Transcript Andrew’s Chapter 33

Andrew’s Chapter 33-Part II

JoAnne M.LaRow,
D.O.
Folliculitis Decalvans
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An inflammatory
reaction of the hair
follicles
Leads to cicatricial
alopecia
Small pustules surround
the follicles
Erythema, scaling, and
smooth shiny depressed
scars are apparent
Folliculitis Decalvans
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Pseudopelade
When the pustules have
healed and scarring
remains –pseudopelade
occurs
Note intact follicles and
single hairs growing
May occur on axillae
and groin as well
Folliculitis Decalvans
Etiology is unknown
 Scarring alopecia in a
middle-aged man,
associated with a
hyperkeratotic scalecrust with follicular
hyperkeratosis and
erythema
 If gram stain and
culture of the exudate
reveals coagulasepositive staphylococci,
this should be treated
 Many times no
organism is cultured

Folliculitis Decalvans
* Early lesions show
perifollicular
inflammation composed
of acute and chronic
inflammatory cells
Folliculitis Decalvans

Later lesions shows
destruction of
follicular epithelium,
dense interstitial
inflammation, and
perifollicular fibrosis,
with free hairs in the
tissue
Folliculitis Decalvans

There is perifollicular
and interstitial
fibrosis accompanied
by an interstitial
infiltrate of
lymphocytes and
plasma cells
Folliculitis Decalvans
The eventual loss of
follicular epithelium
leads to a
granulomatous
response triggered
by free hair shafts
Folliculitis Decalvans
TREATMENT:
 Cephalosporins, dicloxacillin, and azithromycin
and rifampin may be added to therapy for better
long-term control
 Oral zinc or vitamin C supplementation may
enhance response
 Chronic inflammation reactions may be helped
with topical steroids and by intralesional
triamcinolone

Tinea Amiantacea
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Thick, asbestos-like
(amiantaceous), shiny
scales attached to the
lower part of the hair
shaft, rather like tiles
overlapping on a roof
Crusting may be
localized or, less
commonly generalized
over the entire scalp
There are no structural
changes in the hair, but
in some patches where
the crusting is thick,
there may be purulent
exudate under the crust
and temporary alopecia
may occur
Tinea Amiantacea
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Etiology is likely
secondary to an
infection occurring in
seborrheic dermatitis or
inverse psoriasis
Treatment should be
shampoo daily or every
other day with selenium
sulfide susupension, or
a tar shampoo , for a
few weeks
Prior application of
Baker’s P&S liquid is
helpful to remove scale
and crust
Derma-Smoothe and FS
shampoo are also
effective
Keratosis Follicularis
Contagiosa
Also known as
epidemic acne,
epidemic follicular
eruption, epidemic
follicular keratosis,
and Brooke’s disease
 Unknown etiology
 Occurs in children

Keratosis Follicularis
Contagiosa
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Eruption is widespread
and symmetrical,
affecting chiefly the
back of the neck, the
shoulders, and the
extensor surfaces of the
extremities
Onset is acute, may
affect large numbers of
patients in a localized
geographic area , and
spontaneously involutes
over a 3-to-6-week
period
Keratosis Follicularis
Contagiosa
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There is a horny
thickening of these
areas, especially
pronounced about the
follicles, where small
black corneous may be
discerned
Etiology has been
hypothesized to be
infectious- but not
proven
Folliculitis Nares Perforans
Charcterized by small pustules near the tip of
the inside of the nose
 The lesion becomes crusted, and when the
crust is removed it is found that the bulbous
end of the affected vibrissa is embedded in
the inspissated material
 Staphylococccus aureus may at times be
cultured from the pustules
 Treatment is removal of the hair and topical
ointment like mupirocin
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Perforating Folliculitis
Charcterized by an
asymptomatic eruption
of erythematous
follicular papules 2 to 8
mm diameter
Involving the extenxor
ssurfaces of the upper
arms, the buttockss, or
the upper thighs
When small, whitish
kerratotic plug is
removed from the
follicular papule, a small
bleeding crater remains
Perforating Folliculitis
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A dilated follicular unit
contains a keratotic
plug with an admixture
of basophilic debris.
The follicular epithelium
is perforated, and there
are degenerated
collagen fibers in the
adjacent dermis.
Perforating Folliculitis
Resistant to
treatment
 Topical tretinoin is
reported to be
effective
 One report of an
HIV-infected man
who responded well
to thalidomide
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Kyrle’s Disease
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Originally termed
hyperkeratosis
follicularis et
paarafollicularis in
cutem penetrans
Rare disorder
Characterized by
hyperkeraatosis,
forming a horny cone
that projects into the
dermis, so that when it
is removed a pitlike
depression remains
Usually discrete
papules, but may
ccoalesce to form
circinate plaques
Kyrle’s Disease
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There is a prediliction
for the lower
extremities, but the
upper extremities,
head, and neck may be
involved
Koebner’s phenomenon
may also be observed,
in which elevated
verrucous streaks or
plaques are formed
The elevated verrucous
streaks are only seen in
the antecubital and
popliteal spaces
Kyrle’s Disease
Occurs almost
exclusively in adults
ages 20 to 63
 No sex or racial
differences seen
 Has been associated
with diabetes
mellitus
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Kyrle’s Disease
Histologically:
 Large keratotic and
parakeratotic plugs
penetrating through the
epidermis into the
dermis
 These plugs cause an
inflammatory an
foreign-body giant cell
reaction about the
lower end of the plug in
the dermis
 Mild degenerative
changes in the
connective tissue with
no increase in the
elastic tissue also occur
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Kyrle’s Disease
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Kyrle’s disease remains
stable for years, with
possible clearing when
the associated illness is
controlled
Ultraviolet treatment,
methotrexate, topical
corticosteroids, 5fluorouracil, and
keratolytics are usually
ineffective
Topical retinoic acid
0.01% cream,
isotretinoin, and
etretinate have been
effective in flattening
the lesions
Reactive Perforating
Collagenosis
 Pinhead-sized, skin-
colored papules that
grow to 4 to 6 mm and
develop a central area
of umbilication in which
keratinous material is
lodged
 The discrete papules
may be numerous and
involve sites of frequent
trama
 The lesions reach a
maximum size in 4
weeks the slowly
regress
Reactive Perforating
Collagenosis
A cup-shaped channel
containing
degereated collagen
bundles and
basophilic material
Reactive Perforating
Collagenosis
Trichrome stain :
Blue-stained collagen
fibers perforating
the channel and
extending to the
surface
Reactive Perforating
Collagenosis
It is believed that this is
caused by a peculiar
reaction of the skin to
superficial trauma
Koebnerization is often
observed
Young children are most
frequently affected
There is no specific
treatment indicated,
since the lesions
involute spontaneously
Perforating Disease of
Hemodialysis
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It is reported that
between 4% and 10%
of dialysis patients
develop perforating
disorders
Lesions are
characterized by domeshaped papules on the
legs, or less often on
the trunk, neck, arms,
or scalp, with variable
itchiness
Early lesions may be
pustular; late lesions
resemble prurigo
nodularis
Perforating Disease of
Hemodialysis
The disease may remit
promptly after a renal
transplant and stopping
dialysis
 Topical tretinoin may be
tried

