Nails Abnormalities

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Hair, nails and mucous membranes SECTIONC11

hapter
Antonella Tosti and Bianca Maria Piraccini
Chapter Contents
Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1129
Nail signs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1129
Congenital and hereditary nail diseases . . . . . . . . . . . . . . . . . . . . . . . . 1134
The nail in dermatologic diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1136
The nail in systemic diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1138
Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1141
Environmental nail disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1142
Traumatic nail abnormalities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1142
Nail tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1144
Anatomy (Fig. 71.1)

Key features
Nail matrix nail plate production
proximal matrix dorsal nail plate
distal matrix (lunula) ventral nail plate
Proximal nail fold nail matrix protection
Nail bed and hyponychium nail plate adhesion and distal
detachment
Nail growth rate:
fingernails: 3mm/month
toenails: 1mm/month
71
Nail Disorders
Nail Disorders
71
The nail plate is a fully keratinized structure produced by the germina

tive epithelium of the nail matrix. As it grows, the nail plate emerges
from the proximal nail fold and progresses distally, lying across and
strictly adhering to the nail bed. As the nail plate approaches the tip of
the digit, it detaches from the underlying tissues, forming the hyponych
ium. Proximally and laterally, the nail plate is surrounded by the nail
folds. The proximal nail fold consists of two layers of epithelium a
dorsal layer, which is the distal continuation of the skin of the dorsum
of the digit, and a ventral layer, which is continuous with the nail
matrix.
The nail matrix is responsible for the production of the nail plate
and consists of an epithelium that keratinizes without the formation
of a granular layer. Nail matrix keratinization occurs along an oblique
axis, reflecting the upward and distal movement of cells during the
process of maturation and differentiation1. For this reason, the proxi
mal portion of the nail matrix produces the dorsal portion of the nail
plate, while the distal matrix is responsible for the production of the
ventral nail plate. The distal portion of the nail matrix is visible through
the transparent nail plate as a white, distally convex half moon known
as the lunula. The matrix contains melanocytes that are normally
quiescent; however, they may become activated and synthesize melanin,
which is transferred to the surrounding keratinocytes. Distal migration
of melanin-containing keratinocytes then gives rise to a pigmented nail
plate2.
Nail plate production occurs continuously, proceeding from the 15th
week of embryonic life until death. Under normal conditions, the mean
growth rate of a fingernail is 3mm/month and that of a toenail is
1mm/month. Nail growth rate can be influenced by several factors,
including age, systemic diseases, and medications.
Nail signs
By clinical examination, it is possible to identify which site within the
nail apparatus is affected by a particular disease process (Table 71.1 &
THE NAIL APPARATUS
CORRELATION OF NAIL FINDINGS WITH ANATOMIC SITE OF NAIL DAMAGE

Proximal matrix
Distal matrix
Proximal nail fold
Affected site
Clinical manifestation
Proximal matrix
Beaus lines
Pitting
Longitudinal ridging
Longitudinal fissuring
Trachyonychia
Distal matrix
True leukonychia
Proximal + distal matrix
Onychomadesis
Koilonychia
Nail thinning
Nail bed
Onycholysis
Subungual hyperkeratosis
Apparent leukonychia
Splinter hemorrhages
Cuticle
Nail bed
Nail plate
Hyponychium
Distal phalanx
Fig. 71.1 Schematic drawing of the nail apparatus in longitudinal section.
Bolognia_Chapter 71_main.indd 1129
Table 71.1 Correlation of nail findings with anatomic site of nail damage.
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Section
Hair, nails and mucous membranes
11
Fig. 71.2). Recognizing specific nail signs is very important in under

standing and diagnosing nail dystrophies1,3,4. Nail signs can be sche
matically divided into three major categories:
l signs due to abnormal nail matrix function
l signs due to nail bed disorders
l signs due to deposition of pigment within the nail plate.
First described by Beau in 1846, these transverse depressions of the nail

plate surface result from a temporary interruption of the mitotic activ
ity of the proximal nail matrix. The depth of the depression indicates
the extent of the damage within the matrix; the width of the depression
(along the longitudinal axis) indicates the duration of the insult. Beaus
lines grow distally with the nail plate, with multiple lines indicating
repeated damage. Most commonly, Beaus lines are caused by mechani
cal trauma (e.g. manicures, onychotillomania) or dermatologic disease
of the proximal nail fold (e.g. eczema, chronic paronychia). The pres
ence of Beaus lines at the same level in all nails suggests a systemic
cause (e.g. severe or febrile illness, erythroderma, drugs).
Nail Signs due to Abnormal Nail Matrix Function

Beaus lines
Onychomadesis (nail shedding)
Key features

Key features
Transverse depressions
More evident in the central portion of the nail plate
Most often traumatic
Involvement of multiple digits suggests a systemic cause
Proximal detachment of the nail

Involvement of multiple digits suggests a systemic cause
Fig. 71.2 Nail signs and nail disorders.

NAIL SIGNS AND NAIL DISORDERS
Beaus lines
Onycholysis
Onychorrhexis
Onychomadesis
Onychoschizia
Trachyonychia
Pitting in psoriasis
Pitting in
alopecia areata
Lichen planus
Darier disease
Oil
drop
Leukonychia
True leukonychia
Clubbing
Normal
Pressure
Brachyonychia
Normal
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Onychogryphosis
Pincer
Yellow nail
Pterygium
Inverse pterygium
Pachyonychia
congenita
Koilonychia
Clubbing
Splinters
Onychauxis
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Chapter
71
Nail Disorders
Fig. 71.4 Nail pitting

due to psoriasis. Pits are
large, deep and
irregularly scattered, in
contrast with alopecia
areata, where they are
small, superficial and
geometrically
distributed.
Fig. 71.3 Onychomadesis after an episode of acute paronychia.
The term onychomadesis describes the detachment of the nail plate

from the proximal nail fold and is due to a severe insult that produces
a complete arrest of nail matrix activity (Fig. 71.3). Onychomadesis
appears as a sulcus that replaces the proximal nail plate. The causes of
onychomadesis are the same as those for Beaus lines4a.
Pitting
Key features
Punctate depressions of the nail plate surface
Migrate distally with nail growth
Proximal nail matrix damage by psoriasis, alopecia areata, eczema
Pitting describes the presence of small depressions on the nail plate

surface (Fig. 71.4). Pits are due to foci of abnormal keratinization of
the proximal nail matrix that result in clusters of parakeratotic cells in
the dorsal nail plate. These clusters are easily detached, leaving the
pits. Diseases that produce nail pitting include psoriasis, alopecia areata
and eczema.
Onychorrhexis
Key features

Thinning
Longitudinal ridging and fissuring
Diffuse nail matrix damage by lichen planus is a possible cause
Mild disease is a common manifestation of aging
Onychorrhexis produces longitudinal ridging and fissuring of the nail

plate. Often associated with nail thinning, it indicates diffuse damage
to the nail matrix. Diseases commonly responsible for onychorrhexis
include lichen planus, impaired vascular supply, trauma, and tumors
that compress the nail matrix. Mild longitudinal ridging of the nails is
a common feature of aging.
Trachyonychia (twenty-nail dystrophy, sandpapered nails)
Key features
Nail roughness
Often associated with thinning
Conditions that may cause trachyonychia include alopecia areata
(common), lichen planus (rare), psoriasis (rare) and eczema (very
rare)
Fig. 71.5 Trachyonychia (twenty-nail dystrophy). The nails seem sandpapered

in a longitudinal direction.
Trachyonychia is a specific nail plate surface abnormality characterized

by diffuse homogeneous roughness (Fig. 71.5). In most patients, the
affected nails are opaque, lusterless and rough; the nail plate surface
has longitudinal ridging due to fine superficial striations distributed in
a regular, parallel pattern (sandpapered nails). A less common variant,
referred as shiny trachyonychia, is characterized by multiple small
punctate depressions distributed in a geometric pattern within parallel,
longitudinal lines. Conditions that may cause trachyonychia include
alopecia areata, lichen planus, psoriasis and eczema. Some authors use
the term twenty-nail dystrophy for idiopathic cases of trachyonychia
in children (see below), even though the disease does not necessarily
involve all 20 nails.
True leukonychia
Key features
White opaque discoloration
Punctate, striate or diffuse
Distal nail matrix damage
Needs to be distinguished from apparent leukonychia (nail bed
discoloration)
Needs to be distinguished from pseudoleukonychia (nail plate
invasion by fungi), which is typical of superficial white
onychomycosis. In this case the nail plate surface is friable due to
the presence of keratin debris and fungal elements

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Section
11
The nail plate has a normal surface but loses its transparency and looks
white because of the presence of parakeratotic cells within its ventral
portion. True leukonychia is caused by diseases that disturb distal
nail matrix keratinization and it presents with three morphologic
variants:
l Punctate leukonychia. The nail plate shows small opaque
white spots that move distally with nail growth and sometimes
disappear before reaching the distal nail. It is caused by
trauma and is most commonly observed in the fingernails of
children.
l Striate leukonychia. The nail plate shows one or more transverse
white opaque parallel lines. It is frequently observed in the
fingernails of women, due to matrix trauma secondary to
manicures. It may also occur in great toenails as a consequence of
trauma from shoes. It is also typical of Mees lines, the white
transverse bands seen in arsenic and thallium poisoning5.
l Diffuse leukonychia. The nail plate is completely or
almost completely opaque and white. Total leukonychia is rare
and sometimes hereditary. It may be associated with
keratoderma and other congenital defects such as
deafness.
Koilonychia (spoon nails)
Key features
Thinned concave nails
Physiologic in the toenails of children
The nail plate is thinned, flat and spoon-shaped due to upward eversion
of its lateral edges. Although it is physiologic and resolves spontane
ously in childrens toenails, in adults it is most commonly occupational
in origin or associated with severe iron deficiency.
Nail Signs due to Nail Bed Disorders

