REVIEW OF LITERATURE
BIOLOGY OF NAILS3
The nail apparatus consists of a horny "dead" product, the nail plate, and four
specialized epithelia: the proximal nail fold, the nail matrix, the nail bed, and the
hyponychium.
Nail Plate
The nail plate is a fully keratinized structure that is continuously produced
throughout life. It results from maturation and keratinization of the nail matrix
epithelium and is firmly attached to the nail bed, which partially contributes to its
formation. Proximally and laterally the nail plate is surrounded by the nail folds. At
the tip of the digit, the nail plate separates from the underlying tissues at the
hyponychium. The nail plate is rectangular, translucent, and transparent.
The proximal part of the finger nails, especially of the thumbs, shows a
whitish, opaque, half-moon-shaped area, the lunula, that is the visible portion of the
nail matrix.
In transverse sections, the nail plate consists of three portions: dorsal nail
plate, intermediate nail plate4, and ventral nail plate. The dorsal and the intermediate
portions of the nail plate are produced by the nail matrix, whereas its ventral portion is
produced by the nail bed.
The mean toenail thickness at the distal margin is 1.65 0.43 mm in meaji and
1.38 0.20 mm in women. Fingernails are thinner; the mean thickness is 0.6 mm in
mean and 0.5 mm in women. There is an increase in nail thickness with age
particularly in the first two decades.
The proximal nail fold is a skin fold which consists of a dorsal and ventral
portion. The dorsal portion is anatomically similar to the skin of the dorsum of digit
but thinner and devoid of pilosebaceous units. The ventral portion, when cannot be
seen from the exterior and proximally continues with the germinative matrix, covers
approximately fourth of the nail plate. It closely adheres to the nail plate surface and
keratinizes with a granular layer. The limit between the proximal nail fold and the nail
matrix can be histologically established at the site of disappearance of the granular
layer.
Nail Matrix
The nail matrix is a specialized epithelial structure that lies above the midportion of the distal phalanx. After elevation of the proximal nail fold, the matrix
appears as a distally convex crescent with its lateral horns extending proximally and
laterally.
In longitudinal sections the matrix has a wedge-shaped appearance and
consists of a proximal (dorsal) and a distal (ventral) portion. Nail matrix keratinocytes
divide in the basal cell layer and keratinize in the absence of a granular zone. 5 In some
conditions nuclear fragments may persist within the intermediate nail plate, producing
leukonychial spots.
Maturation and differentiation of nail matrix keratinocytes do not follow a
vertical axis, as in the epidermis, but occur along a diagonal axis that is distally
oriented. For this reason, keratinization of the proximal nail matrix cells produces the
dorsal nail plate and keratinization of the distal nail matrix cells produces the
intermediate nail plate.
In some fingers the distal matrix is not completely covered by the proximal
nail fold but is visible through the nail plate as a white half moon-shaped area, the
lunula. The white color of the lunula results from two main anatomic factors: (1) the
keratogenous zone of the distal matrix contains nuclear fragments that cause light
diffraction; (2) nail matrix capillaries are less visible than nail bed capillaries because
of the relative thickness of the nail matrix epithelium6.
NAIL BED
The nail bed extends from the distal margin of the lunula to the onychodermal
band and is completely visible through the nail plate. The nail bed epithelium is thin
and consists of two to five cell layers. Nail bed keratinization produces a thin horny
layer that forms the ventral nail plate. Nail bed contribution to nail plate formation
corresponds to approximately one fifth of the terminal nail thickness and mass 7. In
pathologic sections, the ventral nail plate is easily distinguishable because of its light
eosinophilic appearance. Nail bed keratinization is not associated with the formation
of a granular layer. This may appear, however, when the nail bed becomes exposed
after nail avulsion5.
Hyponychium
The hyponychium marks the anatomic area between the nail bed and the distal
groove, where the nail plate detaches from the dorsal digit. Its anatomic structure is
under normal conditions the water content of the nail plate is 18 percent, and most of
the water is in the intermediate nail plate. When the water content decreases below 18
percent, the nail becomes brittle; when it increases above 30%, it becomes opaque and
soft8.
Physical Properties
The nail plate is hard, strong, and flexible. The hardness and strength of the
nail plate are due to its high content of hard keratins and cysteine-rich high-sulfur
proteins; whereas its flexibility depends on its water content and increases with nail
plate hydration9. The double curvature of the nail plate along its longitudinal and
transverse axes enhances nail plate resistance to mechanical stress10.
Nail Growth
The nail plate grows continuously throughout life of an individual. Fingernails
grow faster than toenails, with a mean growth of 3 mm/month for fingernails and 1
mm/ month for toenails. Complete replacement of a fingernail requires 100 to 180
days (6 months). When the nail plate is extracted, it is approximately 40 days before
the new fingernail first emerges from the proximal nail fold. After a further 120 days
it will reach the fingertip. The total regeneration time for a toenail is 12 to 18 months.
Physiological and pathological factors affecting nail growth.