Traumatic Anserine Folliculosis
A curious gooseflesh-like follicular hyperkeratosis
that may result from persistent pressure and
lateral friction of one skin surface on another
Such friction is often caused by habitual pressure
of elbows, chin or jaw, or neck, often while
watching television
Two thirds of patients who develop this are atopic
Erythromelanosis Follicularis
Faciei et Colli
A unique erythematous pigmentary disease involving the follicles
A reddish brown, sharply demarcated, symmetrical discoloration
involving the preauricular and maxillary regions
Pigmentation may be blotchy
Follicular papules and erythema are present
Pityriasiform scaling and slight itching may occur
Keratosis pilaris is frequently found on the arms and shoulders
It preferentially affects Asian patients
Disseminate and Recurrent
Infundibulofolliculitis
A mildly pruritic eruption at times,
and is chronic with recurrent
exacerbations
Characterized by uniform papules,
1 or 2 mm in diameter, and
involving all the follicles in the
affected areas, which are usually
the upper trunk and neck
Histologically, the infundibular
portion of the follicles is chiefly
affected, and the lesions are
inflammatory rather than
hyperkeratotic
Disseminate and Recurrent
Infundibulofolliculitis
Edema, lymphocytic and neutrophilic infiltration, and
slight fibroblastic infiltration surround the affected
follicles
Treatment with vitamin A has been reported to be
effective
Isotretinoin or etretinate may also be useful
Lichen Spinulosus
Disease frequently seen in
children
Characterized by minute
filiform horny spines
protruding from follicular
openings independent of
any papule
The spines are discrete
and grouped
Lichen Spinulosus
The lesions appear in crops
and are symmetrically
distributed over the trunk,
limbs, and buttocks
There is a predilection for the
neck, buttocks, abdominal wall,
popliteal fossa, extensor
surfaces of the arms
A generalized distribution has
been reported to occur with
HIV infection
Lichen Spinulosus
Lichen Spinulosus
Histology:
Shows simple inflammatory changes and follicular
hyperkeratosis
Treatment:
The lesions respond to mild keratolytics like 3%
resorcin or salicylic acid ointment, Keralyt gel, LacHydrin lotion, and tretinoin
Hyperhidrosis
Palmoplantar
Hyperhidrosis(Emotional
Hyperhidrosis)
* Usually localized to the palms,
soles or axillae
* May be worse during warm
temperature
*Can be AD inherited
*25% of patients with axillary
hyperhidrosis have
palmoplantar hyperhidrosis, but
patients with palm and sole
hyperhidrosis may have axillary
hyperhidrosis
*Sweating may be intermittent
or constant
Hyperhidrosis
Gustatory Hyperhidrosis
 Experience excessive sweating of the forehead, upper
lip, perioral region, or sternum a few minutes after
eating spicy foods, tomato sauce, chocolate, coffee,
tea, or hot soups
 May also be caused by hyperactivity of the
sympathetic nerves(Pancoast’s tumor) or
postoperatively), sensory neuropathy (diabetes
mellitus or subsequent to zoster), parotitis or parotid
abscess, and surgery of the parotid gland
Hyperhidrosis
Other Localized Forms of Hyperhidrosis:
Localized sweating can occur over lesions of blue
rubber bleb nevus, glomus tumors, hemangiomas
and in POEMS syndrome, Gopalan’s syndrome,
complex regional pain syndrome, and as a result of
spinal cord tumors
Hyperhidrosis
Generalized Hyperhidrosis
May be induced by a hot, humid environment, a febrile illness, or
vigorous exercise
Hormonal disturbances such as hyperthyroidism, acromegaly,
diabetes mellitus, pregnancy, and menopause
Other causes include concussion, Parkinson’s disease,
pheochromocytoma, hypogycemia, salicylism, and lymphoma,
and metastatic tumors transecting the spinal cord
Hyperhidrosis
Treatment:
Treat underlying disorder
Topical aluminum chloride or aluminum chlorhydroxide
For the axillae, application of a 20% to 25% solution
nightly to a very dry axillae
Avoid deodorant-use baking soda instead
For palmar hyperhidrosis apply 20% aluminum chloride
tincture nightly, occluded with plastic gloves
Anhidrosis= absence of
sweating
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May be localized or
generalized
Generalized anhidrosis
occurs in anhidrotic
ectodermal dysplasia,
quinacrine anhidrosis,
miliaria profunda,
Sjogren’s syndrome,
hereditary sensory
neuropathy (type IV)
with anhidrosis, some
patients with diabetic
neuropathy, and
multiple myeloma
Anhidrosis with pruritis:
 Present with itching
when attempting to
sweat
 Absence of sweat when
body temperature rises
0.5degrees C, fine
papules appear at each
eccrine orifice
 Cooling completely
resolves symptoms
 Resolution may occur
after several years
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Anhidrosis
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Ross syndrome=
segmental anhidrosis
associated with tonic
pupils( Holmes-Adie
syndrome)
Intolerance and
segmental areas of
anhidrosis, loss of deep
tendon reflexes, and
compensatory
segmental hyperhidrosis
of functionally intact
areas
Anhidrosis localized to
skin lesions over
plaques of tuberculoid
leprosy
 Also over ares of
segmental vitiligo, in
the hypopigmented
streaks of incontinentia
pigmenti, and on the
face of patients with
follicular atrophoderma,
basal cell carcinomas,
and hypotrichosis