Onycholysis
Key features
Distal nail plate detachment
Detached nail looks yellowwhite
Commonly due to environmental trauma, psoriasis or
onychomycosis
The nail plate is detached from the nail bed and appears white because
of the presence of air in the subungual space. Pigmentation of the
onycholytic area may occur as a consequence of microbial colonization
or blood extravasation. In addition to environmental trauma, the nail
bed disorders that most commonly produce onycholysis are psoriasis
and onychomycosis (Fig. 71.6). Photo-onycholysis may be precipitated
by UV light exposure, either alone or in combination with medications
such as tetracycline.
Onychauxis
Key features
Nail thickening
Commonly due to psoriasis or onychomycosis
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The nail plate appears thickened due to the presence of subungual

scales. Causes of subungual hyperkeratosis include psoriasis, onycho
mycosis and eczema.
Key features
White discoloration that fades with pressure
Nail plate transparency maintained
Often due to drugs (chemotherapeutic agents) or systemic
diseases (e.g. hypoalbuminemia)
The nails are white because of abnormalities in the color of the nail
bed and this is usually due to nail bed edema. Apparent leukonychia
does not move distally with nail growth and the white discoloration
fades with pressure (see below for different types).
Splinter hemorrhages
Key features
Thin longitudinal dark-red subungual lines
Damage to longitudinally oriented nail bed capillaries
Splinter hemorrhages appear as dark-red, thin longitudinal lines, usually
localized to the distal portion of the nail. The shape of the hemor
rhages is due to the longitudinal orientation of nail bed capillaries.
The most common causes of splinter hemorrhages are trauma, pso
riasis and onychomycosis. Proximal splinters are rare and possible
indicators of systemic diseases, including endocarditis (infectious and
marantic), vasculitis (including septic vasculitis), trichinosis and the
antiphospholipid antibody syndrome.
A recently described vascular lesion of the nail bed is the red comet,
which occurs in ~30% of patients with tuberous sclerosis complex5a.
Red comets appear as longitudinal short red streaks, partially blanch
able with compression, with an enlarged distal end; they extend from
the mid to the distal third of the nail and never reach the free edge of
the nail. Red comets are thought to represent telangiectasias plus
extravasated blood.
Nail Signs due to Deposition of Pigment

Key features
Exogenous convex proximal border
Endogenous concave proximal border (distally convex)
Subungual deposition often associated with onycholysis
Causes of nail pigmentation include staining from external pigment,
presence of pigment under the nail plate, and deposition of pigment
within the nail plate. Nail pigmentation from external staining typically
follows the shape of the proximal nail fold. Common examples are
yellowbrown nail discoloration due to nicotine in smokers or darken
ing of the nail plate due to hair dyes in hairdressers. Subungual deposi
tion of pigment is often seen with Pseudomonas colonization, where
the green discoloration of the nail results from the production of pyo
cyanin by the bacteria. Dermatophytes usually cause a yellowwhite
subungual pigmentation. The nail plate may be pigmented secondary
to melanin deposition or deposition of other pigments such as iron or
gold. A FontanaMasson stain of a nail clipping is useful in differentiat
ing melanin from other pigments.
Longitudinal melanonychia
Key features

Longitudinal brown to black band

Commonly seen in darkly pigmented individuals
Single band may be a sign of nail melanoma
Multiple bands often due to drugs or systemic disease
5/3/2012 8:17:21 PM
Chapter
Onycholysis
Most
common
etiologies
Environmental
Exposures
Irritants
Water
Trauma
UV (photoonycholysis
in the
absence of
medications)
Primary skin disorders
Infections
Drugs
Metabolic/
systemic disorders
Tumors
Psoriasis
Candida spp. (may

be secondary
invaders)
Dermatophytes
HPV
Tetracyclines
Hyperthyroidism
Subungual
exostoses
SCC
Clues to
diagnosis
History
Fingernails vs. toenails
Minimal, if any,
nail bed changes
Associated hemorrhage
Erythematous
rim to onycholysis
Additional
nail findings,
e.g. oil drop
changes, pits
Nail bed
changes, e.g.
scale
Cutaneous
lesions
Additional nail
findings, e.g.
chronic paronychia,
yellow color,
thickening
Nail bed
changes, e.g.
scale, verrucous
lesions
Green discoloration
2 to pyocyanin*
Fungal and
bacterial cultures
KOH/nail plate PAS
Fingernails
History
Multiple nails
Minimal, if any,
nail bed changes
Woods lamp
examination
Multiple nails
Minimal, if any,
nail bed
changes
Additional
cutaneous
findings
TFTs
Single nail
Nail bed
findings
Radiograph
Biopsy
specimen
Less
common
etiologies
Allergens, e.g.
formaldehyde,
mono(meth)acrylates,
cyanoacrylates
Lichen planus
Eczema
Lichen striatus
Blistering diseases
(epidermolysis
bullosa, pemphigus
vulgaris, pompholyx)
Other disorders
(ectodermal
dysplasias,
sarcoidosis,
Langerhans cell
histiocytosis, PRP,
Darier disease,
keratosis lichenoides
chronica)
Saprophytes
Scabies
Fluoroquinolones
Taxanes
Psoralens
NSAIDs
Photodynamic
therapy
Other drugs
(anthracyclines,
captopril,
chlorazepate,
etoposide,
gemcitabine,
mycophenolate,
phenothiazines,
quinine, retinoids,
sodium valproate,
thiazides,
5-fluorouracil)
Yellow nail
syndrome
PCT
Pseudo PCT
(drugs, dialysis)
EPP
Melanoma
Fibromas
Nail Disorders
71
CAUSES OF ONYCHOLYSIS
Fig. 71.6 Causes of onycholysis. *Due to secondary colonization with Pseudomonas aeruginosa. EPP, erythropoietic protoporphyria; HPV, human papillomavirus
infection; NSAIDs, nonsteroidal anti-inflammatory drugs; PCT, porphyria cutanea tarda; PRP, pityriasis rubra pilaris; SCC, squamous cell carcinoma; TFTs, thyroid
function tests.
Longitudinal melanonychia is very commonly observed in individuals

with darkly pigmented skin, and up to 90% of adult African-Americans
have one or more pigmented bands. It is seen much less frequently in
Caucasians. Longitudinal melanonychia is due to the presence of
melanin within the nail plate, and it may be caused by simple activa
tion of nail matrix melanocytes or by melanocyte hyperplasia as in a
lentigo, nevus or melanoma.
Clinically, melanonychia presents as one or more longitudinal pig
mented bands extending from the proximal nail fold to the distal
margin (Fig. 71.7)6. The band of melanonychia can vary in color
from light brown to black and the pigmentation may be homogene
ous or variable. The width ranges from a few millimeters to the
whole nail width. Multiple bands are usually due to melanocyte acti
vation (Table 71.2).
A range of dermoscopic patterns has been described for longitudinal
melanonychia (e.g. regular, thin, parallel light brown lines reflecting
melanocytic activation), but their accuracy with regard to the diagnosis
of subungual melanoma has not been established (see below)7,8.
Nonetheless, epiluminescence microscopy is increasingly being per

formed and further studies correlating dermoscopic patterns with
histopathologic findings are anticipated. In addition, intraoperative
dermoscopy is being employed to assist in determining margins for
excision8a.
A detailed description of biopsy procedures for longitudinal melano
nychia is found in Chapter 149 (see Fig. 149.17).
Hutchinsons sign
Key features
Brownblack periungual pigmentation
Possible sign of nail melanoma
Needs to be distinguished from pseudo-Hutchinsons sign (dark
bands producing illusory pigmentation of the proximal nail fold
due to cuticle transparency)
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Section
11
CAUSES OF LONGITUDINAL MELANONYCHIA

Melanocyte activation
Racial
Trauma
manicure
nail biting/onychotillomania
frictional, primarily in toenails
Drugs
cancer chemotherapeutic agents, e.g.
doxorubicin, 5-fluorouracil
zidovudine (AZT)
psoralens
Radiation
Pregnancy
LaugierHunziker syndrome/PeutzJegher
syndrome
Addisons disease
HIV infection
Postinflammatory
lichen planus
pustular psoriasis
onychomycosis (T. rubrum and
Scytalidium spp.)
Non-melanocytic tumors
Bowens disease
Verrucae
Basal cell carcinoma
Subungual keratosis
Myxoid cyst
nail plate
Melanocyte hyperplasia
Nail matrix nevus
Nail matrix melanoma
dorsal matrix
Table 71.2 Causes of longitudinal melanonychia.
First described by Hutchinson in 1886, this eponymous sign represents

pigmentation of the proximal nail fold or the hyponychium in associa
tion with longitudinal melanonychia. Hutchinsons sign, in the setting
of nail melanoma, is due to horizontal growth of the tumor. Although
pigmentation of the proximal and lateral nail fold or hyponychium is
a feature of congenital melanocytic nevi9, the development in adults of
periungual pigmentation in conjunction with longitudinal melano
nychia is very suggestive of melanoma.
Green nail syndrome
Key features
Greenish-black or greenish-blue nail plate discoloration due to
pyocyanin
Predisposing factors include exposure to water, detergents and
soaps
Needs to be distinguished from a hematoma, melanocytic nevus,
melanoma or an Aspergillus infection
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In green nail syndrome, the nail has a greenish-black or greenish-blue

color due to the deposition of pyocyanin, a bluegreen pigment pro
duced by Pseudomonas aeruginosa, underneath the onycholytic nail
plate (see Ch. 74). It is often accompanied by paronychia. Predisposing
factors include prolonged exposure to water, use of detergents and
soaps, nail trauma and other causes of onycholysis. Not surprisingly,
this disorder is seen in barbers, dishwashers, bakers and medical per
sonnel. Green nail syndrome is important because affected hospital
staff may spread Pseudomonas aeruginosa to debilitated patients. The
diagnosis is usually clinical, and, if necessary, can be confirmed by
Gram stain and culture of exudate and nail fragments. Treatment
involves avoiding predisposing factors, wearing protective gloves, clip
ping back of the nail, and the use of topical solutions of 2% sodium
hypochlorite or acetic acid for 14 months.
intermediate
matrix
Fig. 71.7 Longitudinal melanonychia due to a congenital melanocytic nevus

of the nail matrix. A The pigmentation involves approximately 75% of the nail
plate and the color varies from light brown to black. B The biopsy specimen
shows nests of melanocytes within the basal layer and lower portion of the nail
matrix epithelium. The cleft (*) represents an artifact.
Congenital and hereditary nail diseases