Faster growth
Day
Right hand
Youth
Fingers
Summer
Male
Middle, index finger nails
Minor trauma and avulsion
Pregnancy
Slower growth
Night
Left hand
Old age
Toes
Winter
Females
Thumb, little finger
First day of life
Lactation
Classification 2
Abnormalities of shape
Abnormalities of nail attachment
Abnormalities of nail surface
Abnormalities of nail color
Tuberous sclerosis
Pachydermoperiostitis
Rothmond Thomson syndrome
Dermatologic diseases of the nail unit
Localized
Systemic
Connective tissue disease
Psoriasis
Lichen planus
Alopecia areata
Eczema
Pemphigus vulgaris
Reiters disease
Nail changes due to systemic diseases
Malignant
Subungual exostosis
Ganglion cyst
Glomus tumour
Pyogenic granuloma Angioma
Fibroma
Onychomatric oma
Actinic keratosis
Clavus
Enchondroma
Local
Squamous cell carcinoma
Melanoma
Bowen disease
Basal cell epithelioma
Sarcoma
Systemic
Metastic cancer
Lymphoma
Infections of nails
Localized process
Bacterial infections
Staphylococcus aureus
Streptococci
Pseudomonas
Mixed bacterial and fungal infection
Fungal infection
T.rubrum
T. mentagrophytes
E.fluoccosum
Yeast infections
Candida albicans
Spirocheatal
Viral
Warts, molluscum contagiosum, herpes simplex
Systemic process
Septicemia and septic emboli
Candidiasis
Psoralens
Antiepileptic drugs
Retinoids
Antimicrobial drugs
2nd Classification
Abnormalities of
Abnormalities
shape
Clubbing
nail attachment
Nail shedding
of Abnormalities
of
nail surface
Longitudinal
Abnormalities of
nail color
Leukonychia
grooves
Koilonychia
Onycholysis
Transverse groove
Longitudinal
melanonychia
Pincer nails
Anonychia
Pterygium
Pterygium ungium
Elkonyxis
Pitting
Mees lines
Muehrckes lines
Brachyonychia
Micronychia and
inversus
Hang Nails
Subungal
Onychoschizia
Onychorrhexis
Macronychia
Polyonychia
hyperkeratosis
Nail thickening
Trachyonychia
Onychogryphosis
Onychomadesis
Beading and
Longitudinal
ridging
erythronychia
Splinter
Ungium incarnates
haemorrhages
Longitudinal
melanonychia
Yellow nail
Nail
findings
Nail hypertrophy nail
dystrophy
Additional findings
Prevalence
56.2%
24.9%
keratoderma rare
Same as type 2
steatocystoma multiplex
Angular
cheiltis,
corneal
11.7%
dyskeratosis, cataract
4
Same as type 1, 2, 3
7.2%
Nails
o Patients have different degrees of nail dysplasia.
o The nails, especially those on the thumbs, are typically absent or short and
never reach the free edge of the finger.
o Nail dysplasia, a typical feature, is more severe on the ulnar side than on the
radial side.
o The toenails are rarely affected.
o Other nail abnormalities in patients with nail-patella syndrome include
splitting, longitudinal ridging, koilonychia, poor lunula formation, and
18
discoloration .
In the absence of other nail changes, V-shaped triangular lunulae with a distal peak in
the midline are pathognomonic for nail-patella syndrome.19,20
No treatment is available for the cutaneous findings of nail-patella syndrome (NPS)
Epidermolysis bullosa21:
Nail abnormalities have been reported in all forms of epidermolysis bullosa except the
Weber-Cockayne and the Koebner types and the Mendes da Costa variant.
They are the result of abnormalities of the nail matrix and nail bed associated
with the pathogenic alterations at the dermo-epidermal junction. In addition,
secondary trauma in the areas of epidermal-dermal separation and chronic
inflammation of the nail unit are the possible contributory factors1.
In the lethal junctional type, nail involvement begins early as paronychia-like
periungual lesions and the nails are easily shed. Sometimes, they are absent or
hypoplastic at birth.
Loss of nails is a constant feature of the most severe forms of the recessive
dystrophic type, the mutilating type and the generalized, non-multilating type. Other
nail
abnormalities
are
dystrophy,
onychogryphosis,
pterygium
formation
White streaks. longitudinal leukonychia: These extend from the proximal nail
fold to the distal margin, which may show a small incision and mild
onycholysis due to a subungual hyperkeratotic papule. The width of the
longitudinal white streak varies from 0.1 to a few millimeters.
Other signs of Darrier's disease are not diagnostic and include: splinter hemorrhages,
nail fissuring and fragility, severe nail bed hyperkeratosis, and keratotic papules of the
proximal nail fold.
In some patients, the nails may be grossly thickened and deformed. Secondary
bacterial infection of more severe cases is not unusual.
Diagnostic signs
Non-diagnostic signs.
Splinter hemorrhages.
Red
streak
Clinical
Site of origin
lesion
longitudinal Nail bed
Histological abnormalities
Mild
epithelial
hyperplasia
with
vasodilatation
While
longitudinal
Nail bed
subungal streak
Epithelial
hyperplasia
orthokeratosis
subungal
and
with
parakeratosis
multinucleated
epithelial
hyponchium
Nail bed dermis
hyperplasia
with
Nail findings
o Nail dystrophy is seen in approximately 90% of patients, with fingernail
involvement often preceding toenail involvement, o Progressive nail
dystrophy begins with ridging and longitudinal splitting. Progressive
atrophy, thinning, pterygium, and distortion eventuate in small, rudimentary,
or absent nails.
Psoriasis 25,26,27
Between 10-50% of patient with psoriasis may have nail involvement in adults and
39% of children. About 10-20% of people who have skin psoriasis also have psoriatic
arthritis, a specific condition in which people have symptoms of both arthritis and
psoriasis of people with psoriatic arthritis, 53-86% have affected nails.
Nail involvement in psoriasis
Proximal germinative matrix
Pitting,
Beau's
lines,
onycholysis,
leukonychia
Focal oncholysis,
Whole matrix
Nail bed
Nail folds
leukonychia,
red
Pitting: It is the most common nail abnormality seen in psoriasis. It results from focal
parakeratosis of the abnormal keratinizing cells of the proximal nail matrix. As the
developing nail plate grows beyond the margin of the proximal nail fold these cells
are lost and leave behind surface indentations that appear as pits. Common in finger
nail than toe nails. Pits are small and deep seated. Occasionally punched out areas are
seen (elkonyxis) sometimes uniform pitting of all nails is seen (thirable nails).
Presence of >20 pits suggests psoriatic etiology. Other causes of nail pitting.
