2018 Overview of Nail Disorders. UPTODATE

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Overview of nail disorders
Author: Phoebe Rich, MD

Section Editor: Erik Stratman, MD
Deputy Editor: Rosamaria Corona, MD, DSc
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Dec 2017. | This topic last updated: Aug 29, 2017.
INTRODUCTION — The human nail shields the distal digit from harm, assists in the picking-up of small objects, improves fine touch, and enhances the aesthetic
appearance of the hand [1]. Aesthetically displeasing nails and nail-associated symptoms such as pain or throbbing are common factors that contribute to a
patient's decision to seek medical attention.
This topic will discuss the clinical features, diagnosis, and treatment of common acquired nail disorders. Nail dermoscopy, nail surgery, nail biopsy techniques,
and hereditary nail disorders are discussed separately.
● (See "Dermoscopy of nail pigmentations".)
● (See "Nail avulsion and chemical matricectomy".)
● (See "Principles and overview of nail surgery".)
● (See "Nail biopsy: Indications and techniques".)
● (See "Nail-patella syndrome".)
● (See "The genodermatoses", section on 'Pachyonychia congenita'.)
ANATOMY AND PHYSIOLOGY OF THE NAIL UNIT — The nail unit is composed of the nail matrix, the nail bed, the proximal and lateral nail folds, and the
hyponychium (picture 1 and figure 1) [2,3].
The nail matrix is the germinative epithelium from which nail matrix keratinocytes differentiate to ultimately form the nail plate [2-4]. Most of the nail matrix is
hidden beneath the proximal nail fold, but the distal third is sometimes visible through the proximal portion of the nail plate as a half-moon-shaped structure
called the lunula (picture 1) [2-4]. Maturation and differentiation of the nail matrix occurs along a diagonal axis oriented distally (figure 2). Thus, the keratinization
of the distal matrix cells forms the ventral portion of the nail plate, whereas the keratinization of the proximal matrix cells forms the dorsal portion of the nail plate
[2-5]. Nail plate abnormalities typically result from pathologic processes involving the nail matrix or space-occupying lesions involving the overlying nail fold.
On average, fingernail regrowth takes approximately 6 months and toenail regrowth about 12 months and up to 18 months for the great toenail [6]. Nail growth
slows with advancing age and vascular disease and can be partially or completely interrupted by systemic illness, trauma, or medications (eg, antimitotic drugs)
[6].
The nail bed dermis lies directly beneath the nail plate and is believed to contribute some epithelial cells to the ventral surface of the nail, allowing the nail to
grow continuously while adhering to the nail bed [2]. For this reason, nail bed surgery may be complicated by mild onycholysis and rarely by permanent nail
dystrophy [2]. (See 'Onycholysis' below.)
The dermoepithelial interface of the nail bed is composed of longitudinal rete ridges and papillary body ridges. Each papillary body ridge contains three to five
longitudinally oriented capillaries, explaining the longitudinal orientation of splinter hemorrhages [2-4]. (See 'Splinter hemorrhages' below.)
The proximal and lateral nail folds are collectively known as the paronychium [2,3]. The nail folds serve to protect the nail plate and direct nail plate growth in the
correct orientation. The hyponychium, located at the distal free edge of the nail plate just proximal to the distal groove, is contiguous with the volar skin. The
hyponychium functions to seal and protect the distal nail unit from the environment. Disruption of the hyponychium may result in onycholysis and allow the
penetration of pathogens that are unable to digest keratin. The paronychium plus the hyponychium and nail bed is called the perionychium.
The surgical anatomy and blood and nerve supply of the nail apparatus are discussed separately. (See "Principles and overview of nail surgery", section on
'Surgical anatomy of the nail unit'.)
NAIL EVALUATION
History — Important aspects of the history in a patient with a complaint of nail problems include the time of onset of the disease, occupation, hobbies, topical
substance exposure, medical history, medication history, and family history of nail disorders [7,8]. Some clinicians ask their patients to complete a detailed nail
questionnaire before the visit (table 1) [7]. (See 'Skin diseases with nail involvement' below.)
Patients should be asked to bring all nail care products, including cosmetics and instruments, to the appointment. Nail polish, lacquer, or other topical
substances should be removed before the appointment to allow the examination of all nails.
Nail examination — All nails should be examined under adequate lighting without glare and with magnification [7,8]. Natural sunlight is preferred over artificial
light. Transillumination of the distal phalanx by using a penlight may help in localizing an abnormality. To detect subtle changes of the nail plate surface, alcohol
or acetone can be used to cleanse the surface, remove any adherent substances, and reduce glare.
Digits should be relaxed and not pressed against a surface during the examination, since any alteration in the hemodynamics of the nail bed can change the nail
appearance. Each component of the nail apparatus, including the nail plate, nail bed, proximal and lateral nail folds, and hyponychium, should be evaluated for
any abnormalities.