Bromidrosis= fetid sweat
Mainly in the axillae
 Caused by bacterial
decomposition of
apocrine sweat,
producing fatty acids
with offensive odors
 True bromidrosis is
usually not
recognized by the
patient
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Antibacterial soaps and
many comercial
deodorants are effective
in controlling axillary
malodor
Frequent bathing,
changing of
underclothes, shaving,
and topical
alumuminum chloride
Surgical removal of the
glands is an option
Bromidrosis
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Often patient s who
complain of offensive
axillary sweat actually
have psychiatric
disorders like delusion,
paranoia, phobia, or a
CNS lesion
Intranasal foreign body
and chronic mycotic
infection of the sinuses
in differential
Fish odor syndrome Caused by excretion of
trimethylamine( which
smells like rotten fish)
in the eccrine seat,
urine, saliva, etc.
 This chemical is
produced from carnitine
and choline in the diet
 Caused by an
autosomal dominant
defect in the ability to
metabolize
trimethylamine

Chromhidrosis
Rare disorder of the
apocrine sweat glands
 Frequently localized to
the face or axillae
 Most commonly sweat
is yellow, less frequently
blue, green, or black
 Colored sweat appears
in response to
adrenergic stimuli,
which cause
myoepithelial
contractions
 Colored apocrine sweat
fluoresces and is caused
by lipofusion
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Eccrine chromhidrosis is
caused by the coloring
of clear eccrine sweat
by dyes, pigments, or
metals on the skin
surface
Examples of this: bluegreen sweat from
copper workers, the
“red sweat” seen in
flight attendants from
the red dye in the labels
of life-vests
Brownish staining from
ochronosis
Small, round, brown or
deep-green macules
occur in the palms and
soles in patients with
excess bile secretion-
Fox-Fordyce Disease
Rare, occurs in women
during adolescence or
soon afterward
Conical, flesh-colored or
grayish, intensely
pruritic, discrete
follicular papules in
areas where the
apocrine glands occur
Fox-Fordyce Disease
Fox-Fordyce Disease
Apocrine gland sweating
does not occur in areas
of involvement
Hair density may be
decreased
90% occur in women aged
13-35
Pregnancy leads to
improvement
May occur
postmenopausally or in
males
Fox-Fordyce Disease
Treatment is difficultNo form of therapy is
uniformly effective
Estrogen therapy usually
in the form of oral
contraceptive pillsis
effective
Topical tretinoin, topical
and intralesional
steroids, topical
antibiotics, oral
retinoids and UV
phototherapy have been
helpful in a small
number of patients
Granulosis Rubra Nasi
A rare familial disease of
children
Occurring on the nose,
cheeks, and chin
Characterized by diffuse
redness, persistent
hyperhidrosis, and a
small dark red papules
that disappear on
diascopic pressure
The tip of the nose is red
or violet
Disappears spontaneously
at puberty without
leaving any traces
Hidradenitis
A term used to
describe diseases in
which the histologic
abnormality is
primarily an
inflammatory
infiltrate around the
eccrine glands

Two Types:
Neutrophilic eccrine
hidradenitis(NEH)
 Idiopathic plantar
hidradenitis(recurrent
palmoplantar
hidradenitis)

Neutrophilic Eccrine
Hidradenitis
Seen mainly in patients with
acute myelogenous
leukemia, usually
beginning about 10 days
after chemotherapy
begins
Lesions are typically
erythematous and
edematous papules and
plaques
Lesions are on the
extremities, trunk,
face(periorbital), and
palms (in decreasing
frequency)
Fever and neutropenia are
often present
Lesions resolve over 1 to 4
weeks
Neutrophilic Eccrine
Hidradenitis