Psoriasis
Reiters disease
Lichen nitidus
Chronic paronychia
After etrinate therapy
Lichen planus
Eczema
Secondary syphilis
Alopecia areata
Salmon patches are circular areas of reddish brown discolorations beneath the nail
plate (oil drop sign) due to focal bed parakeratosis. Splinter hemorrhages occur due to
increased vascularity and fragility of the nail bed dermis.
Onycholysis is common and occurs as distal extension of the salmon patches or may
commence at the distal free edge following disruptions of the onychodermal band. A
separated nail is a good nidus for yeast, dermatophytes and bacteria.
Yellow brown discoloration is due to nail bed disease due to serum glycoprotein
deposition.
Subungal hyperkeratosis results from deposition and collection of cells under the nail
plate that have not undergone desquamation.
Pathophysiology: The pathogenesis of the psoriatic nail disorder is not completely
known. It may be due to a combination of genetic, environmental, and immune
factors. A well known fact is that a familial aggregation of psoriasis exists. Recent
studies have linked psoriasis with certain human leukocyte antigen subtypes (e.g.
Cw6, B13, Bw57, Cw2, Cwlland B27). A T-cell mediated inflammatory process is
being investigated as part of the pathogenesis of psoriasis.
Histopathology: Psoriasis can affect any part of the nail unit. Most changes occur in
the nail plate. Histological findings of nail psoriasis include mild-to-moderate
hyperkeratosis, hypergranulosis, hemorrhage in the corneum layer, papillomatous
epidermal hyperplasia and spongiosis.
Symptoms
Pitting
Sites
Proximal nail matrix
Features
Loss of parakeratotic cells
from surface of nail plate
Beau's lines
Leukonychia
Midmatrix disease
Subungual
hyperkeratosis
Onycholysis
onycholysis
Nail bed and hyponychium Nail plate separates from its
underlying attachment to nail
bed. Nail plate widens and
may
detach.
Nail bed
Secondary
structures
Translucent
of
underlying
yellow-red
Pustular psoriasis: Pustules develop below the nail plate. In some areas there is
complete disruption of the nail plate. Acrodermatitis continua can be very aggressive
disease and may lead to resorptive osteolysis and loss of fingers and toes.
Lichen planus:29,30,31,32
Nail abnormalities are evident in about 10% of patients with skin or mucosal
lichen planus.29 Nail lichen planus, however, most commonly occurs in the absence of
skin or mucosal involvement.
Nail lichen planus may cause permanent nail destruction if not properly
diagnosed and treated.
Diagnosis of lichen planus of the nails is suggested by thinning, longitudinal
ridging, and Assuring of the nail plate. Pterygium formation is a possible outcome and
indicates nail matrix scarring.30,31
In most patients the disease starts abruptly and the nails become markedly
thinned and longitudinally ridged, opaque, and extremely brittle.
Dorsal nail pterygium describes distal extension has a V-shaped appearance
and often splits the nail plate in two portions. Pterygium occurs where the nail plate is
absent due to nail matrix destruction and widens with progressive scarring of the
matrix.
In addition to these very typical symptoms, nail lichen planus may produce
other nail abnormalities including.
Twenty-nail dystrophy.
Pigmentary changes.
Pathogenesis
Minute focal atrophy of the proximal
Course
Reversible
Pterygium
matrix
Severe destruction of the nail matrix
Permanent
with scarring
Total destruction of the nail matrix
Permanent
Reversible
plate
Subungal hyperkeratosis
Pup tent sign
Vniform thinning of the
Reversible
May be reversible
May be permanent
nail plate
Pterygium unguis is the hallmark of severe nail disease. It is due to focal destruction
of Lie nail matrix.
Causes of Pterygium:
Lichen planus
Leprosy
scarring trauma
I-raft versus host disease
Jopecia areata33
Nail abnormalities occur in about 20%- of adults and 50% of children with apecia areata. They are more common in males and in patients with severe alopecia
irsata.33"35 The nail abnormalities may precede or follow the onset of hair loss and
are a . a ^sequence of nail matrix involvement by the disease. Signs that are typical
for alopecia ffsata are geometric pitting and geometric leukonychia.
Trachyonychia (twenty-nail dystrophy) is due to alopecia areata in most cases.
Nail abnormalities may be limited to one nail or involve most of the nails;
Geometric pitting: The nail plate shows multiple, small; superficial pits which
are regularly distributed in a geometric pattern along longitudinal and
transverse lines.
Geometric punctate leukonychia: This is rare but very typical. The nail plate
presents multiple, small white spots, which are geometrically oriented in a
grill pattern36 along longitudinal and transverse lines.
Alopecia areata also produces nail signs that are not diagnostic, including: Beau's
lines, onychomadesis and erythema of the lunulae:
Erythema of the lunulae:37 The Lunula shows mottled erythema. This sign is
most frequently observed in association with trachyonychia.
Chronic eczema usually affects the proximal nail fold and/or the
hyponychium. Chronic paronychia is the most common presentation with
swelling of the proximal nail fold and loss of the cuticle. The nail plate
frequently shows irregular superficial abnormalities, such as pitting and Beau's
lines resulting from nail matrix damage. Yellow or green discoloration of the
lateral margins of the nail is common and due to secondary bacterial
colonization.
Pemphigus:39,40
Nail involvement in pemphigus vulgaris occurs in up to 22% of patients and is
almost always localized to the fingernails, especially the thumb and the index finger.
Erosive acute paronychia results from acantholysis of the proximal nail fold. It
is often associated with hemorrhagic discoloration of the proximal nail fold with
blood and serum fluid discharge. Pseudopyogenic granuloma of the proximal or
lateral nail fold may be observed. It may be the first symptom of a relapse of the
disease. Beau's lines and onychomadesis may follow acute paronychia41.
Other non-specific nail abnormalities that have been reported in pemphigus
include subungual hemorrhages and onycholysis, trachyonychia, subungual
hyperkeratosis, yellow or brown discoloration, and nail destruction.