The congenital and hereditary nail diseases include a number of condi
tions in which nail abnormalities are present at birth or develop during
infancy. In some cases, the nail abnormalities are key features for the
diagnosis of syndromes or hereditary diseases.
Congenital Malalignment of the Great Toenails

Key features
Lateral deviation of the nail plate
Often bilateral
Predisposing factor for ingrown toenails
Congenital malalignment of the great toenails was first described by
Samman in 1978 and well characterized by Baran etal. in 1979. It is
common, but no prevalence data are available. Congenital malalign
ment of the great toenails is possibly caused by an abnormality in the
ligament that connects the matrix to the periosteum of the distal
phalanx. The nail plate of the hallux is laterally deviated with respect
to the longitudinal axis of the distal phalanx. This results in nail matrix
damage with Beaus lines and onychomadesis (Fig. 71.8). The nail plate
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Chapter
71
Nail Disorders
Fig 71.8 Congenital

malalignment of the
great toenails. The
longitudinal axis of the
nail plate is deviated
laterally. There is also
onychomadesis of both
great toenails and mild
nail thickening with
lateral ingrowing of the
right great toenail.
is often thickened and transversely overcurved. Congenital malalign

ment of the great toenails is frequently bilateral and is the most
common cause of ingrown toenails in children and adolescents. The
condition often improves spontaneously10.
Congenital Hypertrophy of the Lateral Fold

of the Hallux
Key features
Hypertrophic lateral nail fold
Newborns and infants
Hypertrophic lateral nail folds in newborns were first described by

Martinet etal. in 1984. The hypertrophy is usually present at birth; it
is characterized by an overgrowth of the soft tissue of the internal
portion of the lateral nail fold, resulting in a hypertrophic lip that par
tially covers the nail plate. This abnormal growth may deviate the nail
laterally and/or cause nail embedding with an acute inflammatory reac
tion and pain. The abnormality is usually bilateral and may regress
spontaneously after a few years11.
Racquet Thumbs (Brachyonychia)

Key features
Congenital
Broad and short thumbnail
Racquet thumb is a common malformation due to shortening of the distal

phalanx. It is usually inherited as an autosomal dominant trait. The nail
is shortened and abnormally wide. Racquet nails are usually an isolated
finding and radiologic examination demonstrates a short distal phalanx.
NailPatella Syndrome (Onycho-osteodysplasia;

Fong Disease)
Key features
Hypoplasia of the radial side of the thumbnails
Triangular lunulae
Presence of bone abnormalities
Nailpatella syndrome is an autosomal dominantly inherited condition

first described by Chatelain in 1820. It has been associated with muta
tions in the LMX1B gene, which encodes a transcription factor that
regulates collagen synthesis. The condition most frequently involves
the thumb and may also involve the other fingers but to a lesser extent
(Fig. 71.9). The nails are absent or hypoplastic, and the dystrophy is
usually more marked on the radial side of the digit. A triangle-shaped
Fig. 71.9 Nail hypoplasia in nailpatella syndrome. There is hypoplasia of the

nails of the first and second digits. This gradient of severity of nail involvement
is characteristic.
lunula is commonly observed. The nail changes are typically associated

with bony abnormalities, including absent or hypoplastic patellae, radial
head dysplasia, and iliac crest exostoses (horns). In children, the
diagnosis is best confirmed by the presence of iliac horns on pelvic X-ray.
Nephropathy develops in approximately 40% of patients. This leads
to renal insufficiency in up to 8% of cases.
Epidermolysis Bullosa
Key features

Periungual/subungual blistering
Periungual granulation tissue
Onycholysis
Nail thickening and shortening
Pterygium and nail atrophy
Nail abnormalities are common in all forms of epidermolysis bullosa

(EB). Repeated blistering produces onycholysis with shortening and
thickening of the nail due to nail bed scarring. Involvement of the
matrix can result in nail thinning and atrophy. Dystrophic or absent
nails with periungual granulation tissue are characteristic of junctional
EB, Herlitz type and laryngo-onycho-cutaneous syndrome. Nail abnor
malities may precede the development of skin blistering, as in late
onset junctional EB and pretibial dominant dystrophic EB, or the nail
dystrophy may be an isolated finding, as in some families with domi
nant dystrophic EB.
Ectodermal Dysplasias
Key features
Abnormal development of hair, teeth and/or eccrine glands
Nail shortening and thickening
Nail hypoplasia
Ectodermal dysplasias were first described by Weech in 1929 and clas

sified by Freire-Maia in 1977. Nail abnormalities are an important sign
in a large number of these syndromes, where they are associated with
hair, teeth and/or eccrine gland abnormalities (see Ch. 63). Most fre
quently, the nails are short and thickened with onycholysis. All the
fingernails and toenails are usually affected.
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11
Pachyonychia Congenita
Key features

Thickened and extremely hard nails

Severe nail bed hyperkeratosis
All digits eventually involved
Palmoplantar keratoderma
Pachyonychia congenita was first described by Jadasson and Lewan

dowski in 1906. Pachyonychia congenita is due to mutations in keratin
genes KRT6a and KRT16 in type I and KRT6b and KRT17 in type II.
Nail abnormalities are present in both of the two major forms of
pachyonychia congenita. The nails are thickened with an increased
transverse curvature due to severe nail bed hyperkeratosis. Nail trim
ming is extremely difficult. Associated findings include hyperhidrosis,
oral leukokeratosis, follicular hyperkeratosis and palmoplantar kerato
derma, with pain on ambulation. Premature dentition and piloseba
ceous cysts are observed more frequently in type II (see Table. 58.4).
Darier Disease (Follicular Dyskeratosis)

Key features
Red and white longitudinal streaks
V-shaped indentation (notching) of the distal margin
Multinucleated giant cells in the nail bed epithelium
Nail abnormalities of Darier disease are common and diagnostic, and
were first described by Ronchese in 1965. The nail plate has multiple
red and white longitudinal streaks (Fig. 71.10). Distally, wedge-shaped
subungual hyperkeratosis and fissuring of the free margin of the nail
plate are also seen. The latter occur in conjunction with the white and
red bands. Histologically, there is hyperplasia of the nail bed epithelium
with multinucleated epithelial giant cells, but not suprabasal clefts12.
Nail changes do not improve with oral retinoid therapy. Similar nail
abnormalities may be seen in patients with HaileyHailey disease. A
single, red longitudinal band (longitudinal erythronychia) with distal
subungual hyperkeratosis is not sufficient for diagnosing Darier disease
of the nails, since single bands may be due to a subungual benign tumor
(onychopapilloma) and, less often, Bowens disease13,13a.
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Psoriasis
Key features
Several nails affected
Diagnostic signs (fingernails only): irregular pitting, salmon
patches (oil drop sign), onycholysis with an erythematous border
In toenails, psoriasis is usually clinically indistinguishable from
onychomycosis
Often associated with psoriatic arthropathy
The Koebner phenomenon worsens nail symptoms
Nail abnormalities are present in up to 50% of patients with psoriasis

and may be the only manifestation of the disease. Nail psoriasis is often
associated with psoriatic arthritis and enthesitis14. Clinical findings
that are diagnostic for nail psoriasis include irregular pitting, salmon
patches of the nail bed, and onycholysis with an erythematous border.
These signs are often seen together in the same patient and are local
ized to the fingernails. Psoriatic pits are large, deep, and irregularly
scattered within the nail plate (see Fig. 71.4); they may be covered by
whitish, easily detachable scales. The oil drop sign (salmon patches)
appears as an irregular area of yelloworange discoloration visible
through the nail plate (Fig. 71.11). Onycholysis surrounded by an ery
thematous border is also typical in nail psoriasis. Patients with psoriasis
often have other nail abnormalities that are not diagnostic, as they are
commonly seen in other conditions. These include splinter hemor
rhages, subungual hyperkeratosis, nail plate thickening and crumbling,
and paronychia.
Diseases that should be differentiated from nail psoriasis are listed
in Table 71.3.
Treatment
Nail psoriasis rarely responds to topical treatment and is often aggra
vated by sun exposure. Treatment measures are reviewed in the thera
peutic ladder (Table 71.4).
Acrodermatitis Continua of Hallopeau