There is a sparse,
perivascular, and
perieccrine infiltrate
seen predominantly
at the dermalsubcutaneous
junction
Neutrophilic Eccrine
Hidradenitis

Upon closer
inspection the
inflammatory
infiltrate is found to
be predominatly
around eccrine coils
at the dermalsubcutaneous
junction
Neutrophilic Eccrine
Hidradenitis

The infiltrate is
mixed but contains
neutrophils. The
eccrine ducts show
focal pallor
consistent with early
necrosis
Recurrent Palmoplantar
Hidradenitis
Primarily a disorder of
healthy children and
young adults
Lesions are primarily
painful, subcutaneous
nodules on the plantar
surface, resembling
erythema nodosum
Children may present
refusing to walk
because of plantar pain
Typically recurrent, and
may be triggered by
ambulation
Sagittal view of nail unit
Proposed derivations of the
adult structures of the nail
unit
Thick arrows
indicate epithelial
derivations of the
matrix
 Thin arows the
cornified products
thereform

Lichen Planus of Nails
Incidence is <1% to 10%
It may occur without skin changes, but 25% with nail
disease will have lichen planus at other locations
Most commonly it begins during fifth or sixth decade
Treatment is unsatisfactory- intralesional injection of
corticosteroids, polyethylene occlusive dressings have not
been successful
Oral predisone and oral retinoids in combination with topical
steroids have been successful in some
Early treatment is mandatory
Lichen Planus of Nails
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Nail changes are
irregular longitudinal
grooving and ridging of
the nail plate, thinning
of the nail plate,
pterygium formation,
shedding of the nail
plate with atrophy of
the nail bed, subungual
keratosis, and
subungual
hyperpigmentation
The plate may be
markedly thinned, and
sometimes the papules
of lichen planus may
involve the nail plate
Lichen Planus
Psoriatic Nails
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Nail involvement in
psoriasis varies from
10% to 50%
There may be pits , or
furrows or transverse
depressions (Beau’s
lines), crumbling nail
plate, or leukonychia,
with a rough or smooth
surface, in the nail plate
Psoriatic Nails
In the nail bed splinter
hemorrhages are found,
reddish discoloration of
a part or all of the nail
bed, and horny masses
 In the hyponychium
yellowish green
discoloration may occur
in the area of
onycholysis
 Up to 86.5% of patients
with psoriatic arthritis
will have psoriatic nail
changes
 Pustular psoriasis may
produce onycholysis,
with lakes of pus in the
nail bed or in the
perionychial areas

Darier’s Disease
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
Longitudinal,
subungual, red or white
streaks, associated with
distal wedge-shaped
subungual keratoses,
are the nail signs
diagnostic for DarierWhite disease
Characteristic is Vshaped nicking, linear
striations, onycholysis,
and subungual keratotic
reaction
Onychomadesis
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A periodic idiopathic
shedding of the nail
beginning at its
proximal end
Temporary arrest of the
function of the nail
matrix may also cause
this-as may penicillin
allergy
Neurologic disorders,
peritoneal dialysis,
mycosis fungoides, and
keratosis punctata have
been reported ad
causes of this type of
nail loss
Beau’s Lines
Transverse furrows that
begin in the matrix and
progress distally as the
nail grows
 Caused by the
temporary arrest of nail
function of the nail
matrix
 They may result from
any systemic illness or
major injury
 Shelley’s “shoreline”
nails appear to be a
very severe expression

Beau’s Lines

Multiple Beau’s lines
caused by
intermittent
inflammation of the
proximal nail fold
with resultant injury
to the underlying
proximal nail matrix
Half and Half Nails


These shoe the
proximal portion of the
nail white and the distal
half red, pink, or brown,
with a sharp line of
demarcation between
the two halves
Found in patients with
renal disease associated
with azotemia
Muehrcke’s Lines  Narrow white transverse


bands occurring in pairs
were described by
Muehrcke in 1956 as a
sign of chronic
hypoalbuminemia
Unlike Mees’ lines the
disturbance appears to
be in the nail bed and
not the nail plate
Andrew’s believes that
this change is caused
edema that induces
microscopic separation
of the normally tightly
adherent nail from its
bed
Mees’ Lines
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
Single or multiple white
transverse bands
described by Mees in
1919 as a sign of
inorganic arsenic
poisoning
Also have been reported
in thallium poisoning,
septicemia, dissecting
aortic aneurysm,
parasitic infections,
chemotherapy, and both
acute and chronic renal
failure
Terry’s Nails
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The distal 1 to 2 mm of
the nail shows a normal
pink color
The entire nail plate or
proximal end has a
white appearance as a
result of changes in the
nail bed
These changes have
been noted in patients
with cirrhosis, chronic
congestive heart failure,
and adult onset
diabetes, and the very
elderly
Onychorrhexis (Brittle Nails)