Onychomycosis due to dermatophytes appears to be common in patients with
pemphigus, probably as a consequence of therapeutic immunosuppressant.
Nail changes in collagen vascular diseases42-45
Systemic lupus erythematosus: Nail abnormalities are seen in 25% of patients.
Cuticle: ragged or hyperkeratotic, lunulae: red, splinter hemorrhages, pitting, ridging
nail fold erythema, telengicctasia can occur. Nail fold capillary loop density is normal
but the individual capillary appear tortuous or cork screw shaped. In chill blain lupus
there is gross distortion of finger tips and nail plate.
Systemic sclerosis: Pterygium ungium inversus and parrot beak nails are the 2 most
characteristic nail changes. This change is also seen in systemic lupus erythematosus,
neurofibromatosis, and trauma. The parrot beak is over curvature of the free edge of
the nail over a shortened finger as a consequence of atrophy of the soft tissue of the
nail.
Dermatomyositis:
Nail fold erythema, telengiectasias, ragged cuticles, cuticular hemorrhages, pits on
finger nails, half and half nails and anonychia may be seen. Periungal ischemia is
considered a sign of internal malignancy.
Rheumatoid arthritis:
Nail growth decreased. Nail fold telengiectasias and longitudinal ridging with beading
of the nail plate can be seen. Beading of at least 6 fingernails or 4 toe nails is
considered highly specific of an established disease. Rheumatoid nodules can occur at
the free edge of nail.
Sarcoidosis:
Nail changes are seen more in lupus pernio of digits. Thickening, opacity increased
fragility, atrophy, perygium, subungal hyperkeratosis can occur.
Nail changes in systemic disease
Yellow Nail Syndrome46
Yellow nail syndrome is characterized by a triad of yellow nails, lymphedema
and respiratory tract involvement. The cause of the disease is unknown. It is more
common in adults but can also occur in children. The sexes are equally affected.
Nail changes are the most typical manifestations of the disease and are an
absolute requirement for diagnosis. All the nails may be affected, although this is not
universal. The affected nails are thickened and sclerotic (scleronychia) and
excessively curved from side to side. The entire nail plate shows a diffuse yellow to
yellow-green discoloration and appears opaque. 47
The spontaneous clearance of nail abnormalities with resolution of the
systemic changes has been documented.
Treatment with oral and topical vitamin E and with oral zinc supplementation
has been shown to improve nail changes. Interamatrical injections of triamcinolone
may help.48,49
Traumatic nail disorders:50
Onychophagia:
It is a common oral compulsive habit in children and young adults, affecting
around 30% of children between 7 to 10 years and 45% of teenagers. In adults it
occurs due to periods of concentration and focusing. Bitten nails are short and
irregular with 50% of nail bed exposed. Fine spicules may be left at the free edge of
the nails. Nail plate abnormality > onychoschizia) and centrally prounced transverse
ridges (wash board nails) and longitudinal melanonychia may be seen. Periungal
warts are common in nail biters.
Treatment: Behavioral treatments are based in discouraging the habit and replace it
with a more constructive habit.
Pincer nails (Trumpet nails):
It is characterized by overall transverse over curvature that is most prominent in the
midline and distally. The nail plate assumes a conical shape and rises above the nail
and in extreme cases a tunnel is formed. The lateral border of the nail tightens round
the soft tissue giving the latter a pinched appearance. It is seen those wearing ill fitting
shoes, psoriasis, tinea ungium, beta blockers use. Paronychial infections may be more
frequent with this type of nail irregularity.
Treatment: Restoration of the normal contour and shape of the nail plate is achieved
with the aid of dermal grafts under the lateral edges of the nail bed.
Habit tic deformity:
Habit-tic deformity, a relatively common nail disorder. It is caused by the
conscious or unconscious rubbing and picking of the proximal nail fold and cuticle
area. Habit-tic deformity is most often found on the thumb of the dominant hand,
which is being rubbed by the index or middle finger of that same hand. Other fingers
may become involved, especially during times of stress. The resulting deformity is a
washboard nail which consists of depression down the center with numerous
horizontal ridges extending across the nail. While habit-tic deformity is considered to
be a compulsive disorder and medications such as selective serotonin reuptake
inhibitors may be used.
Ingrowing toe nail
The soft tissue at the sides of the nail (lat nail fold) is penetrated by the edge of the
nail plate resulting in pain, sepsis and later formation of granulation tissue. Most
common cause in ill fitting footwear. Ingrowth toenails are common in adults but
uncommon in children and infants. They are more common in men than in women.
Teenagers and young adults are most at risk. Any toenail can become ingrown, but the
condition is usually found in the big toe.
Signs and symptoms:
Pain and tenderness in your toe along one or both sides of the nail
Redness around your toenail
Swelling of your toe around the nail
Infection of the tissue around your toenail
Common causes include:
Wearing shoes that crowd toenails.
Cutting toenails too short or not straight across.
Injury to toenail.
Unusually curved toenails.
Thickening of toenails.
Treatment: If no acute infection is found, conservative treatment is recommended.
This consists of warm soaks, proper shoes, and frequent cleaning of the nail.
Excessive granulation tissue is cauterized with silver nitrate. If an infection is present,
then surgical removal of either part of the nail or the whole nail and drainage of the
abscess will be needed. Removal of the nail with phenolization of the relevant part of
matrix can be done. C02 laser has also been used.
Median nail dystrophy of Heller (Dystrophia unguis mediana canaliformis,
Solenonychia)1
In this condition there is a single longitudinal defect in the nail. This condition is
uncommon and is of unknown cause. Usually it affects the thumbnails. The defect
starts at the cuticle and grows out to the distal edge. Extending laterally from the
center are often a few cracks projecting towards but not quite reaching the free edge
of the nail resembling an inverted fir tree.