(Hallopeaus Acrodermatitis, Dermatitis Repens)
The nail in dermatologic diseases
Key features
Several dermatologic diseases often have associated nail abnormalities

that may prove helpful in confirming the clinical diagnosis.
Recurrent, self-limited episodes of painful inflammation

Pustules involving the nail bed and periungual skin
Fingernails most commonly affected, often one digit
Fig. 71.10 Darier disease. Alternating longitudinal red and white streaks with
wedge-shaped subungual hyperkeratosis, V-shaped notching of the distal
margin, and fissuring.
Fig. 71.11 Nail psoriasis. The nails show salmon patches (oil drop changes)
and onycholysis with an erythematous border.
5/3/2012 8:17:26 PM
Chapter
Onychomycosis
psoriasis of the toenails may be
difficult to distinguish from
onychomycosis without
mycologic or histologic
examination of nail clippings
Idiopathic onycholysis and
traumatic onycholysis
the area of onycholysis is not
surrounded by erythema and
there is an absence of
subungual hyperkeratosis
Alopecia areata
pits are small and geometrically
distributed
Acrokeratosis paraneoplastica (Bazex
syndrome; paraneoplastic
acrokeratosis)
the psoriasiform eruption has a typical
acral distribution with involvement of
the nose and ears
Table 71.3 Differential diagnosis of nail psoriasis.
THERAPEUTIC LADDER FOR NAIL PSORIASIS

Avoid trauma (3)
Topical vitamin D3 analogues: calcipotriene (calcipotriol) nail bed psoriasis (2)
Topical tazarotene 0.1% gel: nail bed psoriasis (2)
Topical calcipotriene plus betamethasone dipropionate ointment nail bed
psoriasis (2)
Intralesional corticosteroid (e.g. 2.55.0mg/ml triamcinolone acetonide in
saline) nail matrix psoriasis (1)
Acitretin (0.20.3mg/kg/day) nail matrix and nail bed psoriasis (1)
Methotrexate if indicated for additional manifestations (2)
Cyclosporine if indicated for additional manifestations (2)
Targeted immunomodulators (biologic therapies) if indicated for additional
manifestations (2)
Table 71.4 Therapeutic ladder for nail psoriasis. Key to evidence-based

support: (1) prospective controlled trial; (2) retrospective study or large case
series; (3) small case series or individual case reports.
DIFFERENTIAL DIAGNOSIS OF ACRODERMATITIS CONTINUA OF HALLOPEAU

Acute contact dermatitis and
dyshidrotic hand eczema
lesions are usually vesicular
rather than pustular, with palmar
and/or plantar involvement
Bacterial or viral paronychia
a relapsing course is not typical
of bacterial infection; relapses
are seen in HSV infection, but
the nail heals completely
between episodes
Onychomycosis due to
non-dermatophyte molds
may be associated with
periungual or subungual
inflammation and a purulent
discharge. Toenails are most
commonly affected and the
nail plate contains fungal
organisms; fungal cultures are
required
71
Nail Disorders
DIFFERENTIAL DIAGNOSIS OF NAIL PSORIASIS
Table 71.5 Differential diagnosis of acrodermatitis continua of Hallopeau.

HSV, herpes simplex virus.
Treatment
l
Topical vitamin D3 analogues (calcipotriene [calcipotriol],

calcitriol) or the combination of calcipotriene plus betamethasone
dipropionate. Distal nail bed disease is most easily treated with a
solution or lotion as the vehicle.
l Acitretin (0.3mg/kg/day for 46 months) in severe cases.
Parakeratosis Pustulosa
Key features
Exclusively seen in children
Psoriasiform lesions
One fingernail usually affected
Parakeratosis pustulosa was first described by Sabouraud in 1931 and

better characterized by Hjorth and Thomsen in 1967. It is seen exclu
sively in children, in whom it is usually limited to one digit, most often
the thumb or the index finger. In most patients, nail changes are pre
ceded by erythema, scaling and vesicles of the fingertip. The affected
digit shows mild psoriasiform changes with onycholysis and subungual
hyperkeratosis. The nail abnormalities are usually more marked on one
side of the nail. The disease usually regresses spontaneously, but some
children develop psoriasis.
Treatment
Treatment is with emollients, topical corticosteroids and/or calcipotriene.
Lichen Planus
Key features
Fig. 71.12 Acrodermatitis continua of Hallopeau. Recurrent pustular eruption
of the nail bed and distal finger.
Acrodermatitis continua was first described by Crocker in 1888 and

better classified by Hallopeau in 1890. Nail involvement is a typical
feature of Hallopeaus acrodermatitis, which is usually limited to one
digit. It can also occur in patients with palmoplantar or generalized
pustular psoriasis. Patients complain of relapsing episodes of acute
painful inflammation with pustules around and under the nail plate
(Fig. 71.12). Other symptoms include onycholysis, onychomadesis, and
scaling of the nail bed and periungual skin. Acrodermatitis continua of
the nail is, in most cases, not associated with cutaneous plaques of
psoriasis vulgaris. Proximal spread of the disease is unusual15.
The differential diagnosis of acrodermatitis continua is outlined in
Table 71.5.
Longitudinal fissuring of the nail plate (onychorrhexis)

Possible cicatricial outcome (dorsal pterygium)
Several nails usually affected
Histologic examination is required for diagnosis when nail
involvement is isolated
Nail abnormalities are present in approximately 10% of patients with

lichen planus (see Ch. 11). However, nail lichen planus is most fre
quently seen in the absence of skin, scalp or mucosal involvement.
Clinical findings that are diagnostic include nail thinning, ridging and
fissuring, and dorsal pterygium16 (Fig. 71.13). These signs indicate
matrix involvement and require prompt treatment to avoid scarring.
Dorsal pterygium results from adhesion of the proximal nail fold to the
nail bed due to matrix destruction and disappearance of the nail plate.
Nail bed lichen planus produces nonspecific nail changes such as onycho
lysis, nail thickening and yellow discoloration.
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11
Fig. 71.13 Nail lichen planus. Note the nail thinning with longitudinal ridging
and fissuring.
DIFFERENTIAL DIAGNOSIS OF NAIL LICHEN PLANUS

Systemic amyloidosis
thinning and fissuring are
associated with splinter
hemorrhages; histologic
examination demonstrates
amyloid deposits within the
nail matrix and nail bed
dermis
Lichen striatus
the lichenoid nail changes are
restricted to one or two digits
and limited to one side of the
nail plate
Dyskeratosis congenita
lichenoid nail changes are
associated with oral
leukoplakia and reticulated
hyperpigmentation
Nail pterygium due to bullous

diseases
the history differs and there
are often associated cutaneous
and/or mucosal lesions
Nail pterygium due to digital
ischemia
the digits are typically cold and
there is a history of Raynauds
phenomenon
Graft-versus-host disease
similar lichenoid nail changes
Additional entities
psoriasis, onychomycosis and
the yellow nail syndrome
Table 71.6 Differential diagnosis of nail lichen planus.
When isolated, this diagnosis may be difficult to establish and it

requires histologic evaluation. The differential diagnosis of nail lichen
planus is outlined in Table 71.6.
Treatment
Treatment with systemic therapies may be required to avoid pterygium
formation (see Ch. 11). Intralesional injections of corticosteroid (2.5
5.0mg/ml triamcinolone acetonide in saline) may be utilized when the
disease involves a few nails16a.
Trachyonychia (Twenty-Nail Dystrophy,

Sandpapered Nails)
Key features

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1138
Nail roughness due to excessive longitudinal ridging

Several nails are usually affected
More common in children
Often associated with alopecia areata
Benign outcome
Trachyonychia was first described by Alkiewicz in 1950. Identical nail

abnormalities were given the name twenty-nail dystrophy by Hazel
rigg etal. in 1977. Twenty-nail dystrophy or trachyonychia describes a
spectrum of nail plate surface abnormalities that produce nail rough
ness. Since the nail changes do not always involve all 20 nails, the term
trachyonychia (from the Greek word meaning rough) is preferred over
twenty-nail dystrophy by most authors. It may occur in association

with alopecia areata (12% of children and 3% of adults with severe
alopecia areata) or it may be idiopathic. The latter is more common in
children.
The nails are thin, opaque and lusterless and give the impression of
having been sandpapered in a longitudinal direction (i.e. vertically stri
ated sandpapered nails) (see Fig. 71.5). The cuticles are often hyper
keratotic. Trachyonychia is fairly asymptomatic and patients complain
only of brittleness and the cosmetic appearance. In addition to alopecia
areata, this finding is occasionally caused by several inflammatory
diseases that mildly disturb nail matrix keratinization, including lichen
planus, eczema and psoriasis. Determination of the underlying inflam
matory disease responsible for the trachyonychia requires a nail biopsy.
However, this is generally not recommended because of the benign
nature of the disease and trachyonychia improves spontaneously in the
majority of patients.
When used to treat hair and cutaneous manifestations, systemic
corticosteroids and systemic retinoids can also improve trachyonychia
in patients with alopecia areata and psoriasis, respectively.
Alopecia Areata
Key features
Geometric pitting
Children are most commonly affected
Nail abnormalities are present in approximately 20% of adults and 50%
of children with alopecia areata. Signs that are characteristic of nail
alopecia areata include geometric pitting and trachyonychia. The pits
are small, superficial and regularly distributed in a geometric pattern
(grid-like). Additional nail abnormalities observed in alopecia areata
include punctate leukonychia, erythema of the lunula, and onychoma
desis. Trachyonychia is more common in children and most frequently
seen in male patients with alopecia totalis or universalis.
Eczema (Dermatitis)
Key features
Irregular pitting and Beaus lines
Chronic paronychia
Hand eczema is often associated with nail changes. In acute eczema,
there are vesicles and erythema of the proximal nail fold and the hypo
nychium. Nail matrix damage produces irregular pitting and Beaus
lines; onychomadesis can occur in severe cases. Chronic eczema fre
quently localizes to the hyponychium, resulting in subungual hyper
keratosis, onycholysis and fissuring of the hyponychium. Chronic
eczema of the proximal nail fold can lead to chronic paronychia. In
atopic dermatitis, the nail plate frequently shows mild superficial
abnormalities such as irregular pits and Beaus lines. Controlling the
skin disease results in gradual improvement of the nails.
The nail in systemic diseases

