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NAIL biology and

DISORDERS
NAIL UNIT ANATOMY

The nail apparatus consists of nail plate and specialized epithelial tissues
(nail matrix, nail bed and nail folds)

1- Nail plate
• Fully keratinized, hard, flexible and semitransparent structure.
• Comprised of tightly packed corneocyte (onychocyte), arranged in layers.
• Results from keratinization of nail matrix epithelium.
• Resting on and firmly attached to nail bed which partially contributes to
its formation. It is less adherent proximally.
• Approximately ¼ of nail is covered by proximal nail fold, and a narrow
margin of its sides is occluded by the lateral nail folds.
• Toenails are usually thicker than fingernails.
• Men nails are thicker than women nails.
• Toenails 1.4 - 1.65 mm. Fingernail 0.5 - 0.6 mm.
2- Nail matrix
• Epithelium situated above the middle part of distal phalanx.
• Keratinizes without a granular layer.
• Generates > 90% of nail plate (≤ 1% from nail bed)
• Contains many melanocytes that are normally quiescent (inactive),
however, they may become activated.
• Proximal matrix: produce ~80% of nail plate → dorsal nail plate (outer).
• Distal matrix (lunula): visible as white half moon → Ventral plate (inner)
3- Nail fold
• Skin surrounding lateral and proximal aspects of
nail plate.
• proximal nail fold: a double layer of skin overlying
the matrix, protects this area from trauma, solvents and infectious agents.
Extends onto the proximal nail plate to form the cuticle. Its dorsal layer is
continuous with the skin of digit, while the ventral layer is continuous
with the nail matrix.
• lateral nail fold: a double layer of skin. Continuous with skin on the sides
of digit laterally, and medially they are joined by the nail bed.
• Capillary loops at tip of proximal nail fold are small and non apparent
(apparent in AICTD e.g. SLE).
4- Nail bed Epidermis
 Nail bed epidermis: Dermis
• Thin epithelium (2-5 cell layers). Has no granular
layer, no melanocytes. Extend from distal margin of
lunula to hyponychium. So adherent to nail plate.
• Completely visible through the nail plate.
• Its keratinization produces thin horny layer, that add plate
to the undersurface of nail, thickening it and making
it densely adherent to the nail bed.
Dermis
• Their rete ridges has parallel longitudinal pattern:
o Interdigitate with the underling longitudinal dermal ridges.
o Small blood vessels run longitudinally at the base of these ridges,
explaining the linear pattern of nail bed hemorrhage (i.e. splinter).
 Nail bed dermis: Devoid of fat. Contain numerous glomus bodies.

5- Hyponychium
The site where the nail plate detaches from nail
bed (between nail bed and distal groove).
NAIL EMBRYOLOGY

• 8th week →nail primordium becomes evident on the distal dorsal


surface of digit.

• 12th week nail primordium invaginates beneath the proximal nail fold.

• 15th week →Fully developed nail matrix + Nail plate production occurs
continuously until death.

• 16th week → The outline of nail unit is established.

• 17th week →Fully developed nail plate.


CHEMICAL PROPERTIES OF NAIL PLATE
1. Keratin :
• Nail consist mainly of low-sulfur fibrous protein [keratin], embedded in
an amorphous matrix composed of high-sulfur proteins rich in cysteine +
high-sulfur proteins rich in glycine/tyrosine.
• Nail keratin consist of 80~90% hard keratin (hair-type) and 10~20% soft
keratin (skin-type). Hard keratin contributes to nail hardness.
• Keratin filaments has a transvers orientation, this explains why the nail
plate is more susceptible to transverse fracture than longitudinal.
2. Water : 18% of nail content. Dehydration is faster with nails are kept
long. When water content decrease below 18%, the nail become brittle.
When it increases above 30%, it become soft. Flexibility depends on water
content
3. Lipids : < 5%. Mainly cholesterol.
4. Inorganic (iron, zinc, calcium): trace amount. Don’t contributes to nail
hardness
NAIL GROWTH RATE

• Nails grow continuously, but their growth rate decreases both with age
and with poor circulation.

• Fingernails grow faster than toenails.

• Nails take long time to grow from the matrix to the free edge:
o Fingernails take 3–6 months (2-3 mm/month).
o Toe nails take 12–18 m ( 1 mm/month).
Minor trauma
nail biting
NORMAL VARIATIONS

1- Common feature of aging:


• Increased or decreased thickness of
nail plate. age-related Longitudinal ridging
• Longitudinal ridging. ridging and beading
Longitudinal ridging
• Beading: resemble a candle's wax drippings. arranged longitudinally.
occurs at all ages but is more common in elderly. Also in rheumatoid
arthritis and in patients taking itraconazole.
2- Longitudinal Melanonychia
• Brown to black band, due to presence of melanin in nail plate.
• Seen in darker-skinned, pregnancy.
Note… sudden appearance of such a band in white
necessitates further investigation.
NAIL DISORDERS

Either 1ry (pathology in nail itself) OR


2ry to (cutaneous or systemic disease) (drugs) (environment).

NAIL SIGNS

Nail signs can be divided into three major categories:


1- signs due to abnormal nail matrix function.
2- signs due to nail bed disorders.
3- signs due to deposition of pigment within the nail plate.
Nail Signs due to Abnormal Nail Matrix Function

1-Beau’s Lines
• Transverse grooves on nail plate, that move distally with
nail growth. Often deeper in the center.
• Result from a temporary arrest of proximal nail matrix
proliferation (mitotic activity).
• Causes :
1. Local: Most commonly trauma (e.g. onychotillomania, manicures) OR
skin disease of proximal nail fold (e.g. eczema, chronic paronychia).
2. Systemic:
• Severe illness (e.g. erythroderma).
• Febrile illness (e.g. high fever, scarlet fever, measles, mumps, HFMD)
• Drugs (e.g. chemotherapy).
• Multiple digits involvement + presence of lines at same
level in all the involved nails suggests a systemic cause.
• Multiple lines in the same nail indicate repetitive insult.
• Self-limiting and requires no treatment.
2- Onychomadesis (nail shedding)
• Detachment of nail plate from the proximal nail fold.
• Result from a temporary arrest of proximal nail matrix
proliferation (mitotic activity).
• Causes: same as those for Beau’s lines + It may appear in
the setting of pemphigus vulgaris or SJS + In children often
relates to recent coxsackievirus infection (HFMD).
• Self-limiting and requires no treatment.
3- Pitting
• Small punctate depressions on nail plate surface.
• Result from foci of abnormal keratinization of
proximal nail matrix that result in clusters of
parakeratotic cells in dorsal nail plate, these
clusters are easily detached, leaving the pits.
• Seen in psoriasis, alopecia areata, eczema, fungal
infection or as normal variation.
Note… Large deep irregularly distributed pits in
psoriasis + atopic eczema. Small shallow regular
(rippled or arranged in longitudinal lines) in AA.
4- Onychorrhexis Onychorrhexis
• Multiple longitudinal ridges (raised line) and fissures
(groove) of nail plate.
• Often associated with nail thinning.
• Indicating severe nail matrix damage.
• Seen in lichen planus, trauma (frequent nail polish use)
chronic arterial insufficiency, systemic amyloidosis.
• D/D: the common age-related ridging.

age-related ridging

Note… Physiological (age-related) ridging:


Become more prominent with age. Appear as mild
ridges separated by shallow grooves.
5- longitudinal groove
• Appear as wide, deep, longitudinal depression.
• Occurs due to compression of the nail matrix
by tumours in proximal nail fold (e.g. myxoid
cyst, glomus tumour and wart).
• Disappears if the cause is removed.
Myxoid
cyst
6- Median canaliform dystrophy (Heller’s dystrophy)
• Longitudinal groove or split in center of nail plate. Often a fine short
cracks project from the line laterally giving appearance of inverted fir tree.
• Thumb is most often affected. Usually symmetrical.
• Causes: Idiopathic, inherited or self-induced trauma to nail matrix.
• There is no treatment.
• Nail can return to normal
after a few Months or years.
7- Trachyonychia
(Twenty-nail dystrophy, sandpapered nails)
• Nails are thin, opaque, lusterless and rough, it seem sandpapered in a
longitudinal direction. Cuticles are often hyperkeratotic.
• Changes does not necessarily involve all 20 nails.
• Roughness caused by excessive longitudinal striations.
• It is due to disturbance of nail matrix keratinization.
• Cause: Idiopathic or occur in association with alopecia areata (common),
lichen planus, psoriasis and eczema. improves spontaneously in majority
of pts. systemic corticosteroids used to treat alopecia areata and systemic
retinoids to treat psoriasis, also can improve trachyonychia.
8- Koilonychia (spoon-shaped nails)
• Nail plate is flat or spoon-shaped (In early stages there is flattening of nail
plate. Later, the edges become everted upwards and the nail appears
concave, giving the characteristic ‘spoon’ shape).
• Causes: occupational softening and iron deficiency are the most common
1- familial. 2- Idiopathic.
3- physiologic: in children’s toenails. resolves spontaneously.
4- Acquired: severe iron deficiency (Return normal with correction of
iron), Thyroid disease, alopecia areata, Darier’s disease, lichen planus,
psoriasis, thin nails of any cause (e.g. Occupational softening, old age,
peripheral arterial disease).
9- True leukonychia
The term leukonychia refers to white discoloration of nail. 3 subtypes:
• True; the pathology originates in matrix and emerges in the nail plate.
• Apparent; the pathology is in the nail bed.
• Pseudo; the nail plate pathology is exogenous, e.g. onychomycosis.
True leukonychia
• White opaque discoloration of nail plate,
that move distally with growth and doesn't
fades with pressure.
• Nail plate has a normal surface but loses
its transparency.
• Caused by diseases that disturb distal
nail matrix keratinization.
• Has 4 morphologic variants:
(i) Punctate: small opaque white spots. the most common
type of leukonychia. caused by trauma to nail matrix. most
commonly observed in fingernails of children.
(ii) Striate (transverse): single or multiple transverse white
parallel lines. frequently observed in; fingernails of women
due to matrix trauma from manicure, and great toenails due
to matrix trauma from shoes.