Onychogryposis38
Onychogryphosis is an exaggerated enlargement of the nail plate. In most
often only involves the great toenails. However, it may occur on the fingernails.
Onychogryphosis is common in older individuals. This condition can also be observed
in individuals who are homeless, have senile dementia, or are infirm.51, 52
The primary predisposing factor for onychogryphosis is inadequate foot and
nail care.
Secondary factors that can influence the development of onychogryphosis
include continuous pressure and friction on the toenails due to improper footwear, a
previous history of nail trauma, hypertrophy of the nail bed, biomechanical bony
abnormalities such as hallux valgus, impairment of the vascular and neuronal system,
and onychomycosis.53
Clinically, the shape of onychogryphotic nails appears 'oyster like' or 'ram's
horn-like'. The nail plate is typically uneven, thickened, and brown to opaque.
Multiple transverse striations are often present. In addition, the underlying nail bed
may also be hypertrophic.54
Treatment: Definitive therapy involves nail avulsion and matrix ablation.
Skier's toe footballer's toe.
A subungual hematoma may form from chronic repetitive trauma to the nail,
typically minor shearing damage as occurs when a skier's toe repeatedly presses
against the ski boot or when a football is kicked. It is often bilateral and usually
affects only each hallux.
No treatment is needed.
Splinter hematoma
Splinter hemorrhages are formed by the extravasation of blood from the
longitudinally oriented vessels of the nail bed. The blood attaches itself to the
underlying nail plate and moves distally.
The splinter hemorrhages occasionally appear to remain stationary, probably
because of attachment to the nail bed rather than to the plate.
Splinter hemorrhages can be caused by physical factors, including trauma,
drugs, dermatologic diseases, systemic diseases, and idiopathic conditions, among
others. Trauma is by far the most common cause.55'56At times, certain presentations
of splinter hemorrhages should make one consider a systemic cause, particularly
bacterial endocarditis57. Their simultaneous appearance in multiple nails is more
frequently associated with systemic disease. Also, their occurrence closer to the lunula
as opposed to the distal nail plate seems to be more directly correlated with systemic
disease.55,56
Miscellaneous information about splinter hemorrhages:
1.
Splinter hemorrhages are more common in the elderly, are more common in
Black people, and are located distally.
58,59
In one study, the left hand was involved greater than three times more
frequently than the right hand in patients with single hemorrhages; the left
thumb was the digit most frequently involved.60
3.
In patients with multiple hemorrhages, more occurred on the left hand than the
right hand. The thumb was the most frequently involved digit, followed by the
left index finger and the left thumb.60
4.
In one study, peritoneal dialysis was the single most frequently encountered
factor in patients with splinter hemorrhages.61
5.
Biopsy of splinter hemorrhages that are associated with trichinosis may reveal
the organism.
6.
7.
It is the commonest nail injury. If injury is under the exposed nail the bleeding will be
immediately apparent but if the injury is below the dorsal nail fold the injury may not
be visible for 2/3 days and will move forward with the growth of the nail.
Haemorrhage in the matrix is incorporated in the nail plate. While one distal to the
lunula remains subcuticular unless removed. The possibility of underlying fracture
must consider for larger hematoma.
Types, effect and therapy of acute injury
Type
Effect
1
Small hematoma with small break in
Therapy
Fenestration of nail
nail bed
Large hematoma with significant nail
Remove nail
3
4
5
bed injury
Large hematoma nail plate displaced
Severe crush injury
Amputation of tip of digit
Treatment if more than 25% of the visible nail is affected the nail plate should be
removed. Reduction of pressure is carried out by making a small puncture hole
through nail plate with a hot cautery point.
Pits and ridges: are caused due to trauma to matrix. The associated injuries are usually
the severe and damage to the nail is not appreciated for months.
reported case is 1:2. Average patients are in their twenties, but many teenagers are
affected.
Cutical: The dorsal, medial aspect of the hallux is the most common site. The lesion
appears a pinkish nodule under the free end of the nail plate. Elevation and dystrophy
of overlying nail plate may occur.
Treatment is surgical.
Ganglion cyst64
This entity is highly connected with osteoarthritis, especially in women. Location on
the fingers is most common; involvement of the toes is possible. Anatomically, it
represents the collection of gelatinous material in a cavity within connective tissue, in
connection with the distal interphalangeal joint (DIJ) and lacking epithelial lining.
Located on the proximal nail fold, half way between the DIJ and the cuticle, it
presents as a skin-colored. Smooth surfaced, translucent, dome-shaped
swelling, with a greater or lesser tendency to spontaneous discharge.
Located more distally beneath the proximal nail fold, it faces a longitudinal
groove on the nail plate due to compression of the cyst on the matrix. This
gutter may exhibit transverse grooves of various depths acknowledging for the
successive swelling and discharge episodes of the cyst.
Location beneath the nail matrix is unusual and produces several nail
dystrophies such as red-blue lunula, proximal lamellar splitting, nail fissure or
even pincer nail.
Treatment
Many treatments have been proposed for the condition but best results are
obtained with surgery.
For patients not willing to undergo surgery two other simple techniques are
recommended:
Glomus tumor38
Glomus tumor can occur anywhere on the body but up to 75% are found in the
hand, and the vast majority is located on the finger tips. It arises from the
neuromyoarterial glomus cells of the nail bed dermis. Amazingly, this condition is
encountered in females around 45 years old in almost 90% of cases. The triad of pain,
cold intolerance, and pin-point tenderness is highly suggestive of the condition.
Subjective symptoms typically exceed clinical sings. Pain is the leading symptom; it
may be excruciating and irradiating. Pain is the leading symptom; it may be
excruciating and irradiating proximally.
MRI is the best imaging technique for demonstrating the tumor.
TREATMENT
Treatment is the surgical removal of the tumor.