The nail plate is assessed for discoloration, onycholysis, and changes in thickness and surface texture, including pitting, ridging, and longitudinal and transverse
grooving. (See 'Signs of nail disease' below.)
The nail bed, nail folds, and hyponychium should be assessed for discoloration, erythema, growths, scale, cuticle attachment, and vascular abnormalities. If a
subtle change in pigmentation is found, squeezing the tip of the digit may be helpful in identifying vascular lesions, which become less visible with the application
of pressure.
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The pattern of nail involvement must be identified. In cases where only one or a few nails are affected, infection, trauma, tumor, or circulation disturbances are
possible etiologic factors. However, dermatoses such as lichen planus or psoriasis may affect only one nail.
The skin should also be examined for any concurrent findings [7,8]. Appropriate referral is indicated for patients with nail signs associated with systemic
diseases. (See 'Nail signs of systemic diseases' below.)
SIGNS OF NAIL DISEASE — Nail dystrophy is a general term referring to altered or disturbed nail growth due to a wide range of conditions that may affect the
nail matrix, including trauma, infection, inflammation, tumors, inherited skin and/or nail diseases, and systemic diseases. Depending upon the nature and
duration of the insult to the nail matrix, nail dystrophy can present with a variety of changes involving the nail plate, as discussed below.
Transverse grooves (Beau lines and onychomadesis) — Beau lines result from a temporary arrest of proximal nail matrix proliferation and appear as
transverse grooves that move distally with nail growth (picture 2A-B). The detachment of the nail plate from the proximal nail fold by a whole-thickness sulcus is
called onychomadesis and results from a more severe or prolonged insult to the nail matrix (picture 3A-B). The time of the insult leading to transverse grooving
can be dated by measuring the distance of the groove from the proximal nail fold (approximately one month for every mm from the proximal nail fold).
Causes of transverse grooving and onychomadesis include [9-12]:
● Local trauma (eg, manicure, onychotillomania [habit tic deformity], ill-fitting footwear)
● Local cutaneous disease (eg, dermatitis, paronychia)
● Drugs (eg, retinoids, chemotherapy agents)
● Viral infections (eg, hand, foot, and mouth disease) (see "Hand, foot, and mouth disease and herpangina", section on 'Coxsackievirus A6 HFMD' and "Hand,
foot, and mouth disease and herpangina")
● Pemphigus
● Kawasaki disease (see "Kawasaki disease: Clinical features and diagnosis", section on 'Extremity changes')
Rare idiopathic or familial cases of onychomadesis have also been reported [13,14].
Longitudinal grooves — Focal compression of the nail matrix from tumors located in the proximal nail fold or nail bed may produce a longitudinal groove
(picture 38D). Median nail dystrophy, also called median canaliform dystrophy of Heller, is a distinctive form of longitudinal groove characterized by a
paramedian canal or split in the nail plate of one or more nails (picture 2C) [15]. Small cracks or fissures that extend laterally from the central canal or split
toward the nail edge give the appearance of an inverted fir tree. The condition is usually symmetric and most often affects the thumbs. The condition results from
a temporary defect of matrix function of unknown etiology. Trauma, including habitual nail picking, has been implicated as a causative factor in some cases.
Longitudinal grooves must be distinguished from physiologic furrows and ridges, which are accentuated in lichen planus, rheumatoid arthritis, peripheral vascular
disease, older age, and Darier disease.
Onychorrhexis — Onychorrhexis occurs when superficial grooves in the nail plate lead to a distal split (picture 4).
Onychoschizia — Onychoschizia is characterized by lamellar splitting of the free edge of the nail, due to impairment of intercellular adhesive factors of the nail
plate (picture 5).
Pitting — Nail pitting results from focal areas of abnormal keratinization of the nail matrix that produce foci of parakeratotic cells in the dorsal nail plate as it
grows beyond the cuticle (picture 6A-B).
Trachyonychia — Trachyonychia is a nail plate abnormality characterized by roughness, excessive longitudinal ridging, pitting, thickening of the cuticle, and
distal brittleness (picture 6C). Trachyonychia results from multiple foci of defective keratinization of the proximal nail matrix.
Trachyonychia involving most or all nails is also called "twenty nail dystrophy" (picture 7). It occurs predominantly in children and may be idiopathic or associated
with other skin diseases, most commonly psoriasis, lichen planus, alopecia areata, or atopic eczema [16]. Histology shows a psoriasiform/lichenoid infiltrate or a
spongiotic infiltrate [17]. In approximately 50 to 80 percent of children, the disease resolves spontaneously over several years [18,19].