Note .. Mees’ lines (special form of Striate): white


transverse bands. seen in; arsenic and thallium
poisoning, leprosy, tuberculosis, after high fever
and during chemotherapy cycles.

(iii) Diffuse (Totalis. Porcelain nail): Nail plate is completely or


almost completely involved. milky porcelain white. it may be;
• Congenital: rare . all nails are involved.
• Acquired: cytotoxic drugs, leprosy.
(iv) Longitudinal: it may be a sign of
Onychopapilloma (benign neoplasm
of nail matrix), Hailey-Hailey Disease.

• D/D of True leukonychia:


1. Apparent leukonychia : It fades with pressure and
doesn't move distally with nail growth. See later ….

2. Pseudoleukonychia: sign of;


• White superficial onychomycosis. White superficial
• Proximal Subungual Onychomycosis.
• Chemical damage to nail keratin
(e.g. varnish).

Proximal Subungual
10- Onychauxis and Onychogryphosis
Onychauxis
• Onychauxis: refer to thickening (Hypertrophy) of nail
plate in general, without deformity (curvature).
If neglected (not trimmed), the nail may grow to form
Onychogryphosis.

• Onychogryphosis: nail plate is grossly thickened, hard and


deformed (curved) either oyster-like or ram’s horn.
It turned yellow–brown, with multiple transverse striations.
• Causes; Hereditary (Pachyonychia Congenita, EB), Ageing, repeated nail
trauma [e.g. tight shoes or trauma related to foot deformities (overlapping
digits)], Psoriasis, onychomycosis, Darier’s disease, congenital ichthyoses,
DM, poor circulation.
• D/D : Subungual hyperkeratosis.
Note.. (1) the term ‘nail Hypertrophy’ should be restricted to conditions
causing nail thickening by their effects on nail matrix. While ‘subungual
hyperkeratosis’ restricted to conditions leading to thickening beneath the
preformed nail plate, i.e. thickening of nail bed or hyponychium.
(2) In practice, differentiation b/w nail Hypertrophy and subungual
hyperkeratosis is difficult to define and mixed cases are commonly seen,
for example in psoriasis.
• Treatment:
o Treat the underlying cause (nail will start growing normal again).
o Continuous trimming. Reducing the thickness with nail file.
o Nail removing in extreme cases (e.g. severe pain or severe deformity).
o Recurrent cases (e.g. Hereditary, Age related) may require permanent
destruction of nail matrix with phenol.
Nail Signs due to Nail Bed Disorders

1- Apparent leukonychia
• The nail are pale white, due to nail bed discoloration, that fades with
pressure and does not move distally with nail growth.
• Often due to drugs (chemotherapy) OR
systemic diseases (e.g. Chronic renal and liver disease).
• 3 different types:
(i) Terry’s Nails: leukonychia affects the whole nail except for
1 to 2 mm distal normal band. common sign of liver cirrhosis
(80 % of pt). Also in chronic renal disease, adult-onset DM, Terry’s
also frequently seen in normal individuals.

(ii) Half-and-Half nails (Lindsay nails ): proximal white zone


and distal normal zone separated by a well-defined line.
Seen in up to 25% of pts with chronic renal disease on
hemodialysis. Chemotherapy. also seen in normal individual. Lindsay
D/D; Terry’s Nails and pseudo half and half nails in psoriasis.
(iii) Banded (Muehrcke’s) nail: multiple transverse whitish
bands parallel to lunula. typically spare the thumbnail.
Seen with chemotherapy and in pts with hypoalbuminemia
(e.g. nephrotic syndrome, glomerulonephritis, liver disease
and malnutrition).
Disappear when the serum albumin level returns to normal.

2- Subungual hyperkeratosis
• Accumuloation of scales under the nail plate, as a result of excessive
proliferation of keratinocytes in nail bed and hyponychium.
• Nail plate appears thick.
• Commonly seen in psoriasis, PRP, atopic dermatitis,
subungual warts and distal subungual onychomycosis.
3- Onycholysis
• Detachment of nail plate from nail bed.
• Starting at distal margin and progressing proximally.
• Signs:
1. Detached nail looks yellow-white (b/c of air in subungual space) OR
discoloured (green-black in pseudomonas aeruginosa).
2. Subungual scales in case of Psoriasis, wart, onychomycosis.

Onycholysis
with 2ry
Pseudomonas
infection
• Causes of Onycholysis:
1-Environmental: The most common. Occur as a consequence of:
• Repetitive water immersion and exposure to irritant (most common).
• Trauma. e.g. manicure tools pushing beneath the nail.
• UV (photo-onycholysis) i.e. non-drug-induced-photo-onycholysis.
2-Skin disease affecting nail bed:
• Inflammatory: Psoriasis, Lichen Planus, Eczema.
• Infection: candida, dermatophyte, Pseudomonas, Herpes simplex, HPV.
• Neoplastic: SCC.
3- Drug. e.g. Tetracyclines, Retinoids.
4- Drug-induced-photo-onycholysis e.g. Psoralen, OCP, Griseofulvin,
tetracycline (demecycline > doxycycline > tetracycline > minocycline).
5- Subungual exostoses.
6- Pregnancy.
7- Internal diseases: iron deficiency anemia, DM, hyper- hypothyroidism.
4- Splinter hemorrhages
• Appear as thin longitudinal dark-red lines due to deposition of
haemoglobin on the undersurface of nail, which grows out.
• occur due to rupture of the longitudinally oriented nail bed capillaries,
either by trauma, vascular disease (e.g. lupus), or inflammatory nail dx
(e.g. psoriasis or onychomycosis)

5- Erythronychia
See later …

Onychopapilloma
Chromonychia (nail colour changes)

Chromonychia means abnormality in color of the substance or the surface


of the nail plate and/or subungual tissue. May occur as a result of:
(1). Deposition of pigment. Either
• Endogenous, i.e. within the nail plate →
• Brown longitudinal streak due to deposition of melanin.
• Nail pigmentation with concave proximal border. Due to
deposition of pigments such as iron or gold.
Or
• Exogenous:
• On the dorsal nail surface. Follows the shape of proximal
nail fold → convex proximal border. e.g. yellow–brown in
smokers due to nicotine, dark nail due to hair dyes, red nail
due to Henna.
• On undersurface of nail. e.g. Pseudomonas → green color.
Splinter haemorrhages, deposit haemoglobin .

Onycholysis with 2ry


Pseudomonas infection
(2). Changes affecting the substance of the nail plate or the nail bed.
• Nail plate changes:
• The disruption of normal nail plate formation by alteration of the
normal cellular and intercellular organization, will result in loss of
normal lucency and produce true leukonychia.
• This disruption may occur due to disease (e.g. onychomycosis [OM],
psoraiasis), chemotherapy, poisons or trauma.
• The disruption can be achieved at nail formation, via the matrix, or
subsequently as in the case of OM, where discoloration may start distaly

• Nail bed changes:


• Vascular changes in nail bed (as in systemic disease or nail bed
tumour), Subungual hyperkeratosis or drugs (e.g. antimalarials,
chemotherapy) may change the apparent colour of the nail.
• Splinter haemorrhages appear as thin longitudinal dark-red lines, due
to deposit haemoglobin on the undersurface of the nail.
• Cyanosis makes the nail bed blue.
• Carbon monoxide poisoning makes the nail bed bright red.
1- White chromonychia (leukonychia).
2- Green nail syndrome
Nail has green–black color due to deposition of pyocyanin
(green pigment) and pyoverdin by Pseudomonas aeruginosa,
underneath the onycholytic nail plate. See later …
3- Blue chromonychia (Blue nails)
• Blue lunula: Drug (antimalarials , Zidovudine, hydroxyurea ), Argyria and
Wilson’s disease (hepatic degeneration).
• Blue nail bed: Drug (Minocycline. Antimalarials, Zidovudine), Glomus
tumor, Advanced AIDS infection.

NOTE (1) Argyria occurs in 2 forms; local (from topical


use of silver containing cream e.g. silver sulfadiazine
cream). systemic (Silver Poisoning from topical use,
inhalation or ingestion of silver).
(2) Transient bluish discoloration may indicate
Cyanosis or methemoglobinemia
bluish nail bed with sparing
of lunulae (Minocycline)
4- Red chromonychia (Erythronychia)
Red discoloration of nail. Disappear under pressure. Either limited to
lunulae Or Affect lunula and nail plate.
1- limited to lunula (Red lunula):
Redness does not extent over the borders of lunula. It may Affect;
• All digits: Spotted (mottled) in psoriasis, LP, AA and 20-nail dystrophy.
Diffuse in SLE, RA, CO poisoning, heart failure, hepatic cirrhosis.
• Single digit: Glomus tumour and subungual myxoid cysts are the most
common cause.