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Brittleness with
breakage of the nails
may result from
excessive strong soap
and water exposure,
from nail polish
remover,
hypothyroidism, or after
oral retinoid therapy
It affects up to 20% of
the population
Fragilitas unguium (nail
fragility) is part of this
process
B-complex vitamin
biotin increases the nail
thickness by 25% and
may help
Onychoschizia
Splitting of the distal nail
plate into layers at the
free edge
Is a common problem
among women
Represents a dyshesion of
the layers of keratin,
possibly as a result of
dehydration
Longitudinal splits may
also occur
Nail polish should not be
used
Biotin has been shown to
be effective in doses of
up to 2.5mg daily and
frequent emollients
Pitted Nails (Stippled Nails)
Small, pinpoint
depressions in an
otherwise normal nail
May be an early change
seen in psoriasis
Also seen in aa, in early
lichen planus, psoriatic
arthritis, perforating
granuloma annulare, or
in individuals with no
apparent disease
The pitting in aa tends to
be shallower and more
regular and has been
referred to as a “Scotch
plaid” (tartan) pattern
Pitted Nails (Stippled Nails)

Coalescence of
transverse pits
resulting in surface
nail plate crumbling
in alopecia areata
Racquet Nails (Nail en
Raquette)
The end of the thumb is
widened and flattened
The nail plate is flattened
as well
The distal phalanx is
abnormally short
They occur on one or both
thumbs
Inherited as AD trait
Chevron Nail (Herringbone
Nail)
A rare fingernail
ridging pattern of
children
The ridges arise from
the proximal nail
fold and converge in
a V-shaped pattern
toward a midpoint
distally
Hapalonychia
=Softened nails
It results from a defect in
the nail matrix that
makes the nails thin
and soft so that they
can be easily bent
May be due to malnutrtion
and debility, myxedema,
leprosy, Raynaud’s
phenomenon, oral
retinoid therapy, or
radiodermatitis
Platonychia
The nail is abnormally
flat and broad

Permanently
dystrophic nails
secondary to matrix
scarring in a patient
with StevensJohnson syndrome
Pitted Nails (Stippled Nails)

Coalescence of
transverse pits
resulting in surface
nail plate crumbling
in alopecia areata
Racquet Nails (Nail en
Raquette)
The end of the thumb is
widened and flattened
The nail plate is flattened
as well
The distal phalanx is
abnormally short
They occur on one or both
thumbs
Inherited as AD trait
Chevron Nail (Herringbone
Nail)
A rare fingernail
ridging pattern of
children
The ridges arise from
the proximal nail
fold and converge in
a V-shaped pattern
toward a midpoint
distally
Hapalonychia
=Softened nails
It results from a defect in
the nail matrix that
makes the nails thin
and soft so that they
can be easily bent
May be due to malnutrtion
and debility, myxedema,
leprosy, Raynaud’s
phenomenon, oral
retinoid therapy, or
radiodermatitis
Platonychia
The nail is abnormally
flat and broad

Permanently
dystrophic nails
secondary to matrix
scarring in a patient
with StevensJohnson syndrome
Nail-Patella Syndrome
Comprises numerous
anomalies
Characterized by the
absence or
hypoplsia of the
patella and
congenital nail
dystrophy
Triangular lunulae are
characteristic
Nail-Patella Syndrome
Other bone features:
Thickened scapulae,
hyperextensible joints,
radial head
abnormalities, and
posterior iliac horns
Skin changes may include
webbing of the elbows
Eye changes such as
cataracts and
heterochromia of the
iris
Hyperpigmentation of the
pupillary margin of the
iris (“Lester iris”) is a
characteristic finding in
50% of cases
Nail-Patella Syndrome





Glomerulonephritis with
albuminuria, hematuria,
and a variety of casts –
hyaline casts
40% have renal
dysplasia
25% suffer from renal
failure
AD trait localized to
chromosome 9q34.1
Micronychia, triangular
lunulae, and
clinodactyly

Tiangular lunulae of
nail-patella
syndrome
Median Nail Dystrophy





AKA dystrophia unguis
mediana canaliformis or
solennonychia
Consists of longitudinal
splitting or canal
formation in the midline
of the nail
The split, which often
resembles a fir tree,
occurs at the cuticle
and proceeds outward
as the nail grows
Trauma has been
implicated, but not
proven
A papilloma in the nail
matrix forcing a
tube(solenos) like
structure distal to it has
Pterygium Unguis



An abnormal extension
of the cuticle over the
proximal nail plate
Classic example is
lichen planus
Has been reported to
occur in sarcoidosis and
Hansen’s disease and
peripheral circulatory
disturbances
Onychogryphosis




Hypertrophy may
produce nails
resembling claws or a
ram’s horn
May be caused by
trauma or peripheral
vascular disease, or
more commonly by
neglect
Most commonly seen in
the elderly
If the blood supply is
adequate avulsion of
the nail with surgical
destruction of the
matrix is recommened
by some
Onychogryphosis

Characterized by an
opaque, thickened
nail plate with
subungual
hyperkeratosis and
transverse striations
in which there has
been exaggerated
growth in an upward
and lateral direction
Onychogryphosis
Is an exaggerated
enlargement of the
nail p;ate.
 Most often involves
the great toenail
only
 Self-neglect is the
most common cause

Onychophosis








A common finding in the elderly
It is a localized or diffuse hyperkeratotoic tissue that develops
on the lateral or proximal nailfolds, within the space between
the nailfolds and the nail plate
May involve the subungual area as a result of repeated trauma
Most frequently involves the first and fifth toes
Encourage the use of comfortable shoes
Involved areas should be debrided and treated with keratolytics
Emollients are also helpful
Precautionary measure to prevent this =wearing comfortable
shoes and relieving any pressure exerted by the nail on soft
tissues
Anonychia




