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| TITLE: |
NAIL
DISEASE: SIGNS FOR THE INTERNIST |
| AUTHORS:
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SHARON LONGSHORE THORNTON, MD -- Department of Dermatology |
| |
KENNETH J. TOMECKI, MD -- Department
of Dermatology |
| PUBLISHED: |
JULY
8, 2005 |
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| The
primary function of the nail is protection. Nail concerns are common, but
the exact prevalence of nail disease is unknown. Nail disease occurs with
certain skin diseases (psoriasis, eczema), often follows external trauma,
may be an adverse effect of medication, and may occur with certain illnesses.
The most common cause of nail disease is infection, usually fungal (onychomycosis)
and less often bacterial. In many respects, the nails may indicate or reflect
medical illness. |
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| The
nail plate is a hard keratinized structure derived from keratinocytes
within the nail matrix, which begins proximal to the nail plate and extends
to the lunula, the white half-moon shaped portion often seen through
the nail plate. The proximal and distal nail matrices generate the
dorsal and ventral nail plate, respectively. The nail bed beneath
the nail plate extends from the lunula to the hyponychium, and the hyponychium
lies under the free edge of the nail plate. The proximal and lateral
nail folds comprise the paronychium. |
NAIL
ABNORMALITIES
AND SYSTEMIC DISEASE
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BEAU'S
LINES
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| Beau's
lines (Figure 1) are transverse depressions in the nail plate
that often follow local trauma. They may reflect poor nutritional status,
febrile illness, or a reaction to medication. Occasionally, they occur with
eczema, usually atopic or chronic hand eczema. Beau's lines are a temporary
cessation in nail growth and tend to appear about one month after the inciting
event. Beau's lines tend to resolve spontaneously, by moving distally. |
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ONYCHOLYSIS
|
| Onycholysis
(Figure 2) is the distal separation of the nail plate from
the underlying nail bed. Onycholysis often occurs with thyroid disease,
either hyperthyroidism or hypothyroidism, and commonly occurs with psoriasis
and eczema. It may occur with porphyria cutanea tarda, lichen planus, eczema,
and with some of the autoimmune blistering diseases; it may also indicate
an adverse reaction to medication, for example, tetracycline. Onycholysis
may be persistent. |
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ONYCHOMADESIS
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| Onychomadesis
is the proximal separation of the nail plate from the nail bed,
which typically results in shedding of the nail. Trauma is the usual cause.
Less common causes include poor nutritional status, febrile illness, or
drug sensitivity. The inciting event causes complete cessation of nail matrix
activity. Loss of the nail may or may not be permanent. |
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ONYCHORRHEXIS
|
| Onychorrhexis
(Figure 3) is the presence of longitudinal striations or ridging
of the nails. These striations may simply reflect advanced age, but they
do occur in some patients with rheumatoid arthritis, peripheral vascular
disease, lichen planus, or Darier's disease. Longitudinal red/white striations
invariably occur with Darier's disease, and V-shaped notching or nicking
of the free edges of the nails are also common. Onychorrhexis is usually
persistent. |
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KOILONYCHIA
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| The
spoon-shaped, concave nails of koilonychia (Figure 4) commonly
occur with iron-deficiency anemia and Plummer-Vinson syndrome, as a result
of thinning and softening of the nail plate. Spoon-shaped nails are a normal,
physiologic occurrence in children. Koilonychia tends to resolve, either
with treatment or with aging (children). |
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CLUBBING
|
| Clubbing
(Figure 5) is an increase in the transverse and longitudinal
nail curvature and fibrovascular hyperplasia of the soft tissue proximal
to the cuticle. With clubbing, the angle (Lovibond's angle) formed between
the dorsal distal phalanx and the nail plate is increased. Clubbing occurs
frequently with cardiopulmonary disease, for example aortic aneurysm and
bronchogenic carcinoma. The change is usually permanent. |
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SPLINTER
HEMORRHAGES
|
| Splinter
hemorrhages often follow trauma to the nail, but classically occur in patients
with bacterial endocarditis, less so in patients with rheumatoid arthritis,
trichinosis, and renal disease, esp. those on dialysis. Splinter hemorrhages
represent extravasation of blood from the vessels of the nail bed. They
may resolve, recur, or persist. |
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LEUKONYCHIA
|
| Leukonychia
(Figure 6) is a white discoloration of the nail secondary
to color change in the nail bed. Leukonychia includes Terry's nails, half-and-half
nails, and Muehrcke's lines. Terry's nails exhibit almost complete
discoloration except for the distal edge; they occur most commonly with
cirrhosis and congestive heart failure. The discoloration of half-and-half
nails affects the proximal half of the nail; they occur with chronic
renal failure. Muehrcke's lines (Figure 7) are narrow,
white transverse lines (usually two); they occur with hypoalbuminemia. Mee's
lines (Figure 8) resemble Muehrcke's lines, but the lines
are thicker and may be single or multiple. Mee's lines occur with arsenic
exposure. |
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YELLOW
NAIL SYNDROME
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| Yellow
nail syndrome (Figure 9) is a permanent cessation of nail
growth resulting in hard, thickened, curved, yellow nails, occasionally
with onycholysis. Yellow nails occur with lymphedema, chronic bronchitis,
or bronchiectasis. Yellow nails tend to be persistent. |
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PITTING
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| Pitting,
(Figure 10) or small punctate depressions in the nail plate,
most commonly occurs with psoriasis, less so with alopecia areata and eczema.