Diffuse red
Red spotted Red spotted with
(Psoriasis) Trachonychia
(AA)
2- Involve lunulae and nail plate:
Redness extent over the borders of lunula. Has 2 patterns;
i. Longitudinal erythronychia: linear red streak or band. Extending from
proximal nail to distal edge. It may:
• localized [i.e. single or paired (bifid) band in only 1 nail]: commonly
due to benign tumors (Onychopapilloma, glomus tumor) Or rarely
due to malignant (SCC, MM) or scarring of nail bed
• Multiples [i.e. multiple bands in many nails]: Characteristically
present in Dariers's disease. Other causes: LP, Amyloidosis, idiopathic.
ii. Diffuse erythronychia: SLE, CO poisoning, heart failure, liver cirrhosis

Band
Bifid

Onychopapilloma
5- Yellow chromonychia (Yellow nails)
causes;
i. Yellow-nail syndrome:
• Rare acquired condition.
• Defined by presence of yellow nails associated with
lymphedema and/or chronic respiratory diseases.
• Linear nail growth is arrested or greatly reduced.
• Nail are thick, diffuse yellow colored +/- Onycholysis.
• Associated with loss of both the cuticle and the lunula.
• In most pts, all 20 nail are involved.
• Treatment: not effective in all cases and must be prescribed for several
months; vitamin E 1200 IU/day + pulse itraconazole (400 mg daily for 1 wk
a month) or fluconazole (150 mg daily for 1 wk a month).
ii. Other : Onycholysis, Psoriasis, LP, tetracyclines(fluorescent lunula),
penicillamine. AIDS, RA, DM, Carotene.
6- Brown or black chromonychia (Melanonychia)
Melanonychia is the black-brown discoloration of nail due to deposition of
melanin. It has three Pattern:
1. Diffuse: chemotherapy, Antimalarial, Malnutrition, HIV.
2.Transverse black bands: chemotherapy, lichen planus.
3. Longitudinal.
Chemotherapy: Diffuse melanonychia
+ white bands (Muehrcke’s nail).
Longitudinal melanonychia:
• Longitudinal brown to black band extending from proximal nail fold to
distal margin. very commonly seen in darker-skinned (up to 90%).
• Due to deposition of melanin within nail plate. Single band may be a sign
of nail matrix nevi or melanoma. Multiple bands are usually due to
melanocyte activation. Causes:
1- Matrix melanocyte hyper/neoplasia: lentigo, nevi, melanoma.
2- Matrix melanocyte activation:
• Physiological: Racial, Pregnancy.
• Nail disorder: psoriasis, LP, amyloidosis, OM.
Note… OM due to T. rubrum (var. nigricans) and Scytalidium
dimidiatum can cause nail piment of nonmelanocytic origin
Congenital melanocytic nevus
• Drugs: chemotherapy (hydroxyurea, 5FU, cyclophosphamide),
antimalaria, zidovudine, psoralen, steroid, sulphonamide, minocycline,
MTX, azathioprine, phenytoin.
• Endocrine diseases: Addison’s disease, hyperthyroidism, acromegaly,
cushing syndrome.
• Nutritional: vitamin B12 or folate deficiency.
• Nonmelanocytic nail Tumors: Bowen’s disease
• ACTD: SLE, scleroderma.
• Trauma [manicure, nail biting, friction (primarily in toenails)
• Peutz-Jeghers syndrome
• AIDS.
NOTE (1) … An acquired single streak of dark pigmentation is a melanoma
until proved otherwise. The following features should be considered
indicative of possible malignant melanoma:
1. Only one digit affected
2. Periungual spread of the pigmentation (Hutchinson’s sign)
3. Change in appearance (for example, it may become wider or darker
over time with blurring of its border).
4. Age over 50 years.
NOTE (2) … Hutchinson’s sign
• Periungual extension of brown–black pigmentation from nail bed and
matrix to the proximal and lateral nail folds, hyponychium and cuticle.
• The development of periungual pigmentation in conjunction with
longitudinal melanonychia in adults is very suggestive of melanoma.
• Causes: • Melanoma, due to horizontal growth of the tumor.
• Racial (Skin type V and VI), Nail matrix nevi, Trauma,
malnutrition, Addison’s disease, Peutz-Jeghers syndrome,
Drug-induced, AIDS, Non melanoma tumor e.g. Bowen's disease.
Note… Pseudo Hutchinson's sign: The pigmentation can be observed
through the translucent cuticule.
Pseudo
Melanoma with Hutchinson's
Hutchinson sign sign
Nail changes due to cutaneous disorders
1- Darier’s Disease (Follicular Dyskeratosis)
• Nail changes in Darier are common, diagnostic and pathognomonic:
• Multiple red and white longitudinal streaks.
• The streaks often terminating in a V-shaped notching of distal margin.
• Wedge-shaped subungual hyperkeratosis.
• Similar changes may be seen in Hailey–Hailey disease.
NOTE … A single longitudinal erythronychia with distal subungual
hyperkeratosis is not sufficient for diagnosing Darier disease of nails, since
single bands may be due to a subungual benign tumor (e.g. glomus tumor,
onychopapilloma) and, less often, Bowen disease.
Darier’s Darier’s
2- Lichen Planus
• Nail changes present in 10% of patients. However, nail lichen planus is
most frequently seen in absence of skin, scalp or mucosal involvement.
• Clinical findings that are diagnostic include:
1. Nail thinning, longitudinal ridging and fissuring: Indicates nail
matrix involvement and the need for prompt treatment to avoid scarring.
2. Dorsal pterygium: Result from adhesion of proximal nail fold to nail
bed due to matrix destruction (scarring) and disappearance of nail plate.
• Other nonspecific nail changes: Red lunula. Nail bed lichen planus may
produces onycholysis, nail thickening, and yellow discoloration.
3- Psoriasis
• Most common dermatosis affecting the nail. Nail changes
seen in up to 50% of patients. Fingernails > toenails.
• Often associated with psoriatic arthropathy. patient with
nail Psoriasis has increased incidence of psoriatic arthritis.
• Koebner phenomenon worsen nail sign.
• It may affects:
(1) Nail folds: Typical psoriatic lesion.
(2) Nail Matrix:
• Pitting (most common): large, deep, irregularly scattered.
• Trachyonychia. • Longitudinal ridging • Thick yellow nail plat.
• Red spotted lunula. • Punctate leukonychia (pathognomonic)
• Nail Deformity: due to extensive involvement of nail matrix.
(3) Nail Bed
• Oil Spot (salmon patch): localized separation of nail plate. Cellular
debris and serum accumulate in this space giving brownish-yellow color.
• Onycholysis: The nail detaches in an irregular manner . Surrounded by
erythematous border. Nail plate turn yellow, simulating fungal infection
• Subungual hyperkeratosis: commonly mistaken for fungus infection.
• Splinter hemorrhage.
NOTE …
(1) Diagnostic signs of nail Psoriasis (fingernails only) are pitting, oil drop,
and onycholysis surrounded by an erythematous border.
(2) In toenails, psoriasis is usually clinically indistinguishable from OM.
(3) Nail psoriasis is often aggravated by sun exposure.
(4) Acrodermatitis continua of Hallopeau: A variant of localized type
Pustular Psoriasis (other variant is palmoplantar pustulosis). In most cases,
A single digit is involved + Nail involvement is a typical feature + It is not
associated with cutaneous plaques of psoriasis vulgaris. It presents with
recurrent episodes of acute painful inflammation with sterile pustules on
erythematous base around and under fingernail plate (less likely toenial) ,
followed by scaling and crust formation. Onycholysis may be seen.
4- Eczema
• Hand eczema is often associated with nail changes.
• In acute eczema, there are;
1. Vesicles and erythema of proximal nail fold and hyponychium.
2. Nail matrix damage produces irregular pitting and Beau’s Lines.
onychomadesis can occur in severe cases.
• Chronic eczema of hyponychium result in subungual hyperkeratosis,
onycholysis, and fissuring of hyponychium. Chronic eczema of
proximal nail fold can lead to chronic paronychia.
• In atopic dermatitis, the nail plate frequently shows irregular pits and
Beau’s lines.
• Controlling the skin disease results in gradual improvement of the nails.
5- Alopecia areata
• Nail abnormalities are present in ~20% of adults and 50% of children.
• Signs that are characteristic include geometric pitting and trachyonychia.
• The pits are small, superficial, and regularly distributed in a geometric
pattern (grid-like).
•Trachyonychia is more common in children and most frequently seen in
male patients with alopecia totalis or universalis.
• Additional nail abnormalities observed in alopecia areata include
punctate leukonychia, Mottled erythema of lunulae, and onychomadesis.
The nail and internal disease
1-Beau’s Lines 2-Yellow-nail syndrome 3-Terry’s Nails
4-Koilonychia 5-Clubbing

Clubbing
• Nail plate is enlarged and excessively curved +/-
enlargement of periungual soft tissue.
• The changes are permanent.
• There are 2 signs:
1.Schamroth sign: The normal diamond-shaped
window is obliterated.
2.Lovibond’s sign: The angle between proximal nail
fold and nail plate > 180° (normally = 160°).
• Causes: Idiopathic, hereditary–congenital, and
acquired. 80% of acquired are associated with
pulmonary disease. Other; CV disease, liver disease,
thyroid disease, inflammatory bowel disease, AIDS.
Enlargement of
periungual soft tissue
HIV Infection
1- Onychomycosis: common (up to 25%). Dermatophyte is most commonly
responsible, but Candida is also often isolated:
• Proximal subungual onychomycosis: due to T. rubrum. considered as a
marker of immunosuppression.
• Candida onychomycosis: Candida does not invade the nail plate of
immunocompetent. It indicates immunosuppression or chronic
mucocutaneous candidiasis.
2- Longitudinal melanonychia: Usually, several nails are involved.
3- HPV-induced SCC: Longstanding periungual warts in HIV pts should
always raise the suspicion of SCC [HPV types 16 and 35].