Pyogenic granuloma38:
Pyogenic granuloma is a benign vascular tumor, mostly seen in the early
decades, usually presenting as a rapidly evolving, solitary, sessile or polypoid vascular
nodule prone to ulceration or hemorrhage. Its precise pathogenesis remains unclear.
Location on the nail apparatus is a common finding: Pyogenic granuloma formation is
most commonly due to trauma on the fingernails; the nail-plate-lateral- sulcus
interaction is responsible for their arising on the toenails.
Several systemic drugs have been responsible for pyogenic granulomas in the
lateral nail sulcus: indinavir, retinoids, cyclosporine. lamivudine, capecitabine. Cast
immobilization and friction from footwear have been reported as causing pyogenic
granulomas .
Treatment
Treatment consists in curettage or C02 laser vaporization of the lesion under
local anesthesia. In growing toenail resulting from improper nail trimming, curettage
must be completed by the resection of the offending spur.
Fibrokeratomas38:
The acquired digital fibrokeratoma (FK) is a benign fibroepithelial growth of
unknown origin. Localization in the nail apparatus is common. FKs are usually
unique, more common in men over 50 years old and do not resolve spontaneously.
Trauma has often been considered as a trigger of this overgrowth but some reports
suggest a hamartomatous origin. They are pinkish elongated tumors often associated
with a hyperkeratotic tip.
Clinical features depend on the location of the tumor:
Developed from the ventral aspect of the proximal nail fold it causes a
longitudinal depression on the nail plate due to compression on the underlying
matrix. The tumor may be hardly visible as it may be covered by the proximal
nail fold. Otherwise it rests within the gutter.
Originating from the dermis beneath the nail matrix it may be responsible for
permanent nail dystrophy (even after excision of the tumor).
Originating from the nail bed it pushes up the nail plate creating a prominent
ridge. Subungual filamentous tumors are probably a very thin variant of that
type
Blurred borders
Trichophyton
rubrum
Trichophyton
mentagrophytes
Epidermophyton
floccosum
Nondermatophyte:
Acremonium
Aspergillus species
Cladosporium carrionii
Fusarium species
Onycochola Canadensis
Scopulariopsis brevicaulis
Scytalidium dimidiatum\
Scytalidiuni hyalinum
Yeasts:
Candida
albicans
Toenails are about 25 times more likely than fingernails to be infected, and
dermatophyte fingernail involvement rarely occurs without toenail involvement. The
longest toe either the first or the second, which bears the brunt of pressure and trauma
from footwear, is particularly susceptible to invasion although multiple nails are
typically infected. Some authors believe that eliminating the instigating facto** may
T. megninii have also been reported to cause the condition. The fungus initially
invades the stratum corneum of the proximal nail fold and subsequently penetrates the
newly formed nail plate. The clinical result is a white discoloration under the
proximal nail plate in the area of the lunula; the distal nail unit remains normal. As
opposed to WSO the nail plate is intact. Subungual hyperkeratosis, onychomadesis
and eventual destruction and shedding of the entire nail plate may occur in advanced
disease. Because it is infrequent, some authors believe that a preceding episode of
trauma is a prerequisite for it to occur in immunocompetent patients.
Proximal white subungual onychomyscosis (PWSO) has been described with
increasing frequency in patients with acquired immunodeficiency syndrome (AIDS).
In fact, this rare form of onychomycosis was seldom encountered before AIDS
became prevalent.83
T. rubrum is the most common pathogen.
OTHER FUNGAL INFECTIONS
Non-dermatophytic molds cause 1.5-6% of onychomycosis. In the study by
Summerbell and colleagues, non-dermatophyte molds constituted up to 3.3% of the
organisms cultured from nail infections.84
Investigations:
1) Direct microscopy
2) A 20% potassium hydroxide (KOH) preparation in chlorazole black-E(CBE) is
a useful screening test to rule out the presence of fungi.
In CBE, specimen should be obtained, from the nail bed by curettage. It should be
obtained at a site most proximal to the cuticle, where the concentration of hyphae is
greatest. In PSO, the overlying nail plate must initially be pared with a number-15
blade. Then, a sample of the proximal nail-bed may be taken. A number -15 blades
may also be used to remove a specimen from the nail surface in WSO. Specimens
suspected of candidal OM should be taken from the affected nail bed closest to the
proximal and latheral edges. CBE is tris-azo dye selective for chitin. It stains fungal
element green- blue against black gray background making visibility of hyphae
prominent and easy.85
Culture
Direct microscopy cannot identify the specific pathogen involved in
Onychomycosis. A fungal culture must be used to identify the species of organism.
Nondermatophyte molds may be resistant to the conventional therapy used for the
more common dermatophytes. Therefore, 2 types of growth medium should be used,
one with cycloheximide (dermatophyte test medium [DTM], Mycosel, or Mycobiotic)
to select for dermatophytes and one without cycloheximide (Sabouraud glucose agar.
Littman oxygall medium, or inhibitory mold agar) to isolate yeasts and
nondermatophyte molds. Cultures should be obtained from pulverized nail scrapping
or clipping while the patients has abstained from antifungal medication for at least 2
weeks.
Treatment is with oral antifungals like griseofiilvin, fluconazole, itraconazole,
terbinafine.
Scabies:
Nail changes are found in crusted scabies. The subungal material contains
abundant mites and may be a source of dissemination of disease.
Syphilis:
Primary Syphilis: Chancre can present as periungal erosion or ulcer.
Secondary syphilis: Nails are brittle and tend to split (onyxis craquele). Linear pitting,
elkonysis, onychlysis, beaus lines can also be seen.
Tertiary syphilis: Amber colored nails can sometime be seen.
Periungal warts(Subungual warts)38
These are the most common tumor involving the nail unit. They are caused by
various human papilloma virus. They present as hyperkeratotic papules showing a
rough surface. Most commonly they are located on the nail folds (proximal and
lateral) but sometimes extend to the nail bed with associated onycholysis. Fissuring of
the hyperkeratosis may be painful. Distortion of the nail plate is exceptional. Nail
biting enhances spreading on other fingernails. Warts are very common and hard to
treat in the immunosuppressed, particularly in organ-transplant recipients.