Abnormalities of the nails have been anecdotally reported in a number
of systemic diseases, but most of these abnormalities are nonspecific.
In this chapter, we will discuss only those nail changes that are useful
in the diagnosis of systemic disorders.
The Nail Plate in Forensic Medicine

Key features
Drug exposure
Poisoning
Genetic analysis
5/3/2012 8:17:27 PM
Chapter
Clubbing (Hippocratic Fingers, Watch-Glass Nails,

Drumstick Fingers)
Key features
Bulbous digits
Watch-glass nails
Greater than 180 angle between the proximal nail fold and nail
plate (Lovibonds sign)
First described by Hippocrates in the first century BC, clubbing may be
congenital or acquired. Acquired clubbing is uncommon and in 80% of
cases is associated with pulmonary diseases17.
Clubbing is caused by enlargement of the soft tissue of the distal
digit. The nail plate is enlarged and excessively curved, with a greater
than 180 widening of the angle between the proximal nail fold and the
nail plate (see Fig. 71.2). In hypertrophic osteoarthropathy, clubbing is
associated with hypertrophy of the extremities and painful pseudo
inflammatory joint disease. Systemic conditions associated with club
bing are outlined in Table 71.7.
SYSTEMIC CONDITIONS ASSOCIATED WITH CLUBBING

Congenital/genetic
Cardiovascular disorders
Bronchopulmonary disease
Cystic fibrosis
Other
Pachydermoperiostosis (see Ch. 98)

Acquired
Bronchopulmonary diseases
Neoplasms (primary or metastatic cancers,

pleural tumors)
Chronic infections (abscesses of the lungs,
tuberculosis)
Bronchiectasis
Pulmonary fibrosis, sarcoidosis
Cardiovascular diseases
Aneurysm or dialysis fistula (unilateral

clubbing); congestive cardiac failure;
bacterial endocarditis
Gastrointestinal diseases
Inflammatory bowel disease

Carcinomas
Infestations, e.g. amebiasis, ascariasis
Liver disorders, e.g. chronic active
hepatitis, cirrhosis
Infection
HIV infection
Arterial graft sepsis (clubbing limited to
perfused extremities)
Endocrine diseases
Thyroid disease (primarily hyperthyroidism)*

Secondary hyperparathyroidism
Other
POEMS syndrome
Laxative abuse
Hemiplegia (unilateral)
Trauma, median nerve injury (unidigital)
Yellow Nail Syndrome

Key features

Arrest in nail growth

Yellowgreen nails
Absence of the cuticle
Overcurvature and thickening
All or most nails are affected
Congenital heart disease (usually cyanotic)

Pulmonary arteriovenous malformations
(often in the setting of hereditary
hemorrhagic telangiectasia)
71
Nail Disorders
The slow growth rate of nails makes it possible to utilize nail clippings
to detect previous exposure to drugs or toxins (e.g. heroin). Compounds
that can be measured in the nails include heavy metals and drugs5.
*Most often in the setting of thyroid acropachy (clubbing together with digital swelling
and periosteal reaction) in patients with autoimmune thyroid disease.
Table 71.7 Systemic conditions associated with clubbing.
The yellow nail syndrome was first described by Samman and White
in 1964. It is not common and its pathogenesis is still unknown. Linear
nail growth is arrested or greatly reduced. The nails are thickened and
transversely and longitudinally overcurved with disappearance of the
cuticle. The nail color varies from pale yellow to dark yellowgreen
(Fig. 71.14). Onycholysis is frequently seen, as is nail plate shedding.
In most patients, all 20 nails are involved. Characteristically, the nail
abnormalities are associated with lymphedema and respiratory tract
involvement, including chronic bronchitis, bronchiectasis, sinusitis
and pleural effusions. The diagnosis of yellow nail syndrome, however,
requires only the presence of the typical nail changes.
Treatment
Treatment is not effective in all cases and must be prescribed for several
months:
l vitamin E 1200IU/day
l pulse itraconazole (400mg daily for 1 week a month) or
fluconazole (150mg daily for 1 week a month); theoretically, the
drugs act by accelerating nail growth.
Apparent Leukonychia
Key features
White discoloration that fades with pressure
Nail plate transparency is maintained
On the basis of the pattern of the nail discoloration, three different
types of apparent leukonychia can be distinguished. Nail bed edema
may be a common factor.
Fig. 71.14 Yellow nail syndrome. Note the overcurvature of the nail and loss
of the cuticle, in addition to the yellow discoloration.
Terrys nails were described by Terry in 1954 as a common sign

of liver cirrhosis, occurring in up to 80% of patients. The
leukonychia affects the whole nail except for a 1 to 2mm distal
band. Terrys nails are also frequently seen in normal individuals.
l Muehrckes nails were described by Muehrcke in 1956 in patients
with hypoalbuminemia (nephrotic syndrome). They are a very
common finding in patients receiving combination chemotherapy.
The nail has multiple transverse whitish bands, parallel to the
lunula (Fig. 71.15).
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11
Fig. 71.15 Muehrckes

nails. There are multiple
parallel transverse white
bands. The curvature
matches that of the lunula.
Half-and-half nails. First reported by Bean in 1963, half-and-half

nails are detected in up to 25% of patients with chronic renal
disease who are undergoing hemodialysis. The leukonychia affects
the proximal half of the nail. Half-and-half nails may also be seen
in normal individuals.
Pseudoinflammatory Nail Changes

Key features
Cold painful whitlow: digital ischemia
Painless whitlow and normal temperature: bone metastases
The distal portion of the digit is greatly enlarged and red, simulating
an acute paronychia, but the skin temperature is cold or normal. This
picture may be caused by digital ischemia or metastases to the distal
phalanges. In digital ischemia, the digit is cold and painful. The most
common causes include arterial obstruction and autonomic neuropa
thies causing ischemia (including diabetes). Digital metastases are
usually painless; metastases in the fingers are most commonly associ
ated with pulmonary neoplasms and those in the toes with genitouri
nary neoplasms. X-ray examination is required for the diagnosis18.
Peripheral Neuropathies
Key features
Nail abnormalities limited to the first three digits: carpal tunnel
syndrome
Onychomadesis with pseudopyogenic granuloma: mild peripheral
nerve injury
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1140
Examination of the nail may suggest the diagnosis in carpal tunnel

syndrome, since nail abnormalities are limited to the first three fingers
and are associated with fingertip paresthesias. Depending on the sever
ity of nerve damage, nail changes vary from Beaus lines to onycho
madesis and necrosis. Neurologic examination and electromyography
confirm the diagnosis.
Onychomadesis associated with pseudopyogenic granuloma of the
proximal nail fold is a sign of mild and transitory peripheral nerve
injury. It is typically seen in patients with phalanx, metacarpal or
wrist bone fractures during or after cast immobilization. Patients often
report a history of pain or paresthesias of the hand during the period
of immobilization. The condition spontaneously resolves over a few
weeks19.
Autoimmune Connective Tissue Disorders

Key features

Nail fold capillary abnormalities

Cuticular hemorrhages
Ventral pterygium
Nail beaking
Abnormalities in the proximal nail fold capillaries are frequently seen

in autoimmune connective tissue disorders, as they occur in most
patients20. Roughness, hemorrhages and necrosis of the cuticles are
common in dermatomyositis and scleroderma, where capillaroscopy
shows reduced capillary density and avascular areas alternating with
dilated capillary loops. In systemic lupus erythematosus, capillaroscopy
shows a normal capillary density with dilated tortuous capillaries.
Ventral pterygium (pterygium inversus unguis) is characterized by the
adhesion of the distal nail plate to the hyponychium, resulting in pain
during nail trimming. Ventral pterygium is a distinctive sign of sclero
derma related to impaired peripheral perfusion21. In scleroderma,
ischemic changes and bone resorption may result in nail beaking with
bending of the nail plate around the shortened fingertip (see Fig. 43.2).
HIV Infection
Key features

Proximal subungual onychomycosis due to Trichophyton rubrum

Candida onychomycosis
Squamous cell carcinoma
Onychomycoses are common in HIV-infected patients, occurring in up

to 25% of affected individuals. Dermatophytes are most commonly
responsible, but Candida spp. and molds are also often isolated22.
Although a large number of nail abnormalities have been reported in
association with AIDS17, only a few clinical entities can be considered
typical of this disease.
l Proximal subungual onychomycosis due to T. rubrum. This
variety of onychomycosis is considered a marker for HIV infection
and immunodeficiency. The proximal nail plate is opaque
and white due to the presence of fungi in its ventral
portion.
l Candida onychomycosis. Candida does not invade the nail plate
of immunocompetent individuals, and the diagnosis of true
Candida onychomycosis indicates immunosuppression often,
HIV infection.
l Longitudinal melanonychia. Usually, several nails are involved,
and it is often associated with skin hyperpigmentation.
l HPV-induced squamous cell carcinoma. Longstanding
periungual warts in HIV patients should always raise the
suspicion of squamous cell carcinoma and be examined
histologically. HPV types 16 and 35 have been detected in
such lesions23.
Drug-Induced Nail Abnormalities (Table 71.8)

Key features

Involvement of most or all nails

Beaus lines and onychomadesis
Painful hemorrhagic onycholysis
Multiple bands of melanonychia
Paronychia with pyogenic granulomas
Drug-induced nail changes usually involve several or all nails. The

pathogenesis is most often toxic in nature24.
5/3/2012 8:17:28 PM
Chapter
71
Nail abnormality
Responsible agents
Beaus line and onychomadesis
Chemotherapeutic agents
True leukonychia
Nail thinning and brittleness
Retinoids
Onycholysis/photo-onycholysis
(see Fig. 71.6)
Chemotherapeutic agents, particularly

taxanes
Tetracyclines
Psoralens
NSAIDs
Apparent leukonychia (e.g.