Drug Induced Nail Abnormalities


usually involve several or all nails.
Paronychia and periungual pyogenic granuloma: Retinoid, Methotrexate,
Antiretroviral drugs (indinavir, lamivudine), Epidermal growth factor
receptor (EGFR) inhibitors (cetuximab, erlotinib).
Cancer chemotherapeutic agent: the most frequent cause of nail change.
Beau’s line, onychomadesis, onycholysis, fragility, paronychia,
pigmentation, subungual hemorrhages, hematomas.
Colour changes due to drugs
• Tetracycline:
• Yellowing of nail. Rare, seen in prolonged therapy.
• Dark distal photo-onycholysis (associated with photosensitivity).
• The whole nail is affected and return to normal when discontinue drug.
• Mepacrinea:
• Bluish nail.
• Nails fluorescing yellow–green or white when viewed under Wood’s
light (Normal nails show slight fluorescence of violet–blue colour).
• Chloroquine:
• Blue-black pigmentation of the nail bed, (Other antimalarials may
produce longitudinal or vertical pigmented bands on nail bed or in nail).
• Inorganic arsenic:
• Mees’ lines.
• chemotherapy:
•Apparent leukonychia.
Infections

1- Acute Paronychia (Painful whitlow)


• Inflammation of proximal and/or lateral nail folds.
• Presented with painful red swelling of nail fold + purulent discharge or
accumulation of purulent material behind the cuticle.
• Cause: Usually infection, most commonly bacterial, in particular Staph-
aureus or strepto- pyogenes. Sometimes Pseudomonas. Recurrent acute
paronychia are due to HSV infection (Herpetic Whitlow).
• Cpx : permanent matrix damage. Deep extension forming a felon.
• D/D: Chronic paronychia, Drug-Induced Paronychia, acrodermatitis
continua of Hallopeau (type of localized pustular psoriasis).
• Treatment:
1. Drainage of the abscess to avoid matrix damage by inserting needle tip
between nail fold and nail plate and lifting the nail fold with tip of needle.
Pain is abruptly relieved as purulent material drains. [Note.. if there is no
abscess, treat with warm saline soaks three to four times a day].
2. Systemic antibiotics according to culture results (antistaphylococcal:
cephalexin, clindamycin, augmentine, trimethoprim, sulfamethoxazole).
3. Systemic antivirals when due to HSV.

Elevation of lateral nail fold


releases a purulent material
NOTE 1 … Felon
• infection of fingertip pulp. Presented with
painful, tender abscess with surrounding
erythema and swelling.
• Route of infection: Either penetrating
injury (e.g. blood glucose needle stick) Or
local spread (e.g. Acute paronychia).
• causes: Staph- aureus is the most common
organisim. Other includes HSV (herpetic
whitlow).
• Need urgent treatment to avoid CPX;
1. spread of infection to bone. 2. It can
compress blood vessels and cut off fingertip
circulation leading to permanent damage.
NOTE 2 : Chronic paronychia
• Not a yeast infection, rather, is a chronic inflammation of
nail folds that may be colonized by Candida or bacteria.
• Typically affecting hands that are continually exposed to
a wet environment and repeated minor trauma causing
cuticle damage.
• When the cuticle is lost, the epidermal barrier of proximal
nail fold is impaired. Irritants and allergens may easily penetrate proximal
nail fold and produce contact dermatitis that is responsible for the chronic
inflammation.
• Clinically, proximal and lateral nail folds becomes red, tender or painful,
and mild swollen with loss of cuticle. Proximal nail fold become separated
from the nail plate. With time the nail fold retracts and becomes
thickened and rounded.
• Recurrent acute exacerbations are common, Either due to:
2ry candidal and/or bacterial infections, causing self-limiting episodes of
painful acute inflammation, with small abscesses that drain spontaneously
explaining why such exacerbations subside without TTT in a few days. Or
by irritants or allergens.
• 2ry infections with Candida and Pseudomonas aeruginosa are common.
• Effect on nail plate: In the early stage, nail plate is unaffected, but later:
1. Lateral edges may develop irregularities and discoloration (yellow,
brown or black), this may extend over a large portion of the nail, and
occasionally the whole nail may become involved.
2.Damage to the nail matrix results in nail plate surface abnormalities
such as Beau’s lines, pitting and nail becomes rough and friable.
3. Eventually the size of the nail is considerably reduced.

• Typically, many or all fingers are involved simultaneously (In general,


acute paronychia involves only one nail).
• It disappears when the physiologic barrier is restored.
• The process is chronic and responds very slowly to treatment.
• D/D : Psoriasis of fingers may present in a similar form.

Thickened
rounded nail fold
• Treatment:
1- avoid exposure to contact irritants.
2- keep proximal nail fold dry.
3- Controlling inflammation is the primary goal by Topical steroid creams
or tacrolimus ointment 0.1% twice daily for up to 3 weeks is more effective
than systemic antifungals.
4- Miconazole topical suspension at proximal nail fold to flow into the
space created by the absent cuticle, 2 or 3 times a day for weeks, until the
cuticle is re-formed.
5- Topical antiseptics (e.g. 4% thymol in 95% ethanol).
6- Cuticle may never re-form in pts with long standing inflammation. So
Fluconazole (150 mg/day) for 1-4 weeks may control chronic inflammation.

NOTE 3 : Drug-Induced Paronychia


1. Retinoids.
2. Epidermal growth factor receptor inhibitors: may cause Paronychia,
periungual pyogenic granulomas, and xerosis.
3. Antiretroviral drugs e.g. lamivudine and indinavir [used to treat HIV]:
reported to cause paronychia and ingrown toenails in about 4% of pts.
Other lesions include Staphylococcal superinfection, onycholysis, severe
xerosis , pyogenic granuloma like lesions. Complete regression occurs
within 9 to 12 weeks after drugs are withdrawn.
NOTE 4 : Herpetic paronychia (Herpetic Whitlow)
• Herpes simplex infection of the pulp of the distal phalanx.
• Results from direct inoculation by either HSV-1 or HSV-2.
• Often in young children, usually due to HSV-1.
• Increasing frequency in adolescents/adults, due to HSV-2.
• Clinically:
1. A prodrome of tender lymphadenopathy and fever may
occurs before the onset of lesions.
2. During the incubation period (3–7 days), local pain, tenderness,
tingling. erythema and swelling of fingertips may develop.
3.After the incubation period, Painful, grouped vesicles appear on an
erythematous base and may become umbilicated, that soon progress to
pustule, erosion and/or ulcerations with a characteristic scalloped border.
4.For 10–14 days the vesicles increase in size, often coalescing into large,
honeycombed bullae. New crops of lesions may appear during this time.
5. Crusting of lesions and resolution of symptoms typically occurs within
2 to 6 weeks.
6. Recurrent lesions are often fewer in number, with decreased severity
and duration compared to those of a primary infection.
• Complications:
1. Lymphangitis and painful lymphadenitis.
2. Numbness of the finger following infection.
3. 2ry bacterial infection.
4. Recurrences.
• Diagnosis:
1- viral culture. 2- Direct fluorescent antibody assays (DFA). 3- PCR.
4- Tzanck smear of scrapings from early lesions (e.g. the base/edges of a
freshly unroofed vesicle): reveals multinucleated epithelial giant cells in
the majority of HSV cases (60–75%).

• D/D: Often misdiagnosed as paronychia. Recurrences in the same


location can be a clue to the diagnosis.

NOTE 5 … Causes of paronychia


• Infective (Viral, Bacterial, Fungal).
• Drugs (e.g. Retinoids, EGFR inhibitors, lamivudine and indinavir).
• Trauma (e.g. Ingrowing toe nail).
• Dermatoses (e.g. Contact dermatitis, Lichen planus, Psoriasis).
2- Pseudomonas Infection (green nails syndrome)
• Bacteria are not capable of attacking a healthy nail plate.
• Pseudomonas aeruginosa (G –ve) may colonize dorsal or ventral nail plate
surface under propitious conditions e.g. Onycholysis, chronic paronychia.
• Nail plate assumes a yellow–green to green–black color due to deposition
of pyocyanin and pyoverdin pigment by Pseudomonas aeruginosa.
• There is little inflammation, discomfort but no pain.
• D/D : subungual hematoma.
• TTT: eliminate the colonization from nail surface by applying a drops of
2% sodium hypochlorite solution and from subungual space by soaking
affected digits in the same solution for 5 minutes per day for 20–30 days.
No need for systemic antibiotics.
3- Warts
• Periungual warts are the most common periungual growth, it may extend
deep under the nail (subungual).
• Warts appear as keratotic papules and plaques.
• Complication:
o Nail bed wart can lead to subungual hyperkeratosis and onycholysis.
o Warts over the nail matrix can lead to longitudinal nail groove.
o Massive warts can erode the underlying matrix by displacement.
o SCC can arise in or mimic a Wart.
4- Onychomycosis (OM)
• A term used for all fungal nail infection, including those due to;
I. Dermatophytes (tinea unguium): the most common cause of OM.
Account for 90% of cases of toenail OM (50% of fingernail OM).
Men > Women. Toenail > fingernail. Frequently associated with chronic
tinea pedis. Unlike most other fungi, it produce keratinase (enzymes that
break down keratin). All dermatophyte can cause tinea unguium. Two
major pathogen are responsible for 90% of all OM cases, T. rubrum (70%)
and T. interdigitale (old name: mentagrophyte var. interdigitale) (20%).
Other pathogen are T. tonsurans (in children) and E. floccosum.
Microsporum spp. are very rare.