Absence of nails
A rare anomaly
May be the result of a
congenital ectodermal defect,
ichthyosis, severe infection,
severe allergic contact
dermatitis, self inflicted
trauma, Raynaud’s
phenomenon, lichen planus,
or severe exfoliative diseases
Permanent anonychia has
been reported as a sequel of
Stevens-Johnson syndrome
Anonychia


May also be found in
association with
congenital
developmental
abnormalities such as
microcephaly, and widespaced teeth(AR)
Or Cook’s
syndrome(AD)- bilateral
nail hypoplasia of digits
1 through 3, the
absence of nails of
digits 4 and 5 of the
hands, total absence of
all the toenails, and
absence or hypoplasia
of the distal phalanges
of the hands and feet
Onychoatrophy





Faulty underdevelopment of the nail that may be
congenital or acquired
The nail is thinner and smaller
A side effect of etretinate therapy
Also may be seen in vascular disturbances,
epidermolysis bullosa, lichen planus, Darier’s disease,
multicentric reticulohistiocytosis, and leprosy
It is also seen in the nail-patella syndrome
Onychomadesis



A periodic idiopathic
shedding of the nail
beginning at its
proximal end
Temporary arrest of the
function of the nail
matrix may also cause
this-as may penicillin
allergy
Neurologic disorders,
peritoneal dialysis,
mycosis fungoides, and
keratosis punctata have
been reported ad
causes of this type of
nail loss
Beau’s Lines
Transverse furrows that
begin in the matrix and
progress distally as the
nail grows
 Caused by the
temporary arrest of nail
function of the nail
matrix
 They may result from
any systemic illness or
major injury
 Shelley’s “shoreline”
nails appear to be a
very severe expression

Beau’s Lines

Multiple Beau’s lines
caused by
intermittent
inflammation of the
proximal nail fold
with resultant injury
to the underlying
proximal nail matrix
Half and Half Nails


These shoe the
proximal portion of the
nail white and the distal
half red, pink, or brown,
with a sharp line of
demarcation between
the two halves
Found in patients with
renal disease associated
with azotemia
Muehrcke’s Lines  Narrow white transverse


bands occurring in pairs
were described by
Muehrcke in 1956 as a
sign of chronic
hypoalbuminemia
Unlike Mees’ lines the
disturbance appears to
be in the nail bed and
not the nail plate
Andrew’s believes that
this change is caused
edema that induces
microscopic separation
of the normally tightly
adherent nail from its
bed
Mees’ Lines


Single or multiple white
transverse bands
described by Mees in
1919 as a sign of
inorganic arsenic
poisoning
Also have been reported
in thallium poisoning,
septicemia, dissecting
aortic aneurysm,
parasitic infections,
chemotherapy, and both
acute and chronic renal
failure
Terry’s Nails



The distal 1 to 2 mm of
the nail shows a normal
pink color
The entire nail plate or
proximal end has a
white appearance as a
result of changes in the
nail bed
These changes have
been noted in patients
with cirrhosis, chronic
congestive heart failure,
and adult onset
diabetes, and the very
elderly
Onychorrhexis (Brittle Nails)




Brittleness with
breakage of the nails
may result from
excessive strong soap
and water exposure,
from nail polish
remover,
hypothyroidism, or after
oral retinoid therapy
It affects up to 20% of
the population
Fragilitas unguium (nail
fragility) is part of this
process
B-complex vitamin
biotin increases the nail
thickness by 25% and
may help
Onychoschizia
Splitting of the distal nail
plate into layers at the
free edge
Is a common problem
among women
Represents a dyshesion of
the layers of keratin,
possibly as a result of
dehydration
Longitudinal splits may
also occur
Nail polish should not be
used
Biotin has been shown to
be effective in doses of
up to 2.5mg daily and
frequent emollients
Pitted Nails (Stippled Nails)
Small, pinpoint
depressions in an
otherwise normal nail
May be an early change
seen in psoriasis
Also seen in aa, in early
lichen planus, psoriatic
arthritis, perforating
granuloma annulare, or
in individuals with no
apparent disease
The pitting in aa tends to
be shallower and more
regular and has been
referred to as a “Scotch
plaid” (tartan) pattern
Pitted Nails (Stippled Nails)

Coalescence of
transverse pits
resulting in surface
nail plate crumbling
in alopecia areata
Racquet Nails (Nail en
Raquette)
The end of the thumb is
widened and flattened
The nail plate is flattened
as well
The distal phalanx is
abnormally short
They occur on one or both
thumbs
Inherited as AD trait
Chevron Nail (Herringbone
Nail)
A rare fingernail
ridging pattern of
children
The ridges arise from
the proximal nail
fold and converge in
a V-shaped pattern
toward a midpoint
distally
Hapalonychia
=Softened nails
It results from a defect in
the nail matrix that
makes the nails thin
and soft so that they
can be easily bent
May be due to malnutrtion
and debility, myxedema,
leprosy, Raynaud’s
phenomenon, oral
retinoid therapy, or
radiodermatitis
Platonychia
The nail is abnormally
flat and broad

Permanently
dystrophic nails
secondary to matrix
scarring in a patient
with StevensJohnson syndrome
Tumors of the Nail
Signs heralding such
neoplasms=
Paronychia, ingrown nail,
onycholysis, pyogenic
granuloma, nail plate
dystrophy, bleeding,
and discolorations
Symptoms of pain, itching,
and throbbing may also
be seen