Muehrckes nails)
Chemotherapeutic agents, particularly

polychemotherapy including
anthracyclines, vincristine
Melanonychia
Psoralens
Zidovudine (AZT)
Discoloration (non-melanin)
Minocycline
Antimalarials
Gold
Paronychia and periungual

pyogenic granulomas
Retinoids
Antiretroviral drugs (indinavir, efavirenz,
lamivudine)
Epidermal growth factor receptor (EGFR)
inhibitors (cetuximab, gefitinib, erlotinib,
panitumumab)
Methotrexate
Capecitabine (prodrug of 5-fluorouracil)
Sirolimus
Ischemic changes
Beta-blockers
Bleomycin
Nail Disorders
DRUG-INDUCED NAIL ABNORMALITIES
Fig. 71.16 Multiple periungual pyogenic granulomas in a patient taking

indinavir.
Fig. 71.17 Acute

paronychia. In addition
to the erythema and
purulent discharge, there
is significant tenderness.
Table 71.8 Drug-induced nail abnormalities.
l
l
l
Cancer chemotherapeutic agents, due to their cytotoxic properties,

are the most frequent cause of nail changes. These include Beaus
lines, onychomadesis, fragility, pigmentation, onycholysis,
paronychia, and vascular problems, including subungual
hemorrhages, hematomas and ischemia. Painful subungual
hemorrhages and abscesses are typically seen with taxanes
(paclitaxel and docetaxel).
Paronychia associated with pyogenic granulomas has been reported
with epidermal growth factor receptor (EGFR) inhibitors used as
chemotherapeutic agents (see Ch. 21). Nail changes appear from
1 to 3 months after starting treatment and disappear after
interruption of treatment.
Oral retinoids are frequently responsible for nail abnormalities,
especially nail fragility, paronychia and pyogenic granulomas.
Antiretroviral drugs often cause nail pigmentation, paronychia and
pyogenic granulomas (Fig. 71.16).
Although uncommon, photo-onycholysis is a characteristic side
effect of tetracyclines, taxanes, psoralens and photodynamic
therapy (see Fig. 71.6).
Certain drugs (e.g. anticoagulants and anticonvulsants) taken
during pregnancy may impair the development of the digits,
leading to congenital nail hypoplasia.
Infections
Acute Paronychia
Key features
Painful whitlow
Recurrent when due to herpes simplex virus (HSV)
Viral and bacterial cultures are mandatory
The affected digit becomes swollen, red and painful (Fig. 71.17). Com
pression of the nail fold may produce purulent drainage. Acute paro
nychia is most commonly due to bacteria, in particular Staphylococcus
aureus or Streptococcus pyogenes, and it follows minor trauma to the
nail. Recurrent episodes of acute paronychia should raise the suspicion
of an HSV infection. Viral cultures, direct fluorescent antibody assay,
and/or PCR should be obtained to identify the responsible agent.
Treatment
l
Drainage of the abscess.

Systemic antibiotics according to culture results.
l Systemic antivirals when due to HSV.
l
Warts (see Ch. 79)

Key features

Periungual keratotic papules

Hyperkeratosis of the cuticle
Onycholysis and subungual hyperkeratosis
In recalcitrant lesions, consider the possibility of squamous cell
carcinoma
Periungual warts are common in nail biters, in whom they are multiple
and involve several nails. The warts appear as keratotic papules, and
when localized to the proximal nail fold, they frequently produce peri
ungual hyperkeratosis simulating a hyperkeratotic cuticle. Nail bed
warts lead to uplifting of the nail plate with onycholysis. Squamous cell
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11
THERAPEUTIC LADDER FOR PERIUNGUAL WARTS

Keratolytics (2)
Cryotherapy (2)
Topical cantharidin (2)
Topical imiquimod (2)
Intralesional injection of Candida, Trichophyton or mumps skin test antigens (1)
Topical immunotherapy (2)
Laser: CO2, erbium:YAG, pulsed dye (2)
Photodynamic therapy (2)
Ablative CO2 fractional laser followed by PDT* (2)
*With methyl 5-aminolevulinic acid and red light.
For details, see Table. 129.13.
Table 71.9 Therapeutic ladder for periungual warts. Key to evidence-based

support: (1) prospective controlled trial; (2) retrospective study or large case
series; (3) small case series or individual case reports. PDT, photodynamic
therapy.
of the cuticle. One or several fingernails (especially the thumb and

second or third fingers of the dominant hand) are affected. Damage
to the nail matrix results in nail plate surface abnormalities such as
Beaus lines26. Chronic paronychia usually assumes a prolonged course
with superimposed, recurrent, self-limited episodes of acute exacerba
tion. Secondary infections with Candida spp. and Pseudomonas aeruginosa are common.
Treatment
l
Avoidance of water and chemical exposure.

Topical corticosteroids.
l Topical imidazoles.
l Topical antiseptics (e.g. 4% thymol in 95% ethanol).
l Systemic antifungals are not useful.
l
Idiopathic Onycholysis
Key features
Limited to fingernails
White or green discoloration
Absence of subungual hyperkeratosis
carcinoma can arise in or mimic a verruca; therefore, suspicious lesions

must be further evaluated.
Treatment
See therapeutic ladder (Table 71.9).
Onychomycosis (Tinea Unguium)

See Chapter 77.
Environmental nail disorders

Brittle Nails (Fragility, Onychoschizia)
Key features
Lamellar exfoliation (onychoschizia)
Fingernails affected
Nail dehydration due to environmental factors
Brittle nails are very common and usually affect women. Nail brittle
ness results from dehydration of the nail plate as a result of environ
mental factors such as frequent handwashing. In lamellar onychoschizia,
the distal nail plate splits horizontally into multiple layers (see Fig.
71.2). Other signs of nail brittleness include splitting, softening and
onychorrhexis.
Treatment
Biotin (2.55mg/day).
l Topical moisturizers and humectants.
Idiopathic onycholysis frequently involves the fingernails as a conse

quence of repetitive water immersion and exposure to irritants (see Fig.
71.6). The affected nail is detached from the nail bed and often shows
an abnormal color due to secondary microbial contamination of the
subungual space26.
Treatment
l

Clipping of the detached nail plate.
l Topical antiseptics.
l
Traumatic nail abnormalities (Table 71.10)

Onychotillomania
Key features
Cuticle absent and proximal nail fold inflamed
Nail plate surface abnormalities, e.g. longitudinal central
depression
Melanonychia
Hemorrhages and crusts
l
l
Chronic Paronychia
Key features

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1142
Proximal nail fold inflammation

Absence of the cuticle
Fingernails affected
Nail plate surface abnormalities
Chronic paronychia commonly involves the fingernails of adult women.

Although the pathogenesis is still debated, there is accumulating
evidence that the condition represents a contact reaction to irritants
or allergens25. Occupational chronic paronychia is common in food
handlers. Chronic paronychia is clinically characterized by inflam
mation of the proximal nail fold with erythema, edema and absence
Self-induced nail abnormalities are common, but often not recognized

due to the wide variety of nail signs and the difficulty in obtaining a
correct clinical history.
l Nail biting may affect the nail plate and/or the proximal nail fold;
the trauma often produces nail matrix damage with secondary nail
plate abnormalities, including surface irregularities and
longitudinal melanonychia.
l Habit-tic deformity affects the thumb and is due to the nervous
habit of rubbing and pushing back the mid-portion of the cuticle
of the thumb with the index finger. The nail plate of the thumb
shows multiple midline Beaus lines with a prominent longitudinal
central depression. Median canaliform dystrophy of Heller, also
known as dystrophia unguis mediana canaliformis, is characterized
by an inverted fir tree-like split or canaliform defect in the nail
plate (Fig. 71.18) and is possibly a variant of habit-tic deformity27.
l Nail destruction associated with psychiatric disorders. The clinical
picture is variable, with nail plate destruction, hemorrhages, and
periungual crusts and erosions. Instruments (scissors, clippers,
etc.) are often utilized to destroy the nails.
5/3/2012 8:17:30 PM
Nail abnormality
Etiology
Beaus lines
Matrix damage from cuticle

removal
Treatment
True leukonychia (transverse)
Matrix damage from cuticle

removal
Acute hematomas require drainage of the blood by creating a hole

through the nail plate.
Nail brittleness (onychorrhexis,

onychoschizia)
Nail brittleness (superficial granulation)
Nail varnish solvents; electric drills
Distal onycholysis (roller coaster)
Prolonged nail polish wearing
Diffuse onycholysis
Mechanical (nail tools utilized to

clean the nail plate free edge)
Mechanical (artificial nails acting as
a lever)
Chemical (acrylic nails)
Discoloration (yellow/orange)
Nail varnishes
Acute paronychia
Chronic paronychia
Periungual pyogenic granulomas
Periungual warts
Periungual cuts from sharp

instruments
Contact allergy
Constant cuticle removal
Periungual wounds
Mechanical trauma to the cuticle
Diffuse onycholysis
Table 71.10 Nail changes associated with a manicure or pedicure.