II. Non-dermatophytes: Account for 10% of all cases. Includes;


 Non-dermatophyte molds (NDM): Mainly toenails.
• Fusarium, Aspergillus, acremonium spp → Deep variant of SWOM.
• Scytalidium dimidiatum → black discoloration of nail.
• Scytalidium hyalinum → white discoloration of nail.
 Yeast: Candida albicans accounts for 70% of candidal OM.
• The toenails are affected in about 80% of cases of OM.
• Symptoms: Initially, it is usually asymptomatic. As the disease progresses,
Pts may report discomfort and pain that interfere with trimming, standing,
walking, and exercising.
• There are several clinical presentations of OM:
1. Distal and lateral subungual OM. 2. Proximal Subungual OM.
3. Superficial white OM. 4. Endonyx. 5. Candidal OM.
6. Mixed pattern (with ≥ 2 of the above patterns in the same nail).
7. Total dystrophic OM (the most sever and advanced form of any subtype)

Different entry points by


infecting organisms
Distal/lateral subungual onychomycosis (DLSO):
• The commonest clinical variant affecting both finger and toenails.
• Nearly always caused by dermatophytes. Subungual debris
• The commonest causative species is T. rubrum
followed by T. interdigitale.
• The fungus enters via distal and lateral nail groove,
invade hyponychium and/or nail bed, and spreads
proximally through the under surface of nail plate,
forming linear channel. Channeling is a highly
characteristic feature of a fungal infection.
• Clinically, there are:
(1) Subungual hyperkeratosis which act as a mycotic linear channel
reservoir for fungal proliferation. (2) nail plate
becomes thick and opaque (turn yellow or creamy).
(3) onycholysis. (4) linear channel. (5) Finally, entire
nail bed and plate may involved giving total
dystrophic pattern.
• Surrounding skin is nearly always affected by T. pedis
Note …
• One hand two feet tinea syndrome:
This is a distinct clinical pattern in DLSO
caused by T. rubrum. in which the fungus
spreads from the plantar and palmar surface
of feet and hands.

• Chronic dermatophytosis syndrome:


Caused by T. interdigitale. starts as minute plantar vesicles of 1 mm size
and collarettes over the soles that are the site of abundant hyphae. The
vesicles dry leaving a keratinous collarette and later on other sites become
infected, especially the nail bed, leading to DLSO.
Superficial white onychomycosis (SWO):
• Caused by direct penetration into the dorsal surface of nail plate.
• Nail plate is the primary site of invasion. It confined to the toenails.
• There are 4 variants:
1. Classic form:
• Often due to T. interdigitale.
• Discrete small, white, speckled or powdery patches on the surface of
nail plate which may coalesce to involve the entire nail plat.
• Nail become rough and friable, but not thickened and no Onycholysis
(i.e. remain adherent to nail bed).

Powdery
patches Diffusely opaque

Discrete small
patches
Speckled patches
2. Transverse striate bands variant. • More likely caused by T. rubrum.
3. Origination from proximal nail fold. • The more invasive forms seen in
4. Deep variant (diffuse involvement of healthy children and
nail plate + presence of fungi in both immunocompromised pts.
superficial and intermediate layers). • Deep variant can also result from
NDM such as Fusarium, Aspergillus

Transverse striate Bands

Deep
Proximal Subungual Onychomycosis:
• The most common pattern seen in patients with AIDS.
• T. rubrum is the most common cause.
• Microorganisms enter the Proximal nail fold cuticle
area, migrate to the matrix, and finally invade the nail
plate from below.
• Infection occurs within the substance of nail plate, but
the surface remains intact (but it may involve the entire
thickness of proximal plat).
• Presents as an area of leukonychia in proximal nail
plate (Proximal leukonychia) that moves distally with
nail growth + subungual hyperkeratotic debris that may
cause proximal onycholysis.
• In the cases caused by molds, leukonychia is typically
associated with marked periungual inflammation with
purulent discharge.
Endonyx
Presents as a milky white discoloration of nail plate. Nail plate is smooth.
There is no subungual hyperkeratosis or onycholysis.
Total dystrophic onychomycosis:
• The most advanced form of any subtype. It involves the entire nail unit.
• The nail matrix may become permanently scarred, and the nail plate can
be completely destroyed.
• Start as a thickened, opaque, and yellow-brown nail, that finally
disappears leaving a thickened nail bed retaining keratotic nail debris.

Thickened nail bed


retaining keratotic nail
debris thickened,
opaque, yellow-
brown nail

thickened nail bed retaining


keratotic nail debris
Candidal onychomycosis (CO)
• More commonly affects the fingernails > toenails.
• About 50% of fingernail OM is caused by Candida spp.
• Candida albicans is the most frequent species causing CO.
• CO is observed in children (C. albicans is a common cause of OM in
children < 3 years ), immunocompromised pts, and in CMC.

• who are at risk for developing CO?


• People who frequently immerse their hands or feet in water.
• Pts with chronic mucocutaneous candidiasis (CMC). They are usually
affected by special type of onychomycosis called Candida granuloma.

• CO is increasingly found in pts with defective/lowered immunity e.g.


aging, DM, vascular disease, immunosuppression, and broad spectrum Ab.
• CO generally presents in one of these patterns:
1. Candida paronychia.
2. Candida Onycholysis.
3. Candida granuloma
1. Candida paronychia: The most common form of CO. Begins as infection
of the soft tissue around the nail (i.e. paronychia). There is painful swelling
and erythema of proximal and lateral nail folds, from which pus can be
expressed at times. After that, Candida penetrate the nail matrix and plate.
Infection of nail matrix results in Beau's lines. The end result is thickened,
rough, irregular nail with patches of opacification or discolouration (white,
yellow, green or black) and finally dystrophic nail. Usually, pressure on the
nail causes pain. There is no subungual hyperkeratosis.
2. Candida Onycholysis: In this case, distal subungal
hyperkeratosis lifts off the nail plate.

3. Candida granuloma: < 1% of OM. Seen in immunocompromised pt with


CMC. In this severe form, the Candida invades the nail directly (without 1st
involving the surrounding tissues) and affects the full thickness of the nail.
In advanced cases there is swelling of the lateral and proximal nail folds
that may end up with a digit
deformity called
“chicken drumstick” or
“pseudoclubbing”
• Complication of onychomycosis:
It is reported in elderly, immunocompromised and person with DM:
1. Nail dystrophy: ≥ 50% of nail dystrophy are due to onychomycosis.
2. Cellulitis. 3. Osteomyelitissepsis. 4. Tissue necrosis.

• Differential Diagnosis:
1- Psoriasis: pitting is the single distinguishing feature of psoriasis. pitting
is not a feature of fungal infection.
2- Bacterial infection: especially Pseudomonas aeruginosa, which turns
the nail black or green.
3- Onychauxis and Onychogryphosis
4- True Leukonychia: White opaque discoloration of nail plate, that move
distally with growth. Nail plate has a normal surface.
5- Onycholysis.
6- Eczema and habitual picking of proximal nail fold: induces the nail
plate to be wavy and ridged, but its substance remains intact and hard.
• Laboratory Diagnosis:
KOH examination, culture, and occasionally nail biopsy.

Nail Collection Techniques for KOH


• Before obtaining a specimen, the nails must be clipped and cleansed with
an alcohol swab to remove bacteria and debris.
• In DLSO, obtain a specimen from beneath the nail plate. Cut back the
onycholytic nails to the most proximal point and take a subungual and nail
bed samples at a site most proximal to the cuticle, where the concentration
of hyphae is greatest. The outermost debris should be discarded.
• In PSO, use a surgical blade to remove the overlying dorsal nail plate,
then sample the ventral nail plate. OR perform punch biopsy to include
material from nail bed.
• In WSO, use a surgical blade to take a specimen from nail surface.
• In suspected CO, specimens should be taken from the affected nail bed
closest to the proximal and lateral edges.
• Nail fragments must be small.
Clip the nail plate to expose subungual
material for KOH examination

Remove subungual debris for


KOH examination
Fungal hyphae: The identifying characteristic is bending, branching,
filamentous (hyphae) structure that is uniform in width. Other features
are translucent, lines of separation (septa) appearing at irregular intervals.
Note: the thin lines represent the cell walls of the keratinocytes
Treatment
• Treatment and prevention guidelines
1. Preventive measures: breathable footwear. use antifungal or absorbent
powder in shoes. Keep feet dry. frequent nail clipping, not going barefoot
in locker room. discard or treat old shoes with disinfectants or antifungal
powder (often harbor large numbers of infectious organisms). Treat
tinea pedis (the likely reservoir for relapse). Treat close family contacts..
2. Confirm the diagnosis before systemic treatment (e.g. KOH, culture).
3. Identify poor prognostic signs (see later).
4. Periodic debridement and trimming of infected nail during course of
treatment increase cure rate.