The most significant lesion from which warts need to be distinguished is
epidermoid carcinoma (Bowen's disease).
Wide range of therapies is available, including keratolytics combined with
abrasion, cryosurgery, bleomycin puncture, imiquimod, laser therapy, interferon and
immunotherapy using sensitization to diphencyprone, naturally acquired immune
sensitivity to pathogens.
Longitudinal melanonychia
Longitudinal melanonychia, brown-pigmented longitudinal streaks of the nail,
may be a normal variant in darker-skin individuals or they may actually be nevi. Less
commonly, they may represent Addison's disease, acanthosis nigricans, Peutz-Jeghers
syndrome, trauma, subungual hemorrhage and fungal infection, or even an underlying
melanoma, especially when they occur on the thumb (most common site for
melanoma of the nail unit). Black discoloration of the proximal nail fold at the base of
the pigmented streak (Hutchinson's sign) is an ominous sign for melanoma.
Longitudinal melanonychia in one nail without an obvious explanation warrants a
biopsy of the nail matrix.
Nail changes following systemic drug use1
Systemic drugs can produce nail abnormalities.asymtomatic growth rate changes &
pigment abnormalities are the most common changes . the nail changes observed with
commonly used drugs are as follows.
Calcium Channel
blockers
Captopril
Losartan
Cancer chemotherapy
Carbamazepine
Phenytoin (use in
pregnancy)
Chloroquine
Mepacrine
Quinine
AZT
Lamivudine
growth
Paronychia,
Psoralens
melanonychia
Photo-onycholysis,
longitudinal
pseudopyogenic
pain,
granuloma,
splinter
melanonychia,
proximal
longitudinal
hemorrhages,
nail
plat
Oral contraceptives
Vitamin A toxicity
Retinoids
(sometimes
painful),
paronychia,
Aspirin
Ibuprofen
Cyclosporine
Sirolimus
EGFR inhibitors
(imatinib, sorafenib,
sunitinib, gefitinib,
cetuximb)
Thiazide diuretics
Onycholysis
Therapeutic agents
Penicillamine
Salbutamol
Phenolphthalein
Cephalexin
Cloxacillin
Chloramphenicol
Tetracyclines
Fluoroquinolones
Clofazimine
Itraconazole
Fluconazole
Ketoconazole
Sulfonamide
(including
dapsone)
hypersensitivity
Nail changes secondary to systemic chemical poisoning
Amiline
Arsenic
Blue-violet nails
Mees' lines, Beau's lines, onychomadesis, longitudinal
brown
bands,
diffuse
melanonychia,
subungual
Gold
Carbon monoxide
Fluorine
Lead
Mercury
Paraquat
hyperkeratosis
Nail pigmentation, fragility, onycholysis
Cherry red nail beds and lunulae, leukonychia
Longitudinal brown bands
Onychomadesis, onychalgia, leukonychia
Ridging, fragility, dark discoloration of the nail plates
Greenish-black nails, white bands, brown bands, softening
Selenium
Silver
Thallium
CLASSIFICATION 2
Abnormalities of shape
Clubbing:
Since Hippocrates first described digital clubbing in patients with empyema, digital
clubbing has been associated with various underlying pulmonary, cardiovascular,
neoplastic, infectious, hepatobiliary, mediastinal, endocrine, and gastrointestinal
diseases. Finger clubbing also may occur, without evident underlying disease, as an
idiopathic form or as a Mendelian dominant trait. Clubbing is a clinically descriptive
term, referring to the bulbous uniform swelling of the soft tissue of the terminal
phalanx of a digit with subsequent loss of the normal angle between the nail and the
nail bed.
Digital clubbing is classified into primary (i.e., idiopathic, hereditary) and secondary
forms. Digital clubbing may be symmetric bilaterally, or it may be unilateral or
involve a single digit. Anatomic considerations, such as the classic measurement of
the Lovibond angle or the more recently derived index of nail curvature by Goyal et
al.86
Usually can be identified on simple physical examination and can be used to identify
digital clubbing and to monitor this dynamic process objectively Although clubbing is
a common physical finding in many underlying pathological processes, surprisingly,
the mechanism of clubbing remains unclear. Different pathological processes may
follow different pathways to a common end. Many studies have shown increased
blood flow in the clubbed portion of the finger. Whether the vasodilatation results
from a circulating or local vasodilator, neural mechanism, response to hypoxemia,
genetic predisposition, or a combination of these or other mediators is not agreed on
currently.87
definitive clubbing exists. An angle between 160-180 falls in a gray area and
may indicate early stages of clubbing or a pseudoclubbing phenomenon.
Mees lines are transverse types of true leukonychia associated with systemic disease.
Arsenic intoxication was classically believed to be the major cause of Mees lines. It is
now well know that numerous severe systemic insults may be the stimuli to initiate
the abnormality. 100 It has been said that Mees lines that occur as a result of arsenic
poisoning are due to actual deposition of arsenic in the nail plate.
101
One may
approximate the time of onset of systemic illness by measuring the distance from the
Mees line to the proximal nail fold, as one can do with Beau's lines.
Leukonychia totalis
Leukonychia totalis is a rare nail disorder which may be hereditary or acquired.
Hereditary or congenital leukonychia, 102 is present since birth, with positive family
history and has autosomal dominant inheritance. Acquired leukonychia totalis appears
in early childhood .102 The white nail has been associated with various diseases or
with occupation. Frequently the cause may be obscure. Albright quoted Giovannini
that leukonychia totalis develops after typhoid fever. Baran and Drawber also reported
leukonychia after infectious diseases such as measles and herpes zoster.103
The other causes may be leprosy, exposure to extreme cold, hepatic cirrhosis,
ulcerative colitis, onychophagia, anemia, hypoproteinemia or occupational trauma.