Fig. 71.18 Median
canaliform dystrophy
(of Heller).
Chapter
71
Nail Disorders
NAIL CHANGES ASSOCIATED WITH A MANICURE OR PEDICURE
within the ventral nail plate and migrate distally with nail growth.
The color of subungual hematomas ranges from purplered to black.
Dermoscopy is useful for differentiating hematomas from melanin
deposition28.
Traumatic Toenail Abnormalities

Key features

Onycholysis without subungual hyperkeratosis

Leukonychia and melanonychia
Often bilateral
Podiatric abnormalities
Retronychia characteristically affects the proximal nail fold
Traumatic toenail abnormalities are most commonly seen in women

who wear high-heeled pointed shoes.
l Traumatic onycholysis of the hallux is the most common clinical
presentation. The onycholysis may be distal, where the
detachment is caused by compression of the distal pulp by poorly
fitting shoes, or lateral, when there is overlapping of the second
toe onto the first. Clipping of the detached nail plate reveals a
normal nail bed. The differential diagnosis includes
onychomycosis, but here the onycholysis is associated with
subungual hyperkeratosis.
l Transverse leukonychia of the hallux results from repeated
microtrauma by shoes to untrimmed, long, great toenails29.
Multiple bands of true leukonychia move distally with nail growth.
l Frictional melanonychia affects the toenails of the fourth and/or
fifth digits of women. It is due to activation of nail matrix
melanocytes by friction from shoes or from the adjacent digit. The
bands may be multiple and black in color.
l Retronychia is caused by embedding of the nail into the proximal
nail fold following trauma and may present with inflammation of
the proximal nail fold.
Onychogryphosis
Key features
Treatment
l
Topical preparations that taste unpleasant.

Bandages.
l Serotonin reuptake inhibitors (fluoxetine, paroxetine, sertraline).
l
Rams horn nails

Thick, hard, yellowbrown nail plate
Hallux most frequently affected
Elderly patients
Subungual Hematoma
Key features

History of acute trauma

Purplered to black in color
Typical dermoscopic features
Discoloration moves distally with nail growth
Acute hematomas occur after trauma severe enough to lead to accu

mulation of blood under the nail plate. Toenails are most commonly
affected. Acute hematomas are typically associated with pain,
and compression of the matrix may cause secondary nail plate dyst
rophy. An X-ray of the digit may be advisable to exclude a bone
fracture.
Repeated microtrauma or hemorrhagic conditions produce asymptom
atic small subungual hematomas of the nails that are incorporated
Onychogryphosis is common in elderly persons and almost exclusively

affects the toenails, usually the hallux. The nail acquires a typical rams
horn shape due to asymmetric growth. The nail plate is thick, hard and
yellowbrown. Multiple transverse striations are often present. Nail
hardness and self-neglect may lead to monstrous deformities.
Pincer Nails (Trumpet Nails)

Key features

Nail bed pinching by overcurved nail plate

Toenails most frequently affected
Severe pain
Subungual exostosis should be excluded by X-ray examination
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11
Pincer nails are usually seen on the toes and may be hereditary or
acquired; the latter is most commonly due to ill-fitting shoes. The nail
plate displays an excessive transverse overcurvature, especially in its
distal portion (see Fig. 71.2). This results in compression of the distal
nail bed with severe pain30.
Treatment
Lateral matricectomy (see Ch. 149) is the treatment of choice. Surgery
is necessary when a subungual exostosis is present.
Ingrown Toenails (Onychocryptosis)

Key features

Teenagers and young adults

Congenital malalignment often present
Precipitated by improper nail cutting and hyperhidrosis
Painful inflammation of the lateral fold
Growth of granulation tissue
Lateral ingrowing usually affects the hallux of young adults with

congenital malalignment of the great toenails. Precipitating factors
include improper or aggressive nail cutting and trauma. Penetration of
nail plate spicules into the lateral nail fold epithelium causes painful
inflammation. Chronic onychocryptosis is characterized by the growth
of granulation tissue that eventually undergoes epithelialization1.
Distal embedding is a common complication of nail avulsion. The
nail plate growth is blocked by the hyponychium, which forms a distal
rim. Retronychia represents the ingrowth of the proximal nail plate into
the proximal nail fold, with one to three nail plates misaligned beneath
the uppermost nail plate31. There may be associated proximal peri
ungual pyogenic granulomas.
Treatment
l
l
l
l
l
l
Prevention through patient education.

Removal of the embedded spicule.
Uplifting of the lateral nail plate with cotton or dental floss.
Granulation tissue may be prevented by topical antibiotics and
topical corticosteroids or treated by cryotherapy, chemical cautery
or electrodesiccation.
Chemical (88% phenol), laser or excisional surgical removal of the
lateral matrix is advisable in severe cases.
In retronychia, surgical avulsion of the proximally embedded nail
plate as well as the underlying plate(s) is necessary.
Nail tumors
papulonodule (see Fig. 71.16). When subungual, it is associated with

onycholysis. The differential diagnosis includes amelanotic melanoma.
Treatment is surgical.
Fibromas/Fibrokeratomas
Key features
Filiform growth
Nail plate furrow
Possible sign of tuberous sclerosis
Isolated periungual and subungual fibromas are not rare in the general
population. Multiple lesions occur in 50% of patients with tuberous
sclerosis (Koenens tumors). Periungual fibromas appear as pink or
skin-colored papules originating from the proximal nail fold. The
fibroma may compress the nail matrix and produce a longitudinal
groove in the nail plate. Subungual fibromas that grow underneath the
nail plate produce longitudinal erythronychia or onycholysis. A fibro
keratoma is characterized by a hyperkeratotic tip and may be sur
rounded by a collarette of raised skin.
Subungual exostosis
Key features

Subungual hard nodule

Onycholysis
Toenails (especially hallux)
X-ray examination is diagnostic
Subungual exostoses, first described by Dupuytren in 1847, are the

most common benign bony proliferations associated with nail abnor
malities. Subungual exostoses are commonly precipitated by trauma
and usually seen on the great toe of young patients (Fig. 71.19A). An
exostosis produces a firm, tender subungual nodule that elevates the
nail plate. The nodule may ulcerate or become hyperkeratotic. The
diagnosis is confirmed by X-ray examination (Fig. 71.19B)33.
Myxoid cysts (mucous cyst)
Key features
Proximal nail fold swelling
Nail plate depression and grooves
Periodic fluid drainage
Nail surgery is discussed in Chapter 149.
Benign Tumors
Pyogenic granuloma (botryomycoma)
Key features
Bleeding angiomatous papulonodule
Periungual or subungual
Often traumatic
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1144
Pyogenic granulomas commonly appear within the nail apparatus,

where they may be periungual or subungual, and often follow penetrat
ing trauma. Other common causes of pyogenic granuloma of the nail
include ingrown toenails, systemic drugs (e.g. retinoids, EGFR inhibi
tors), peripheral nerve damage, and frictional onycholysis due to pro
longed walking32,32a. The tumor appears as a bleeding, friable, soft red
First described by Hide in 1883, myxoid cysts are the most common
nail tumor, often occurring in middle-aged women. Typically located in
the proximal nail fold of the fingernails, they appear as small soft
nodules that often spontaneously drain a viscous jelly-like fluid.
Compression of the matrix produces nail plate depression and grooves
(Fig. 71.20). Occasionally, the cysts are subungual. These cysts are
connected to the distal interphalangeal joint by a tract, and osteoarthri
tis of the distal joint is a frequent association34.
Treatment
Possible treatments for myxoid cysts include sclerotherapy, cryosurgery,

and intralesional corticosteroid injections. Caution must be taken
because septic arthritis is a potential complication of surgical proce
dures that allow access of surface bacteria into the underlying synovial
space. All of these procedures are associated with a high frequency of
relapses. Definitive cure of myxoid cysts can be obtained with surgical
procedures that include ligature of the pedicle that connects the cyst to
the joint.
5/3/2012 8:17:30 PM
Chapter
71
Nail Disorders
Fig. 71.19
Subungual exostosis. A
Skin-colored subungual
nodule elevating the nail
plate. B Radiograph of the
digit demonstrating the
subungual bony
proliferation.
Fig. 71.21 Onychomatricoma. The nail is thickened and overcurved with

longitudinal yellow streaks. The distal border shows several holes.
Glomus tumor
A
Key features
Severe pain
Temperature sensitivity
Subjective symptoms contrast with minimal clinical signs
First described by Wood in 1812, a glomus tumor arises from the neuro
myoarterial glomus cells of the nail bed dermis. It is associated with
severe pain that radiates proximally and is often aggravated by physical
or thermal stimuli (especially cold). Subjective symptoms typically
exceed clinical signs. The tumor appears as a redbluish nail bed
macule visible through the transparent nail plate (see Ch. 149). MRI
allows the diagnosis in doubtful cases (see Ch. 114).
Onychomatricoma
Key features

Fig. 71.20 Myxoid cyst.