• Oral antifungal therapy is usually required to achieve a complete cure,


with the occasional exception of classic superficial white onychomycosis.
• Oral terbinafine, itraconazole, and fluconazole have cure rates of up to
80%. penetrate keratinizing tissue. level reached in nail plate exceed those
in plasma. Their therapeutic level persist in nail for at least 1 month after
discontinuation of drug.
• Tavaborole 5%, ciclopirox 8% solutions and efinaconazole 10% solution
are FDA-approved topical therapies for mild to moderate onychomycosis.
• Terbinafine: 1st line therapy for dermatophytic infections (most cases of
onychomycosis). has higher cure rates and slower relapse rate. relatively
free of drug interactions. Has the highest mycologic cure rate (up to 88%):
 Continuous: the optimal therapy for onychomycosis. 250 mg/day/12 wk
in toenail infection (6 wk in fingernail infection). It is more effective and
safe than pulse dosing itraconazole. Mycologic cure rate up to 88%.
 Pulse dosing (intermittent): 500 mg/day for 1st wk of each mo/ 3 pulses.
It is less effective but more safe than continuous. Mycologic cure rate 58%

• Itraconazole: Alternative 1st line therapy of dermatophytic infections.


Preferred therapy for candidal and non-dermatophyte molds infections.
 Continuous: 200 mg/day/12 wk in toenail infection (6 wk in fingernail)
Mycologic cure rate 63%.
 Pulse dosing: 200 mg bid for 1st wk of each mo/ 2-3 pulses in fingernails
(3-4 pulses in toenails). Mycologic cure rate 38%.

• Fluconazole: 1st line therapy for candidal, but also active against
dermatophytes. Mycologic cure rate 47%.
 150-450 mg each week for 9 months or until nails are clear in toenails
(6 months or until nails are clear in fingernail).
• Nonpharmacologic approaches include the following:
1. Laser treatment (Nd: YAG laser. Diode laser). 2. Photodynamic therapy.
3. Chemical or surgical nail avulsion:
• Surgical Nail Removal: for Painful or extremely infected nails
• Nonsurgical Avulsion of Nail Dystrophies: Painful or very thick nails
can be removed with 40% urea cream under occlusion. This technique
can be used to treat other hypertrophic nail conditions, such as psoriatic
nails. It is also facilitates treatment with topical antifungal agents.
Note… Pregnancy and lactation
• Terbinafine is category B, itraconazole and fluconazole are category C.
• Use of all these oral drugs should be avoided in pregnancy.
• All oral antifungals are excreted in breast milk, therefore C/I in lactation.
Note… Indications for topical monotherapy include:
1. Superficial white onychomycosis (SWO).
2. In children with thin, fast growing nails.
3. No matrix area involvement.
4. Involvement limited to distal 50 % of nail plate, 3 or 4 nails involvement.
5. As prophylaxis in patients at risk of recurrence.
6. Patients where oral therapy is inappropriate.
From Bolognia dermatology book 4th edition (2018)
Areas of nail
involvement >50%

Significant
lateral disease

Subungual
keratosis >2 mm

Total dystrophic
onychomycosis
Environmental nail disorders
1- Chronic paronychia
2- Brittle Nails
• Brittleness meaning that the nail easily break, split or
peel off. It is very common and usually affect women.
• It can manifest in different ways: Onychoschizia

* Onychoschizia: Transverse splitting of distal nail into layers ( peeling).


* Dryness (too little moisture) or softening (too much moisture).
* Longitudinal splitting. * Thinning. * Onychorrhexis.
• Causes:
1. Eexternal factors that may Dry the nail plate. e.g. repeated soaking of
hands in water (the usual cause, especially hot water). OR
Soft the nail plate. e.g. exposure to chemical such as detergents and nail
polish removers (especially the acetone containing).
2. Nail diseases that disrupt nail formation and structure. e.g. Psoriasis,
Lichen planus, onychomycosis.
3. Dermatological conditions that may thin the nail plate. e.g. Alopecia
areata, Amyloidosis, Darier’s disease, Eczema.
4. General factors. e.g Aging, Pregnancy, iron deficiency, oral retinoids.
Note.. 1- If brittleness involve only fingernails, it is likely due to external
factor. whereas, if the toenails also involved, this will suggest other causes.
2- proximal onychoschizia seen in Psoriasis, Lichen planus, oral retinoids.
• Treatment of Brittle Nails
1. Wearing cotton lined rubber gloves (protect from water and chemical).
2. Topical moisturizer for dry nail after 1st soaking nail in water for 5 min.
3. Biotin (2.5–5 mg/day) for at least 6 months (C/I in pregnancy).

3-Idiopathic (environmental ) Onycholysis


 Detachment of nail plate from nail bed as a consequence of:
• repetitive water immersion and exposure to irritants (the most common).
• Trauma. • UV (photoonycholysis) without medication.
 Signs • Detached nail looks yellow-white (b/c of air in subungual space)
• Discoloration underneath the nail may occur as a result of 2ry infection,
e.g. green-black in pseudomonas aeruginosa.
• Absence of subungual scales is characteristic [+ve in Psoriasis, Candida,
dermatophytes, HPV].
 TTT: Avoid water and chemical exposure. Clip the detached nail plate.
topical antiseptics.
Traumatic Nail Abnormalities
1- Self-induced nail trauma
I. Onychophagia (Nail biting):
• The habit of biting the free edge of the nails.
• Bitten nails are short and irregular.
• Treatment: Topical preparations that taste
unpleasant and bandages.

II. Onychotillomania:
A . Nail picking:
• Uncontrollable desire to pick at, tries off, or
harmfully bite the nails. It may also affect
proximal nail fold.
• Often produces nail matrix damage with 2ry
nail plate abnormality, e.g. surface irregularity,
longitudinal melanonychia.
• Treatment: Topical preparations that taste
unpleasant and bandages. Serotonin reuptake
inhibitors (e.g. paroxetine).
B . Habit-tic deformity:
• Due to nervous habit of picking, rubbing and pushing back the
midportion of cuticle of thumb by index finger, causing injury to the
underlying nail matrix.
• The nail plate shows multiple midline Beau’s lines that resemble a
washboard (i.e. longitudinal band of horizontal grooves).
• The lunula is usually large.
• The proximal nail fold skin is often thickened, scaly or otherwise
abnormal reflecting manipulation of the skin (picking).
• When proximal nails and nail fold are covered with a tape continually,
normal nails regrew in 5 months.
2- Subungual Hematoma
•Collection of blood under nail plate. May caused by trauma, which cause
immediate bleeding and pain. Quantity of blood may be sufficient to cause
onycholysis. Compression of the matrix may cause 2ry nail plate dystrophy.
• Subungual hematomas migrate distally with nail growth.
• The color will change over time initially red to purple and later to dark
brown and black as the blood clots.
•D/D: some cases can be difficult to distinguish from subungual melanoma
by the naked eye, so Dermoscopy is useful in these cases.
• Treatment: If the hematoma is large and causing pain, drainage of blood
is required, by creating a hole through the nail plate, either by a red-hot
paperclip tip (the quickest and most effective+ painless) or with
hypodermic needle (painful).
This relieve the pressure and
give the patient immediate
pain relief.
3- Traumatic Toenail Abnormalities
Most commonly seen in women who wear high-heeled pointed shoes, but
can also be seen in athletes. They are often bilateral.
I. Traumatic onycholysis of the hallux: the most common
clinical presentation. Clipping of detached nail plate reveals
normal nail bed. The differential diagnosis includes onychomycosis, but
here the onycholysis is associated with subungual hyperkeratosis.
II. Transverse leukonychia of hallux: results from repeated
microtrauma by shoes to untrimmed, long, great toenails.
Multiple bands of true leukonychia move distally with nail growth.
III. Frictional melanonychia: affects the toenails of the fourth and/or fifth
digit of women. It is due to activation of nail matrix melanocyte by friction
from shoes or from the adjacent digit. It may be multiple and black color.
IV. 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.
4- Onychogryphosis
• Common in elderly persons and almost exclusively affects the toenails,
usually the hallux. The nail plate is grossly thickened, hard
and deformed (curved) either oyster-like or ram’s horn.
•It turned yellow–brown, with multiple transverse striations.

5- Pincer Nails (Trumpet Nails)


• Inward folding of lateral edge of nail plate (i.e. transverse
overcurvature) result in pincer-shaped nail (i.e. lateral borders
pinch the soft tissue). Eventually, soft tissue may disappear.
• In extreme cases the lateral borders may join together.
• Can be associated with severe pain.
• Usually seen on the toes. May be;
• Hereditary. OR
• Acquired: wearing ill-fitting shoes (most common),
psoriasis, subungual exostosis.
• Treatment: Lateral chemical or surgical matricectomy is the
treatment of choice for pincer nails.
6- Ingrown Toenails (Onychocryptosis)
Nail pierces the nailfold epithelium, where it acts as foreign
body, causing acute inflammation (painful swelling).
Chronic inflammation then leads to formation of
granulation tissue alongside the penetrating nail.

I. Lateral ingrowing: usually affects hallux of young adults with congenital


malalignment of great toenails. Precipitating factors include improper or
aggressive nail cutting, trauma and hyperhidrosis. The latter contributes to
fragmentation of the distolateral edge.