Begin tumors of the nail
include: verruca,
pyogenic granuloma,
fibromas, nevus cell
nevi, myxoid cysts,
angiofibromas
(Koenen’s tumors), and
epidermoid cysts
Pyogenic granuloma-like
lesions may occur
during treatment with
isotretinoin or indinavir
Tumors of the Nail

Pyogenic granuloma
after trauma to the
lateral nail fold
Tumors of the Nail

Pyogenic granuloma
of the nail bed
mimicking
hematoma or
melanoma
Tumors of the Nail

Pyogenic granuloma
formation secondary
to chronic irritation
from a genetic
subungual exostosis
Tumors of the Nail

Advanced pyogenic
granuloma
formation resulting
from long-standing
pressure and lowgrade infection
Tumors
Glomus tumor with
a bluish hue in the
distal nail bed
 Maybe painful

Tumors

Mucous cyst in its
most common
location exerting
pressure on the
matrix, resulting in a
grooved nail plate
Tumors

Normal finger
contour is altered by
focal mucinosis ( a
myxoid cyst), which
presses against the
proximal nail matrix
and results in a
groove in the nail
plate.
Tumors

Giant cell tumor of
the tendon sheath
presenting lateral to
the usual location on
the dorsum of the
digit
Tumors of the nail

Advanced pyogenic
granuloma
formation resulting
from long-standing
pressure and lowgrade infection
Genetic Subungual Exostosis
Dome-shaped
genetic
Subungual exostosis
A, Lateral projection
B, Dorsoplantar
projection

Exostosis, Enchondroma,
Osteochondroma, Epidermoid
Cyst
Four benign tumors of the subungual
region that involve the bone first and
the nail plate second
X-ray films of these lesions should be
taken first, before biopsy, because the
appearance may be characteristic
enough to allow for definitive surgery
initially
Differential Diagnosis
Genetic Subungual Exostosis


A, Tabletop genetic
subungual exostosis,
hallux lateral projection
B, Dorsiplantar
projection. Arrowheads
indicate the medial
origin of the exostosis,
extending medial to the
shaft
Subungual Exostosis

The growing
genentic subungual
exostosiss has
caused
expansion
and tightening of
the periungual skin
distally and medially
Genetic Subungual Exostosis




Subungual nail plate
hemorrhage secondary
to nail plate elevation.
Traumatic implantation
of nail matrix into nail
bed is also seen
There are nail plate
inclusions in the dermis
There is no granular
laayer
Subungual Exostosis


Genetic subungual
exostosis exerts distal
pressure on the
hyponychium,
promoting fibrosis and
implantation of
epidermis into dermis
Note kerrratinizing
epidermal inclusion
developing with the
benefit of a granular
layer, surrounded by
fibrosis of the the
hyponychium
Premalignant and Transitional
Tumors
Bowen’s disease of the
lateral nail groove with
minimal clinical changes
for 2 yrs
 Etiologic factors:
chronic infection,
trauma, HPV
 Seen more commonly in
HIV infected individuals
 Seeing an increase in
Bowen’s disease in nail
bed over the past
decade

Premalignant and Transitional
Tumors
Squamous cell
carcinoma
 Actinic keratosis
 Keratoacanthoma
 Bowen’s disease

Bowen’s Disease

Bowen’s disease
masking as a
pigmented streak
along the lateral nail
groove, clinically
suspected of being a
melanoma
Bowen’s Disease


Histology of the lesion
prior showing cellular
atypia, mitoses, and
individual cell
keratinization
The nail plate
intervenes between the
nail fold on top and the
nail bed on the bottom
in this tangential cut
Subungual Melanoma  Frequently diagnosed



late in the course or
growth, since it
simulates
onychomycosis or
subungual hematoma
Amelanotic melanoma
may occurand nay be
mistaken for
pyogenicum
More frequently found
in Japanese than in
other ethnic populations
Example is in vertical
growth phase showing
nodule formation, nail
destruction, and faint
Hutchinson’s sign of
proximal nail fold
Subungual Melanoma

Histology of acral
lentiginous
melanoma showing
giant dendritic
melanocytes with
atypia
Onychotillomania

A compulsive
neurosis in which
the patient picks
constantly at the
nails or tries to tear
them of
Onycholysis




Spontaneous
separation of the nail
plate
Usually beginning at the
free margin and
progressingg
proxximally
Rarely the lateral
borders may be
involved
Less often separation
may begin proximal to
the free edge, in an
oval area 2 to 6 mm
broad, with a yellowish
brown hue (“oil spot”)
this is psoriasis
Onycholysis
 The nail itself is smooth and firm
Underneath the nail a discoloration may occur as a result
of accumulation of bacteria or yeast
 Mostly seen in women secondary to trauma and
subsequent infection with Candida
 Systemic causes: hyperthyroidism, hypothyroidism,
pregnancy, pellagra, syphillis, trauma, vaginal yeast
infections with secondary spread
 Chemical causes: solvents, nail polish base coat, artificial
fingernails
 Photoonycholysis may occur during or soon after therapy
with tetracycline derivatives, psoralens, fluoroquinolones,
or chloramphenicol, with subsequent exposure to light
 Chemotherapeutic agents like mitoxantrone, may also
precipitate onycholysis
 May rarely be a sign of distal metastasis
 May be inherited as AD trait