The longitudinal nail
groove is a result of the
compression of the nail
matrix by the cyst.
Localized longitudinal thickening with transverse overcurvature

Yellowwhite discoloration
Multiple, longitudinal, tumor-containing hollows
Holes in the distal nail
First described by Baran and Kint in 1992, onychomatricoma is a rare

neoplasm that produces localized or diffuse thickening of the nail plate
with perforations consisting of multiple longitudinal hollows that
contain the digitating tumor. Most commonly, onychomatricoma arises
within the fingernails of middle-aged individuals35 (Fig. 71.21). A
frontal view of the nail typically reveals multiple holes in the thickened
free margin. The affected nail is thickened and yellowwhite in color,
with multiple splinter hemorrhages.
Histologically, the tumor is characterized by multiple fibroepithelial
projections that extend into the thickened nail plate. The tumor epi
thelium is identical to that of the normal nail matrix and keratinizes
without a granular layer.
Melanocytic nevi of the nail matrix
Key features
Onset in childhood
Nail matrix nevi are an uncommon cause of longitudinal melano

nychia, especially in comparison with melanocyte activation (see Table
71.2). They usually develop during childhood and often involve the
fingers, especially the thumb36,37. The color, width and pigment
1145
5/3/2012 8:17:32 PM
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11
distribution may vary considerably and it is not unusual to observe

fading or darkening of the pigmentation over time. Pigmentation of the
periungual tissues is also possible.
Treatment
Optimal management of nail matrix nevi is still debated. The authors

approach is immediate excision of rapidly growing lesions and excision
of all lesions after puberty (see Ch. 149).
Malignant Tumors
Malignant tumors of the nail are summarized below. For further details
of the disorders and their treatment, see Chapters 108 and 113.
Bowens disease (squamous cell carcinoma in situ)
Key features
Verrucous lesion
Onycholysis
Melanonychia
Fig. 71.22 Bowens disease. The lateral portion of the nail plate is absent. The
nail bed shows hyperkeratosis with scaling and fissuring of the epithelium.
Bowens disease of the nail is uncommon and is seen most often in

middle-aged men. Fingers of the left hand are most commonly
involved38. Clinically, it may be difficult to differentiate Bowens disease
from warts. The affected digit shows periungual or subungual verrucous
lesions with onycholysis and longitudinal melanonychia (Fig. 71.22).
Predisposing factors include HPV infection and chronic X-ray
exposure.
Fig. 71.23 Amelanotic

melanoma. Note the
diffuse nail destruction
and ulceration.
Keratoacanthoma
Key features
Painful subungual nodule
Rapid growth
Osteolysis on radiography
Keratoacanthoma is an extremely rare tumor that usually affects the

thumb or the index or middle fingers. It appears as a painful subungual
keratotic nodule that grows rapidly over a period of weeks. Deep inva
sion with bone destruction is frequent. A history of trauma is often
reported. In contrast to cutaneous keratoacanthomas, keratoacantho
mas of the nail do not regress spontaneously39.
Squamous cell carcinoma
Key features

Verrucous lesion
Ulcerated nodule
Onycholysis
Nail plate destruction
Squamous cell carcinoma is the most frequent malignant tumor of the

nail apparatus. It most commonly affects the fingernails of middle-aged
men. A number of studies have confirmed a causative role for HPV,
mostly HPV-16, in the development of this tumor. Clinically, there is
a slowly growing periungual or subungual mass that may ulcerate and
bleed. Periungual swelling and inflammation are often seen. Bony
involvement may occur but metastases are extremely rare40.
Verrucous carcinoma (carcinoma cuniculatum,

epithelioma cuniculatum)
Key features
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1146
Rapidly growing verrucous nodule

Nail destruction
Verrucous carcinoma is a rare, low-grade variant of squamous cell car

cinoma characterized by locally aggressive clinical behavior but low
potential for metastasis. It rarely involves the nail apparatus. Clinically,
the tumor appears as a rapidly growing nodule that often destroys the
nail. Bone resorption is common.
Melanoma
Key features

Thumb is the most frequent site
Nail plate destruction
Hutchinsons sign
Amelanotic in 25% of cases
Nail melanoma is rare, accounting for 0.73.5% of all melanomas. It

most frequently involves the thumb of middle-aged individuals, and
patients often report a history of trauma. Diagnosis is often delayed,
and in 25% of cases the tumor is amelanotic. The 5-year survival is
only 15%41. From a clinical standpoint, nail melanoma may have the
following features:
l
Longitudinal melanonychia. The pigmented band is usually dark

brown or black in color, with blurred margins. Nail plate
abnormalities result from nail matrix damage. The dermoscopic
5/3/2012 8:17:33 PM
age (peak incidence 5th to 7th decades of life)

African-Americans, Asians and native Americans (1/3 of all nail
melanoma cases)
brown to black
breadth (3mm or wider)
borders (variegated)
change in the nail band (color/size)

lack of change in the nail morphology despite presumed adequate
treatment
digit most commonly involved (thumb and big toe)
extension of the pigment into the proximal and/or lateral nailfold

(Hutchinsons sign)
family or personal history of dysplastic nevi or melanoma
Chapter
71
Nail Disorders
ABCDEF RULE FOR CLINICAL SUSPICION OF NAIL MELANOMA
pattern that has been associated with melanoma is a brown

background with longitudinal lines that are irregular in color,
thickness and spacing. However, a recent study indicated that
these dermoscopic patterns were not useful in the early detection
of nail melanoma, as dermoscopy did not improve accuracy in the
clinical diagnosis of melanoma42.
l A subungual pigmented lesion that may have ulcerated and is
associated with onycholysis and nail destruction.
l An amelanotic nodule that frequently ulcerates and bleeds,
resembling a pyogenic granuloma (Fig. 71.23).
The presence of Hutchinsons sign (pigmentation of the periungual
tissues due to superficial spreading of the tumor) should raise the sus
picion of nail melanoma. An ABCDEF of nail melanoma has been
proposed, to facilitate diagnosis (Table 71.11)43.
Table 71.11 ABCDEF rule for clinical suspicion of nail melanoma. Adapted from
Levit EK, etal. The ABC rule for clinical detection of subungual melanoma. J Am Acad Dermatol.
2000;42:26974.
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42. Di Chiacchio N, Hirata SH, Enokihara MY, et al.
Dermatologists accuracy in early diagnosis of melanoma
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Hair, nails and mucous membranes SECTIONC11

Antonella Tosti and Bianca Maria Piraccini

Anatomy (Fig. 71.1)

The nail plate is a fully keratinized structure produced by the germina

THE NAIL APPARATUS

CORRELATION OF NAIL FINDINGS WITH ANATOMIC SITE OF NAIL DAMAGE

Proximal nail fold

Proximal + distal matrix

Fig. 71.1 Schematic drawing of the nail apparatus in longitudinal section.

Bolognia_Chapter 71_main.indd 1129

Hair, nails and mucous membranes

Fig. 71.2). Recognizing specific nail signs is very important in under

First described by Beau in 1846, these transverse depressions of the nail

Nail Signs due to Abnormal Nail Matrix Function

Onychomadesis (nail shedding)

Proximal detachment of the nail

Fig. 71.2 Nail signs and nail disorders.

Bolognia_Chapter 71_main.indd 1130

Fig. 71.4 Nail pitting

Fig. 71.3 Onychomadesis after an episode of acute paronychia.

The term onychomadesis describes the detachment of the nail plate

Pitting describes the presence of small depressions on the nail plate

Onychorrhexis produces longitudinal ridging and fissuring of the nail

Trachyonychia (twenty-nail dystrophy, sandpapered nails)

Bolognia_Chapter 71_main.indd 1131

Fig. 71.5 Trachyonychia (twenty-nail dystrophy). The nails seem sandpapered

Trachyonychia is a specific nail plate surface abnormality characterized

Hair, nails and mucous membranes

Koilonychia (spoon nails)

Nail Signs due to Nail Bed Disorders

The nail plate appears thickened due to the presence of subungual

Bolognia_Chapter 71_main.indd 1132

Nail Signs due to Deposition of Pigment

Longitudinal brown to black band

Primary skin disorders

Candida spp. (may

Longitudinal melanonychia is very commonly observed in individuals

Bolognia_Chapter 71_main.indd 1133

Nonetheless, epiluminescence microscopy is increasingly being per

Hair, nails and mucous membranes

CAUSES OF LONGITUDINAL MELANONYCHIA

Table 71.2 Causes of longitudinal melanonychia.

First described by Hutchinson in 1886, this eponymous sign represents

Green nail syndrome

In green nail syndrome, the nail has a greenish-black or greenish-blue

Bolognia_Chapter 71_main.indd 1134

Fig. 71.7 Longitudinal melanonychia due to a congenital melanocytic nevus

Congenital and hereditary nail diseases

Congenital Malalignment of the Great Toenails

Fig 71.8 Congenital

is often thickened and transversely overcurved. Congenital malalign

Congenital Hypertrophy of the Lateral Fold

Hypertrophic lateral nail folds in newborns were first described by

Racquet Thumbs (Brachyonychia)

Racquet thumb is a common malformation due to shortening of the distal

NailPatella Syndrome (Onycho-osteodysplasia;

Nailpatella syndrome is an autosomal dominantly inherited condition

Bolognia_Chapter 71_main.indd 1135

Fig. 71.9 Nail hypoplasia in nailpatella syndrome. There is hypoplasia of the

lunula is commonly observed. The nail changes are typically associated

Nail abnormalities are common in all forms of epidermolysis bullosa

Ectodermal dysplasias were first described by Weech in 1929 and clas