II. Distal embedding: common complication of nail avulsion. The nail


plate growth is blocked by the hyponychium, which forms a distal rim. Can
also occur in young infants but it resolves spontaneously.

III. Retronychia: ingrowth of proximal nail plate into proximal nail fold,
with one to three nail plates misaligned beneath the uppermost nail plate.
There may be associated proximal periungual pyogenic granulomas.
Treatment of lateral ingrowing nail:
1- Ingrown nail without Inflammation: Separate distal anterior tip and
lateral edges of ingrown nail from the adjacent soft tissue with a wisp of
absorbent cotton coated with collodion. This gives immediate relief of pain
and provides a firm runway for further growth of nail. collodion fixes the
cotton in place. may need reinsertion in 3 to 6 weeks. Cotton without
collodion may be used, but it may have to be replaced frequently.

2- Ingrown nail with Inflammation → surgical removal of the lateral nail.


Granulation tissue is removed with a curette or reduced by topical
antibiotics and topical corticosteroids. inflamed site is treated with wet
compress until swelling and inflammation have subsided.

3- Recurrent or severe cases: permanent destruction of lateral portions of


nail matrix by liquid phenol, laser or excisional surgical remove.
Nail Tumors
1. Benign Tumors and Proliferations: Pyogenic granuloma, Periungual
Fibroma, Acquired fibrokeratoma, myxoid cyst, glomus tumor,
Onychomatrixoma, Onychopapilloma, nevi, wart, Subungual exostosis
2. Malignant tumors: SCC, Bowen disease, Keratoacanthoma,
Verrucous carcinoma, melanoma, Merkel cell tumor.

Benign Tumors
1- Pyogenic granuloma (Botryomycoma)
• Commonly appear within the nail apparatus. It may be periungual or
subungual. Appears as a bleeding, friable, soft red papulonodule. When
subungual, it is associated with onycholysis.
• Causes: often follow penetrating trauma. Other include
ingrown toenails, systemic drugs (e.g. retinoids,
EGFR inhibitors), frictional onycholysis.
• Treatment is surgical.
• D/D: amelanotic melanoma.
2- Periungual Fibroma
• Benign fibrous tissue tumors that can be;
• Acquired; due to trauma, also in old age. Or
• Congenital; associated with tuberous sclerosis (referred to as Koenen
tumours) and Neurofibromatosis type I.
• Appear as pink or skin colored, fusiform-shaped , firm, protruding papule
or nodule, originating from the proximal nail fold. It may compress the
nail matrix and produce a longitudinal groove in nail plate.
• It may originate from nail bed and grow under the nail plate (Subungual
fibroma), producing longitudinal erythronychia or onycholysis.
NOTE… Koenen tumours; Multiple periungual fibromas, seen in 50% of
tuberous sclerosis patients.

fusiform
3- Acquired periungual fibrokeratoma (APF)
• Clinically and histologically identical to acquired digital fibrokeratoma.
• Rare tumor. Usually presents as;
• Solitary (Multiple APF is rare. May misdiagnosed as Koenen tumors).
• Firm, smooth, skin colored papules.
• Protruding on nail plate from beneath the cuticle (sometimes from
matrix or nail bed) with hyperkeratotic tip.
• May be surrounded by a collarette of raised skin.
• D/D of APF: Periungual fibroma (Koenen’s Tumor), Wart, keloid,
Pyogenic granulomas, cutaneous horn, exostosis.

APF

acquired digital fibrokeratoma


surrounded by a collarette of raised skin
4- Myxoid cyst (mucous cyst)
• A pseudocyst because it is not surrounded by a capsule, unlike a true cyst.
• The most common nail tumor. favor middle-aged women.
• Typically located in the proximal nail fold of the fingernails.
• Appear as small, soft, shiny, translucent, skin-colored papulonodules.
• Jelly-like sticky fluid may be expressed from the cyst.
• Compression of the matrix produces nail plate depression and grooves.
• Occasionally, the cysts are subungual and connected to the DIF joint by a
tract, and osteoarthritis of the this joint is a frequent association.
• Treatment: Cryosurgery and IL corticosteroid injections are possible
treatments, but they associated with a high
frequency of relapses. Definitive cure of this
cysts requires a surgical procedure.

Jelly-like sticky fluid


5- Glomus tumor
• Arises from the neuromyoarterial glomus cells of nail bed dermis.
• It is associated with severe pain that radiates proximally and is often
aggravated by physical (e.g. compression) or thermal stimuli (especially
cold). Subjective symptoms typically exceed clinical signs.
• Appears as Red–bluish, nail bed macule, visible through the nail plate.
• MRI allows the diagnosis in doubtful cases.

presented with pain and ill-


defined subungual erythema
6- Onychomatricoma
• Rare neoplasm that produces localized or diffuse thickening of nail plate
with multiple longitudinal hollows that contain the digitating tumor.
• The affected nail is thickened and yellow–white in color, with transverse
overcurvature and multiple splinter hemorrhages. frontal view of the nail
typically reveals multiple holes in the thickened free margin.
• May leads to longitudinal melanonychia.
7- Onychopapilloma
• Arises from the distal matrix/proximal nail bed and grows under the nail
plate to the distal nail margin.
• It has a thin filiform shape. Usually affecting one nail.
• Small tumors produce thin longitudinal erythronychia while larger one
induce thinning of the overlying nail plate with distal fissuring and a
subungual mass. Less often the tumor manifests as melanonychia or
longitudinal leukonychia or splinter hemorrhages.

longitudinal leukonychia
Longitudinal erythronychia and
leukonychia with focal hemorrhage
8- Melanocytic nevi of nail matrix
•Uncommon cause of longitudinal melanonychia, especially in comparison
with melanocyte activation. Usually develop during childhood.
• Often involve the fingers, especially the thumb. The color, width, and
pigment distribution may vary considerably and it is not unusual to
observe fading or darkening of the pigmentation over time.
• pseudo-Hutchinson’s sign is also possible.
• The age at onset represents the most important clue to the diagnosis.
• Histologically, a junctional nevus is usually seen.
• Treatment: Optimal management of nail matrix nevi is still debated.

Melanocytic nevi of nail matrix presented


with longitudinal melanonychia
9- Subungual exostosis
• The most common benign bony proliferations associated with nail
abnormalities. Commonly precipitated by trauma.
• Usually seen on the great toe of young patients, as hard tender subungual
nodule that elevates the nail plate. The nodule may ulcerate or become
hyperkeratotic. The diagnosis is confirmed by X-ray examination.
Malignant Tumors
1- SCC
• The most common malignant tumor of the nail apparatus. Often affects
the fingernails of middle-aged men. A number of studies have confirmed a
causative role for HPV, usually HPV-16, in the development of this tumor.
• Clinically; slowly growing periungual or subungual verrucous mass that
may ulcerate and bleed. It can lead to onycholysis or nail plate destruction.
• Compared to cutaneous SCC elsewhere, nail SCC tends to more quickly
become invasive, but metastases are rare.
• Bone invasion occurs in <20% of patients and the osteolysis detected on
X-ray examination is more often due to
compression rather than true bone invasion.

Subungual SCC
2-Bowen’s disease (SCC in situ)
• Bowen disease of nail is uncommon and is seen most often in middle-
aged men.
• Clinically, it may be difficult to differentiate it from warts. The affected
digit shows periungual or subungual verrucous lesions with onycholysis
and/or longitudinal melanonychia.
• Predisposing factors include HPV infection and chronic X-ray exposure.

The lateral portion of the nail


plate is absent. The nail bed shows
hyperkeratosis with scaling and
fissuring of the epithelium. Fig
3- Verrucous carcinoma
(Carcinoma cuniculatum, Epithelioma cuniculatum)
• A rare, low-grade variant of SCC 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 verrucous nodule that often destroys the nail.
• Bone resorption is common.
4-Keratoacanthoma
• KA of nail is extremely rare and usually affects a single digit.
• Appears as a painful, subungual, keratotic nodule that grows rapidly over
a period of weeks.
• Deep invasion with bone destruction frequently occurs, with evidence of
osteolysis on X-ray examination.
• A history of trauma is often reported.
• In contrast to cutaneous KA, it does not regress spontaneously.
Osteolysis
5- Melanoma (Melanotic whitlow)
• Nail melanoma is rare, accounting for 0.7–3.5% of all melanomas. Most
frequently involves the thumb of middle-aged individuals. 50% of patients
report a history of trauma. In 25% of patients the tumor is amelanotic.
• Historically, the 5-year survival is only 15%.
• The growth is usually painless and slow, and it can occur anywhere
around or under the nail.
• Clinically, nail melanoma may have the following presentations:
1. Longitudinal melanonychia: Usually dark brown or black in color,
with blurred margins and a width >3–4 mm. An ABCDEF “rule” has
been proposed to aid in diagnosis (next slide).
2. Nail plate abnormalities due to nail matrix damage.
3. Subungual pigmented lesion that may have ulcerated and is
associated with onycholysis and/or nail destruction.
4. An amelanotic nodule that frequently ulcerates and bleeds,
resembling a pyogenic granuloma.
5. Hutchinson’s sign, due to radial spread of the tumor.
• Dermoscopic Findings in nail melanoma. See later …
Nail Melanoma
The melanoma arose in nail matrix Amelanotic Melanoma
of thumb with resultant nail plate Note the diffuse nail
dystrophy and extension into the destruction and ulceration
proximal nail fold (Hutchinson sign)
ABCDEF rule for clinical suspicion of nail melanoma

(A) • Age : peak incidence 5th to 7th decades of life.