The pits represent abnormal keratinization in the nail matrix. In most cases,
the treatment of skin disease has little if any effect on the nail disease.
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TRACHYONYCHIA
|
| Trachyonychia
(Figures 11 and 12),
or twenty nail dystrophy, is a longitudinal ridging and roughness of the
nails. Though uncommon, it occurs with psoriasis, lichen planus, alopecia
areata, and eczema. Treatment of skin disease usually has no effect on the
ridging and roughness. |
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OIL
SPOTS
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| Oil
spots or "oil droplets"—orange-brown patches seen through
the nail plate—are characteristic of psoriasis. Psoriatic nail disease
is often associated with psoriatic arthropathy. |
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PTERYGIUM
|
| Pterygium
(Figure 13) is an adhesion of the proximal nail fold to the
proximal nail bed following inflammation that destroys the nail matrix.
Scar formation may occur, leading to partial loss of the nail plate. Pterygium
occurs almost exclusively with lichen planus. |
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PTERYGIUM
INVERSUS UNGUIS
|
| Pterytium
inversus unguis (Figure 14), or ventral pterygium, occurs
with connective tissue disease, especially progressive systemic scleroderma
and systemic lupus erythematosus. Pterygium inversus is an adhesion of the
distal nail bed to the ventral nail plate; soft tissue is attached firmly
to the underneath side of the nail plate. There is no satisfactory treatment
for pterygium. |
DRUG-INDUCED
NAIL ABNORMALITIES
|
|
Many drugs
can produce nail disease. Some characteristic abnormalities include the
following (Table 1).
| Table
1: |
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Systemic
Drugs Inducing Nail Changes
|
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Drug
|
Nail
Finding
|
| Antineoplastic
drugs |
Hyperpigmentation,
leukonychia, splinter hemorrhages, Beau's lines, onycholysis, onychomadesis |
| Arsenic
|
Mee's
lines |
| Azidothymidine |
Melanonychia |
| Beta-
blockers |
Thickening
and pitting, Beau's lines, ischemic changes |
| Chloroquine |
Blue-grey
to yellow pigmentation |
| Chlorpromazine |
Blue-purple
pigmentation |
| Indinavir |
Paronychia,
pyogenic granuloma |
| Minocycline |
Blue-grey
pigmentation |
| Psoralens/
PUVA |
Photo-onycholysis |
| Retinoids |
Brittleness,
pyogenic granuloma-like growths |
| Tetracycline |
Photo-onycholysis |
If the offending drug
is discontinued, the abnormality typically disappears, except for pyogenic
granuloma.
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ONYCHOMYCOSIS
|
| Onychomycosis
(Figure 15 and 16),
or tinea unguium, is a fungal or yeast infection of the nail, usually caused
by Tinea rubrum, T. mentagrophytes, or Candida albicans.
Onychomycosis is the most common nail disorder. Predisposing factors for
infection include heat, moisture, trauma, diabetes mellitus, and tinea pedis.
Affected nails are dystrophic and hyperkeratotic (thickened), often with
yellow-brown discoloration. Discomfort may occur. Treatment is warranted
for onychomycosis of the fingernails and whenever discomfort occurs. Treatment
can be symptomtatic, frequently with the aid of a podiatrist for toenail
onychomycosis. If warranted, systemic treatment involves the use of either
terbinafine or itraconazole. Success with either agent is less than 50%,
and recurrences are common. |
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PARONYCHIA
|
| Acute
paronychia (Figure 17) is an inflammation of the proximal
and lateral nail folds characterized by erythema, edema, and pain. Purulent
drainage with compression behind the cuticle may also occur. Trauma is often
the initial event with secondary infection with Staphylococcus aureus
or Streptococcus pyogenes. Treatment usually requires compresses
and an oral anti-staphylococcal antibiotic.
Chronic paronychia
is usually a non-infectious disease that follows irritant or allergic
contact dermatitis of the proximal nail fold. The cuticle is invariably
absent. Affected individuals often trim the cuticles aggressively and
meticulously and/or do "wet work' with their hands. Secondary infection
with Candida albicans is common. Treatment involves aeration, topical
corticosteroids, and perhaps an oral antifungal agent, for example, terbinafine,
for secondary infection.