Management
Avoid trauma
 Keep nail bed dry
 Trim affected portion
of the nail

Onychocryptosis





Aka unguis incarnatus;
ingrown nail
One of the most
frequent nail
complaimts
Chiefly occurs on the
great toe
Leads to excessive
lateral nail growth into
the nail fold, leading to
PAIN and inflammation
Causes are: improper
fitting shoes, improper
nail trimming at the
lateral edges so that the
anterior portion cuts int
the flesh as it grows
distally
Onychocryptosis




Rather than remove the
nail a Jansey operation
is frequently successful
This involves removing
the overhanging lateral
nail fold so that the nail
does not cut into it
When healed the nail
edges resembles the
thumb and is very
functional
The nail is not altered

Recurrent
onychocryptosis with
subsequent
periungual
inflammation and
granulation tissue.

Partial regrowth of
normal nail after
previous nail plate
avulsion for
onychocryptosis
secondary to severe
overcurvature of the
nail plate with painful
contriction of the nail
plabed (pincer nails)

Recurrent
onychocryptosis with
subsequent
periungual
inflammation and
granulation tissue
Jansey operation
Under local anesthesia
and using a rubber
band tourniquet at the
base of the toe, (A), a
linear incision is
madeparallel to nail
margin, (B), A convex
incision is made in a
curvilinear plane parallel
to the nail bed meeting
the initial incision
(C), involved tissue is
removed, (E), lateral
flap is then
approximated by 1 –2
sutures
Treatment
Another procedure is to apply saturated solution of phenol
to the nail matrix after a portion of the ingrown nail has
been removed surgically (phenolization)
 Objective here is to permanently ablate the part of the nail
matrix producing the nail that was ingrowing
 Between 60% & 80% of patients treated with partial or
complete nail plate avulsion have a recurrence
 In mild cases, insertion of a cotton pas beneath the distal
corner of the nail may resolve the problem
 A flexible plastic tube to splint the nail is useful, the nail
may be flattened through the use of a stainless steel wire
brace
 The brace fits the over-curved nail exactly and maintains
constant tension over the nail
 Adjustments are made over a period of 6 months
 Liquid nitrogen spray to the area for 20 to 30 sec may be

Leukonychia or White Nails





Four forms are
recognized
Leukonychia
punctata
Leukonychia striata
Leukonychia partialis
Leukonychia totalis
Nail-Patella Syndrome
Comprises numerous
anomalies
Characterized by the
absence or
hypoplsia of the
patella and
congenital nail
dystrophy
Triangular lunulae are
characteristic
Nail-Patella Syndrome
Other bone features:
Thickened scapulae,
hyperextensible joints,
radial head
abnormalities, and
posterior iliac horns
Skin changes may include
webbing of the elbows
Eye changes such as
cataracts and
heterochromia of the
iris
Hyperpigmentation of the
pupillary margin of the
iris (“Lester iris”) is a
characteristic finding in
50% of cases
Nail-Patella Syndrome





Glomerulonephritis with
albuminuria, hematuria,
and a variety of casts –
hyaline casts
40% have renal
dysplasia
25% suffer from renal
failure
AD trait localized to
chromosome 9q34.1
Micronychia, triangular
lunulae, and
clinodactyly

Tiangular lunulae of
nail-patella
syndrome
Median Nail Dystrophy





AKA dystrophia unguis
mediana canaliformis or
solennonychia
Consists of longitudinal
splitting or canal
formation in the midline
of the nail
The split, which often
resembles a fir tree,
occurs at the cuticle
and proceeds outward
as the nail grows
Trauma has been
implicated, but not
proven
A papilloma in the nail
matrix forcing a
tube(solenos) like
structure distal to it has
Pterygium Unguis



An abnormal extension
of the cuticle over the
proximal nail plate
Classic example is
lichen planus
Has been reported to
occur in sarcoidosis and
Hansen’s disease and
peripheral circulatory
disturbances
Characterized by
adherence of the distal
portion of the nail bed
to the ventra lsurface of
the nail plate
 Presents at birth or is
acquired and may be
painful when
manipulating small
objects
 Results from the
extension of the zone of
the nail plate, leading
eventually to a more
ventral and distal
extension of the
hyponychium
 Most commonlysee is
the secondary or
acquired form caused

Pterygium Inversum U
Hangnail




An overextension of the
eponychium (cuticle)
The cuticle becomes
split and peels away
from the proximal or
lateral nail fold
Lesions are painful
Trim with a scissors and
use emollient creams to
keep the cuticle soft

Pincer Nails





AKA trumpet nails or
omega
Common toenail
disorder
Lateral edges of the nail
slowly approach one
another, compressing
the nail bed and
underlying dermis
May occur in the
fingernails and is then
usually asymptomatic
Treatment is use of
commercial plastic
braces after flattening
the nail
Urea ointment under
occlusion has been
reported to be helpful
Onychophagia
Nail biting
 A common
compulsive behavior
 May markedly
shorten the nail bed
 Sometimes damage
the matrix and lead
to pterygium
formation

Difficult habit to
cure
 May apply dimethyl
sulfoxide (DMSO)
every day or two as
a mild deterrent
 Psychopharmacologi
c intervension may
be required

Onychotillomania

A compulsive
neurosis in which
the patient picks
constantly at the
nails or tries to tear
them of