• Africans (dark-skinned races).

(B) • Black or Brown.


• Band width of 3 mm or more.
• Blurred margins.

(C) • Change in the nail or lack of improved change with treatment.

(D) • Digit most commonly involved (thumb).

(E) • Extension of pigment onto proximal and/or lateral nail fold


(Hutchinson’s sign).

(F) • Family or personal history of dysplastic nevus or melanoma.

Note… Except for criterion F, these represent validated clinical criteria.


CONGENITAL AND HEREDITARY NAIL DISEASES

1- Racquet Thumbs (Usually AD)


• Common congenital malformation
• Due to shortening of distal phalanx.
• Short and wide nail (Brachyonychia i.e. width > length).
• Racquet nails are usually an isolated finding.

2- Nail Patella Syndrome (Onycho-Osteodysplasia) (AD)


• Nails are absent or hypoplastic.
• Triangle-shaped lunula is common.
• Nail changes are typically associated with bony
abnormalities [absent or hypoplastic patellae, radial head
dysplasia and iliac crest exostoses (horns)].
• Nephropathy develops in approximately 40% of patients.
3- Pachyonychia Congenita (AD)
• Caused by mutation in genes encoding keratins 6a, 6b, 16, 17.
• These keratins are expressed in nail bed, HF, sebaceous gland and
palmoplantar epidermis.
• Present with:
1-nail signs:
• Pachyonychia (Onychauxis = Elephant nail = thick nail)
From the Greek pachys = thick, onyx = nail
• Increased transverse curvature, due to dense nail bed hyperkeratosis.
• Affected nails are extremely hard and difficult to trim
2-Painful palmoplantar keratoderma.
3-Multiple pilosebaceous cysts (e.g. steatocystoma multiplex).
4-Follicular keratoses.
5-Oral leukokeratosis.
4- Congenital Malalignment of the Great Toenails (Hallux)
• possibly caused by abnormality in ligament that connects matrix to the
periosteum of the distal phalanx.
• The longitudinal axis of Great Toenails is deviated laterally (with respect
to the longitudinal axis of the distal phalanx). This results in nail matrix
damage with Beau’s lines and onychomadesis.
• Nail plate is often thickened.
• Frequently bilateral and is the most common cause of
ingrown toenails in children and adolescents.
• Often improves spontaneously.

5- Epidermolysis Bullosa
• Nail changes are common in all forms of EB. Deviated axis
• Nail signs: Beau’s lines
• Pachyonychia lateral ingrown toenail
• Partial or total anonychia.
• Subungual/ periungual blister.
dermoscopy of longitudinal
melanonychia
Dermoscopy of Longitudinal Melanonychia

• First step we have to determine if the nail pigmentation is of melanocytic


or nonmelanocytic origin;
1-Nonmelanocytic origin:
• Features; pigment tends to be distributed diffusely (i.e homogenous).
No melanin granules in the nail plate under dermoscopy.
• Causes; Exogenous Pigment (e.g. henna and hair dye), subungual
hematoma or fungal infections (Trichophyton rubrum var. nigricans or
Scytalidium dimidiatum can cause nail pimentation).
2-Melanocytic origin:
• Features; melanin granules in the nail plate under dermoscopy.
• Causes; • Matrix melanocyte hyper/neoplasia: lentigo, nevi, melanoma.
• Matrix melanocyte activation: Physiological (Racial, Preg-),
Drugs (chemotherapy), Endocrine (Addison’s disease),
Inflammatory nail disorder (LP, OM), HIV Infection, Trauma,
Nonmelanocytic Nail Tumors (Bowen’s disease), ACTD (SLE).
• Second step, once the nail pigmentation has been identified to be of
melanocytic origin, we have to evaluate if the pigmentation is of (gray
color) or (brown to black color).
• Causes of (gray color) includes all causes of nail pigmentation of
melanocytic origin except for nevus and melanoma.
• Third step, in a lesion with brown to black color we need to determine if
the bands are regular pattern (nevus) or irregular pattern (melanoma);

Diagnostic algorithm
for nail pigmentations
of melanocytic origin
1. The background of the pigmented area of melanocytic origin
is brown homogenous color , with dots, and is due to melanin.

2. Regular pattern: The band comprises multiple longitudinal


brown to black lines that are regular in spacing, thickness,
coloration and parallelism throughout the whole lesion. This
is the typical aspect of nail matrix nevi.
Note ... Parallelism refers to parallel, unbroken line segments.

3.Irregular pattern: The band comprises multiple longitudinal


brown to black lines that are irregular in spacing, thickness
and coloration, with disruption of parallelism (nonparallel
interrupted lines). Irregular pattern is the predominant pattern
in nail matrix melanoma.
Note… Blood spots can also be found in association with an
irregular pattern (melanoma).
Nail Matrix Melanoma with Nail Matrix Melanocytic Nevi
Hutchingson’s sign, brown exhibiting brown background and
background and brown longitudinal brown longitudinal parallel lines
parallel lines irregular in coloration, regular in coloration, spacing and
spacing and thickness through the thickness through the whole lesion
whole lesion
Nail Matrix Melanocytic Nevi

• Can be acquired or congenital. Seen in children and young adults.


• Can be heavily pigmented and simulate melanoma.
• A pseudo-Hutchinson sign is very common.
• Histopathology shows nests of melanocytes at dermoepidermal junction.
• Junctional nevi are more frequently found than compound nevi. In
general, they occur more often on fingernails than on toenails.
• Dermoscopic features indicating nevi:
1. brown homogenous color background.
2. Regular pattern (spacing, thickness, coloration and parallelism).

Note… A history that points in a benign direction includes:


1. Congenital
2. Stable (without change over time)
3. A family history of similar nail pigmentation
4. Exposure to pigmentogenic medication (eg, acyclovir, minocyline)
5. History of trauma or inflammation
Dermoscopy of Nail Matrix
Nevi Showing

1.Homogeneous light and dark


brown background.

2.Uniform thick dark bands (red)

3.Uniform thin dark bands (white)

4.Hutchinson sign (yellow)


Nail melanoma

• The most frequent melanoma subtype in black and Asian populations.


• Most frequently involves the thumb of middle-aged individuals.
• 50% of patients report a history of trauma.
• In 25% of patients the tumor is amelanotic.
• Hutchinson’s sign is frequently observed.
• The prognosis of nail matrix melanoma is generally worse than for
melanoma in other sites.
• Dermoscopic features indicating Nail melanoma;
1. Brown homogenous color background, with dots.
2. Irregular pattern (spacing, thickness, coloration and parallelism).
3. Blood spots in association with criteria of melanocytic lesion.
4. Hutchinson’s sign
5. Micro-Hutchinson’s sign (Pigmentation of the cuticle invisible to the
naked eye).
Dermoscopy of Nail
Melanoma Showing

1.pigmented bands with irregular


spacing, thickness and coloration
(black)

2.disruption of parallelism (red)

3.Hutchinson sign (yellow)


Dermoscopy of Nail Melanoma
Showing

1.Pigmented bands with irregular


thickness (yellow and white)
2.Thin fine pigmented bands (red)
3.Disruption of parallelism (black)
4.Irregular dark dots and globules
(circles)
Dermoscopy of Nail
Melanoma Showing

1.Diffuse dark homogeneous


color (stars)
2.pigmented bands with
irregular spacing, thickness and
coloration (black)
3.disruption of parallelism
(yellow)
4.Nail plate destruction (white)
Dermoscopy of Nail Melanoma
Showing

1.Pigmented bands with irregular


thickness, color and spacing (white)
2.Disruption of parallelism (blue)
Dermoscopy of Nail Melanoma
Showing

1.pigmented bands with irregular


spacing and thickness
2.disruption of parallelism
3.Hutchinson sign
References
 Books;
• Bolognia Dermatology 3rd edition (2012) and 4th edition (2018).
• Clinical dermatology(Thomas P. Habif) sixth edition (2016).
• fitzpatrick’s synopsis of clinical dermatology 6th edition (2009).
• fitzpatrick’s dermatology in general medicine 7th edition (2008).
• Mcgraw-Hill Specialty Board Review Dermatology 2nd edition (2010).
• Review of Dermatology 1st Edition (2017).
• Illustrated Study Guide and Comprehensive Board Review 2nd Edition (2017).
• Nail Disorders 3rd Edition (2003).
• Atlas of dermoscopy 2nd edition (2012).
• Dermoscopy An Illustrated Self-Assessment Guide 1st Edition (2010).
• Handbook of Dermoscopy (2006).

 Journals
 Indian journals of dermatology, venereology and leprosy;
• Singal A, Khanna D. Onychomycosis: Diagnosis and management. Indian J Dermatol Venereol Leprol
2011;77:659-72.
• Archana Singal, Rahul Arora. Nail as a window of systemic diseases. Indian J Dermatol Venereol Leprol
2015 Mar-Apr; 6(2): 67–74.
 Journal of General Internal Medicine;
• Siwadon Pitukweerakul, Sree Pilla. Terry’s Nails and Lindsay’s Nails: Two Nail Abnormalities in Chronic
Systemic Diseases. J Gen Intern Med. 2016 Aug; 31(8): 970.
 Journal of turkish dermatology;
• Yalçın Tüzün, Özge Karakuş. Leukonychia. Turk Acad Dermatol 2009; 3 (1): 93101r.

 Meical web sites


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