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WARTS
|
| Warts
(Figure 18), or verruca vulgaris, are an infection of the
proximal and lateral nail folds caused by human papilloma virus (HPV), types
1, 2, and 4 are primarily responsible for periungual warts. Because of the
location, these warts are particularly difficult to treat, especially if
they extend subungually. Subungual warts may cause deformity or discoloration
of the nail plate. Affected patients are often 'nail biters.' |
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MUCOUS
CYST
|
| A
mucous or myxoid cyst (Figure 19) is a soft nodule at the
proximal nail fold, which may intermittently drain viscous fluid; less commonly,
it may occur subungually. The cyst, a collection of degenerative collagen,
may cause a longitudinal depression or groove in the nail plate from compression
of the nail matrix. Occasionally, it may connect to the underlying joint
space and is often associated with osteoarthritis of the distal interphalangeal
joints. Treatment is excision. |
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SUBUNQUAL
EXOSTOSIS
|
| Subunqual
exostosis is essentially a hard, painful, subungual tumor, most commonly
on the great toe. The exostosis typically occurs with trauma and often causes
elevation of the distal nail plate. Exostosis is an outgrowth of normal
bone. Treatment is symptomatic, for example, orthotics and analgesics, though
surgery may be necessary for some patients. |
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PERIUNGUAL
FIBROMAS
|
| Periungual
fibromas (Figure 20), or Köenen's tumors, are flesh-colored
to pink papules that originate from the nail bed and cause a longitudinal
depression in the nail plate. These fibromas may occur spontaneously, but
are often associated with tuberous sclerosis. Treatment is often unnecessary;
however, excision is curative. |
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PYOGENIC
GRANULOMA
|
| A
pyogenic granuloma (Figure 21) is a benign vascular tumor
that usually develops after trauma and occasionally with pregnancy and some
medication (Table 1). Characterized by rapid growth and a
blue-red color, the nodule/tumor bleeds easily with minimal trauma. Surgical
excision is the treatment of choice. |
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GLOMUS
TUMOR
|
| The
glomus tumor (Figure 22) is a benign vascular growth, arising
from glomus cells of the nail bed. The tumor is usually a red-blue macule
(patch) within the nail bed visible through the nail plate. Pain or pressure
and sensitivity to changes in temperature are common. Treatment, via excision,
is often necessary for symptomatic relief. |
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LONGITUDINAL
MELANONYCHIA
AND MELANOMA
|
| Longitudinal
melanonychia (Figure 23), brown-pigmented longitudinal streaks
of the nail, may be a normal variant in darker-skin individuals (Figure
24), or they may actually be nevi. Less commonly, they may represent
Addison's disease, acanthosis nigricans, Peutz-Jeghers syndrome, trauma,
subungual hemorrhage, and fungal infection, or even an underyling melanoma,
especially when they occur on the thumb (most common site for melanoma of
the nail unit). Black discoloration of the proximal nail fold at the base
of the pigmented streak (Hutchinson's sign) is an ominous sign for melanoma.
Longitudinal melanonychia in one nail without an obvious explanation warrants
a biopsy of the nail matrix. Melanoma of the nail unit has a poor prognosis. |
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SQUAMOUS
CELL CARCINOMA
|
| Squamous
cell carcinoma (SCC) is the most common malignancy of the nail unit (Figure
25). SCC is usually a verrucal (warty) plaque on the lateral nail
fold of the finger, and may resemble a wart unresponsive to traditional
therapy. SCC is associated with HPV-16 infection and less so with trauma
and radiation. SCC of the nail unit grows slowly and metastasis is rare.
|
GENETIC
NAIL ABNORMALITIES
|
| Many
genetic disorders have distinctive nail abnormalities. Nail patella syndrome
(Figure 26) exhibits triangular lunulae, in addition to renal
disease and skeletal abnormalities. Pachyonychia congenita, an ectodermal
dysplasia, exhibits marked subungual hyperkeratosis and increased transverse
curvature of the nails (pincer nails, Figure 27). Other genetic
disorders with nail findings include: hidrotic ectodermal dysplasia
(thickening, longitudinal striations, absence of nails); dyskeratosis
congenita (pterygium, absence of nails); epidermolysis bullosa
(absence of nails), and Darier's disease (V-shaped nicking, red and
white longitudinal striations, onychorrhexis, Figure 28). |
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SUMMARY
|
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This chapter outlines
the major causes of nail disease. An underlying systemic or skin disease
may be associated with nail dystrophy. In other cases, trauma, medications,
or nail unit tumors may be responsible. Definitive treatment is often
difficult; treatment for infections and tumors is usually straight-forward,
though many nail disorders simply require observation and reassurance.
Return
to Medicine Index
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- Drake LA, Dinehart
SM, Farmer EM, et al. Guidelines of care for nail disorders. J Am
Acad Dermatol. 1996;34:529-33.
- Piraccini BM,
Tosti A. Treatment of common nail disorders. Derm Clinics. 2000;18:339-48.
- Rich P. Nail
biopsy: indications and methods. Dermatol Surg. 2001;27:229-34.
- Rich P. Nail
disorders: Diagnosis and treatment of infectious, inflammatory, and
neoplastic nail conditions. Med Clin North Am. 1998;82:1171-83.
- Rogers M. Nail
manifestations of some important genetic disorders in children. Dermatologic
Therapy. 2002;15:111-20.
- Spencer JM. Malignant
tumors of the nail unit. Dermatologic Therapy. 2002;15:126-30.
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to diagnostic and treatment options of a specific patient's medical condition.
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Copyright
2005 The Cleveland Clinic Foundation
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