichostasis spinulosa
Trichostasis spinulosa
Trichostasis spinulosa
the removed plug
 Microscopically the
tufts are made up of
several dozen fine,
stubbly hairs
Intermittent Hair-Follicle
Dystrophy
 New disorder of the hair follicle leading to
increased fragility of the shaft, with no
identifiable biochemical disturbance
Bubble Hair Deformity
 Reported in a 16 year old girl
 Developed brittle, fragile hairs in localized
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areas of he scalp
Hairs became straight and stiff
Small bubble-like defects were found within
the hairs shafts on light and electron
microscopy
Proved to be caused by heating of the hair
All hair will develop bubbles of gas when
exposed to heat
Hypertrichosis
 An overgrowth of hair not localized to the
androgen-dependent areas of the skin.
 Several forms exist
Localized acquired
hypertrichosis
 Dermal tumors, such as melanocytic nevi,
smooth muscle hamartoma, meningioma
or Becker’s nevi may have excessive
terminal hair growth
 PCT and Variegate porphyria
 May be more generalized in the Gunther
variety of erythropoietic porphyria
Becker’s
nevus
Becker’s nevus
PCT
Localized congenital
hypertrichosis
 Progressive, excessive growth of
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lanugo hairs that often begins in infancy
Hairs may reach a length of 10 cm
May become coarse
Regression in adolescence has been
observed
Familial cases and sporadic
Appears to be only of cosmetic
significance
Localized congenital
hypertrichosis
 Other causes include congenital nevocytic
nevi, anterior cervical hypertrichosis,
simple nevoid hypertrichosis, or as a sign
of underlying spinal dysraphism (when
occurring over the sacral midline
Generalized Congenital Hypertrichosis
(congenital hypertrichosis lanuginosa)
 Rare type of excessive and generalized
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hairiness
Fully penetrant X-linked dominant trait
Entire body covered with fine vellus hairs 2-10
cm long, spares palms and soles
Scalp hair is normal
May be assoc with dental anomalies and
gingival fibromatosis
“dog-faced boy” “human werewolf”
“human Skye terrier”
Generalized Congenital Hypertrichosis
(congenital hypertrichosis lanuginosa)
 Other cases may be secondary to drug
ingestion by the mother
 Fetal hydantoin syndrome
 Fetal alcohol syndrome
 Minoxidil – case of generalized
hypertrichosis and multiple congenital
defects in a baby born to a mother who
used minoxidil throughout pregnancy
Generalized or patterned
acquired hypertrichosis
 These cases include those caused by
acquired hypertrichosis lanuginosa, those
associated with various syndromes, and
those secondary to drug intake
 Also an ominous sign of internal
malignancy
Syndromes
 Lipoatrophic diabetes, stiff skin syndrome,
Rubenstein-Taybi syndrome, Laband
syndrome, Cornelia de Lange’s syndrome,
Hurler’s syndrome, Morogu’s syndrome,
leprechaunism, , Winchester’s syndrome,
the Schynzel-Giedier syndrome
Brachmannde Lange syndrome
Hunter syndrome
drugs
 Minoxidil, cyclosporine, diphenylhydantoin,
diazoxide, streptomycin, penicillamine,
corticosteroids, danazol, psoralens,
hexachlorobenzene, PUVA, topical
steroids and topical androgens
cyclosporine
minoxidil
Hirsutism
 An excess of terminal hair growth in women
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in a pattern more typical of men
Androgen-dependent growth areas affected
include the upper lip, cheeks, chin, central
chest, breasts, lower abdomen and groin
May or may not be assoc with other signs of
virilization
Acne may be seen
When virilization accompanies hirsutism,
especially when progression is rapid, a
neoplastic cause is likely
Hirsutism
pathogenesis
 Racial variation
 May result either from excessive of
androgens from either the ovary or the
adrenal gland, or from excessive
stimulation by pituitary tumors
 All cases of progressive or severe
hirsutism should be investigated for an
endocrinopathy
Hirsutism
pathogenesis
 Ovarian causes include polycystic ovarian
disease and a variety of ovarian tumors,
both benign and malignant
 Stein-Leventhal syndrome characterized y
hirsutism 50%
 Serum free testosterone is generally
elevated as is luteinizing hormone
Hirsutism
pathogenesis
 Adrenal causes include congenital adrenal
hyperplasia and adrenal tumors such as
adrenal adenomas and carcinomas
 Adrenogenital syndrome is an autosomal
dominant disorder resulting from deficiencies
in the following enzymes:
 21-hydroxylase
 11B-hydroxylase
 3B-hydroxy steroid dehydrogenase
Hirsutism
pathogenesis
 Pituitary causes include Cushing’s
disease, acromegaly, and prolactinsecreting adenomas
 Other conditions in which prolactin levels
may be elevated and that may lead to
hirsutism include hypothyroidism,
phenothiazine intake, and hepatorenal
failure
Hirsutism
pathogenesis
 Other causes include the exogenous
intake of androgens and certain highprogesterone bcp (uncommonly)
 Minoxidil, diazoxide, corticosteroids and
phenytoin, which have been reported to
cause hirsutism, generally cause
hypertrichosis
Hirsutism
evaluation
 History should focus on onset and
progression, virilization, menstrual
history, and family/racial background
 Laboratory evaluation should include a
total testosterone and a
dehydroepiandrosterone sulfate level for
relatively stable and mild hirsutism
 Dexamethasone suppression test to
screen for Cushing’s disease
Hirsutism
evaluation
 In patients with menstrual dysfunction add
prolactin level and an LH/FSH ratio to
evaluate suspected polycystic ovarian
disease
diagnosis
 Refer to endocrinologist
 Refer to gynecologist
 Major elevation in the DHEA sulfate level
(above 7000 ng/ml) suggests an adrenal
neoplasm CT is recommended
treatment
 Once appropriate testing has led to diagnosis
and referral of patients requiring special
methods of specific treatment, such as
surgical intervention, therapeutic alternatives
including cosmetic treatments, nonspecific
suppressive therapy, and specific
antiandrogens
 Shaving, wax depilatories, chemical
depilatories, bleaching of the hair, laser hair
removal, and electrolysis
treatment
 Oral contraceptives and glucocorticoids
 bcp helpful in 75% of hirsute women
 Antiandrogens include, cimetidine,
cyproterone acetate, spironolactone,
flutamide, and ketoconazole
 Finasteride
 Gonadotropin-releasing hormone agonist
such as leuprolide and nafarelin
Trichomycosis Axillaris
 Characterized by 1-2 mm nodules of different
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colors occurring on the affected hair shafts in the
axillary or pubic areas
Attached firmly to the hair shaft
Color may vary from yellow, red or black
Hyperhidrosis is usually present
Large number of corynebacterium are present in
the concretions
Trichomycosis Axillaris
 Topical antibiotic preparations
 Treat hyperhidrosis
 shaving
Trichomycosis Axillaris