Longitudinal melanonychia
Longitudinal melanonychia, brown-pigmented longitudinal streaks of the nail, may be
a normal variant in darker-skin individuals or they may actually be nevi. Less
commonly, they may represent Addison's disease, acanthosis nigricans, Peutz-Jeghers
syndrome, trauma, subungual hemorrhage and fungal infection, or even an underlying
melanoma, especially when they occur on the thumb (most common site for
melanoma of the nail unit). Black discoloration of the proximal nail fold at the base of
the pigmented streak (Hutchinson's sign) is an ominous sign for melanoma.
Longitudinal melanonychia in one nail without an obvious explanation warrants a
biopsy of the nail matrix.
Muehrcke's Lines
Muehrcke's lines are double white transverse lines that represent an abnormality of
the nail vascular bed. Squeezing the distal digit will cause the lines to disappear
temporarily. They are not palpable and do not indent the nail, and it has been noted
that they are usually found on the second, third, and fourth fingernails.104 They
sometimes occur when chronic hypoalbuminemia persists and tend to disappear when
the serum albumin is above 2.2 g/100 ml.
A number of disease states causing hypoalbuminemia may be associated with
Muehrcke's lines, such as the nephrotic syndrome and glomerulonephritis. Liver
disease and malnutrition are .among those that have been mentioned.
Half-and-Half (Lindsay's Nails)
Lindsay's nails (half-and-half nails) are associated with chronic renal insufficiency
(see p. 164). They are form of apparent leukonychia exhibiting either a whitish, or
normal proximal half and a distinctly abnormal brownish distal portion. This distal
portion begins proximally where the normal or whitish nail ends and terminates
distally where the free end of the nail loses its attachment to the hyponychium. 105, 106
Lindsay's description, as well as crescents, are seen frequently in renal failure but nor
infrequently otherwise.107 Psoriasis is the most common cause of a pseudo half-andhalf nail appearance.
Terry's Nails
In 1954, Terry described apparent leukonychia over the entire nail bed with narrow
distal pink band in 82 of 100 cirrhotic patients.108 In a large prospective study,
Holzberg and Walker revised the description to include a pink brown band 0.5-3:0
mill wide, which histologically demonstrated telangectasias in the dermis,
109
Their
study showed association with cirrhosis, congestive heart failure, diabetes mellitus,
and age. When seen in younger individuals, Terry's nails should prompt consideration
of an investigation for systemic disease.
Thyrotoxicosis, pulmonary eosinophilia, malnutrition, or 'keratoses' were found in
other patients who had Terry's nails but who did nor exhibit cirrhosis.110 We have
noticed numerous patients who had a similar nail appearance (but who never had liver
disease). And we concluded that this disorder is a reaction pattern and is not
pathognomonic for cirrhosis (also see the following discussion of eiyrhematous
crescents).
Melanonychia:
Nails that show bands of black or brown pigmentation. This colorization can be
localized or diffuse. In ethnic groups with more pigmented skin such as black, Asians
and Hispanics this is a common finding. It is also seen in acanthosis nigricans,
secondary to medication (minocycline, azidothymidline, antimalarials). In higher skin
types these pigmented bands are associated with nevi or melanoma.
OBSERVATION
&
RESULTS
139
111
250
55.6
44.4
100
No of patients
81
39
35
30
25
20
20
250
Percentage
32.4
15.6
14.0
12.0
10.0
08.0
08.0
100.0
Complaints (table 3)
Complaints
Discoloration
Pain & swelling
Physical change
Total
No of patients
39
28
26
93
Percentage
15.6
11.2
10.4
37.2
No of patients
64
27
10
09
110
Percentage
25.6
10.8
04.0
03.6
44.0
No of patients
106
67
52
25
250
Percentage
37.2
26.8
20.8
10.8
100
No of patients
13
64
6
130
37
250
Percentage
5.2
25.6
2.4
52
14.8
100
No of
Percentage
patients
Anonychia
Darriers disease
Epidermolysis bullosa
Pachyonychia congenita
Tuberous sclerosis
02
05
02
01
03
00.8
02.0
00.8
00.4
01.2
Psoriasis
Eczema
Lichen planus
Alopecia areata
Vesiculobullous disease
Collagen vascular disease
Reiters disease
Drug reactions
25
11
10
09
02
02
03
02
10.0
04.4
04.0
03.6
00.8
00.8
01.2
00.8
Subungal hematoma
Ingrowing toe nail
Onychogryphosis
03
02
01
01.2
00.8
00.4
Warts
Bacterial Infection
Fungal infection
02
17
111
00.8
06.8
44.4
Koilonychia
Platynychia
Clubbing
Melanonychia
Total
13
05
04
15
250
05.2
02.0
01.6
06.0
100
No of patients
5
3
2
1
2
12
Percentage
2.0
1.2
0.8
0.4
0.4
4.8
No of patients
25
11
10
09
03
02
02
02
64
Percentage
10.0
04.4
04.0
03.6
01.2
00.8
00.8
00.8
25.6
No of patients
15
10
09
07
06
06
04
03
Percentage
60
40
36
28
24
24
16
12
No of patients
4
2
2
2
1
1
Percentage
1.6
0.8
0.8
0.8
0.4
0.4
No of patients
Percentage
Subungal hematoma
1.2
Pterygium
0.8
0.8
Onychogryphosis
0.4
Total
3.2
No of patients Percentage
Koilonychias
13
5.2
Leukonychia
09
3.6
Clubbing
04
1.6
Total
26
10.4
No of patients
111
17
02
130
Percentage
44.4
6.8
0.8
52.0
No of
patients
72
11
11
10
07
Percentage
64.86
09.9
09.9
09.0
06.34