Leukonychia — Leukonychia results from defective keratinization of the distal matrix with persistence of parakeratotic cells in the ventral nail plate. The nail has
opaque white patches or striae, which often disappear before reaching the distal edge of the nail (picture 8). Punctate leukonychia is due to microtrauma and is
typically seen in the fingernails of children. In the rare inherited total leukonychia, a nonsyndromic, isolated nail disorder due to mutation in the phospholipase C,
delta-1 (PLCD1) gene, all nails are milky porcelain white (picture 9) [20].
Transverse leukonychia (Mees' lines) — Transverse leukonychia, also called leukonychia striata or Mees' lines (picture 10), is characterized by a white
discoloration of the nail plate in bands or striae that run parallel to the nail base. It may be caused by repeated trauma, infections, drugs, or systemic disease
[21-25].
Longitudinal melanonychia — Longitudinal melanonychia is a banded brown to black pigmentation of the nail due to the presence of melanin in the nail plate.
Longitudinal melanonychia may appear as a single band involving one nail or as multiple bands affecting several nails. The latter form is most commonly seen in
dark-skinned individuals (picture 11).
Hutchinson sign is the periungual spread of pigment into the proximal or lateral nail folds (picture 12). It may increase the suspicion of ungual melanoma
[2,26,27]. (See 'Melanoma' below.)
Hutchinson sign also may be seen in about one-third of cases of nail lentigines or nevi (picture 13) and in other benign conditions such as Laugier-Hunziker
syndrome (mucosal pigmentation), ethnic pigmentation, or the use of certain medications [28,29]. In these circumstances, it is referred to as pseudo-Hutchinson
sign (table 2 and picture 13) [30,31].
On histologic examination, longitudinal melanonychia may result from activation or hyperplasia of nail matrix melanocytes [26,30,32].
Melanocytic activation — Melanocytic activation (also termed melanocytic stimulation) causes an increased melanic pigmentation of the nail matrix
epithelium without a concurrent increase in the number of melanocytes (picture 14A-C) [30].
Approximately 70 percent of cases of longitudinal melanonychia in adults result from melanocytic activation [30,33]. Physiologic (ethnic or pregnancy-related),
traumatic, dermatologic, systemic, or iatrogenic factors may be the underlying cause (table 3A-B) [30,34]. Melanonychia caused by melanocyte activation often
involves several nails and occurs more frequently in patients with darker skin phototypes (ethnic melanonychia) (picture 14A).
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On dermoscopy, melanonychia due to melanocytic activation appears as a gray background with thin gray regular parallel lines. In melanocyte activation caused
by chronic trauma, tiny dark red to brown spots corresponding to extravasation of blood may also be seen [32]. (See "Dermoscopy of nail pigmentations", section
on 'Benign lesions'.)
Melanocytic hyperplasia — Longitudinal melanonychia caused by a proliferation of melanocytes within the nail matrix or nail bed epithelium is a diagnostic
challenge for the clinician and the pathologist. Differentiating a benign pigmented lesion of the nail (lentigo or nevus) from early nail melanoma may be extremely
difficult [32].
Lentigines and nevi appear as tan to brown bands <3 to 5 mm in width (picture 15A-B) [30,35]. In about one-third of cases, there is associated periungual
pigmentation (pseudo-Hutchinson sign) (picture 13). Other conditions associated with a pseudo-Hutchinson sign include ethnic melanonychia, Laugier-Hunziker
syndrome (mucosal hyperpigmentation), or congenital nevus (table 2).
Nail lentigines are seen more often than nevi in adults, whereas nevi are the most common cause of melanonychia in children [36,37]. Nail nevi may be
congenital or acquired and most are junctional [26,30,33,36,38]. They usually occur on the fingernails, with the thumbnail being most commonly affected.
On dermoscopy, lentigines generally appear as homogeneous, longitudinal thin gray or brown lines on a gray or light-brown background; in contrast, nail matrix
nevi appear as a band of regular lines of light brown to black color on a brown background (picture 16A-C) [39,40]. (See "Dermoscopy of nail pigmentations",
section on 'Benign lesions'.)
On histology, lentigines consist of a slight to moderate increase in the number of single matrical melanocytes without evidence of confluence (nests) [38]. In
contrast, nests of melanocytes, sometimes large and hyperchromatic, are always present in nail matrix nevi.
Malignant melanocytic hyperplasia is characterized by an increased number of atypical matrix melanocytes with large, hyperchromatic, pleomorphic nuclei,
prominent nucleoli, increased mitoses, and long branching dendrites [26]. Malignant melanocytic hyperplasia manifests clinically as in situ or invasive nail
melanoma (picture 17). The clinical and dermoscopic features of nail melanoma are discussed below. (See 'Melanoma' below.)
Approach to differential diagnosis — The approach to the clinical differential diagnosis and management of longitudinal melanonychia involving one or
multiple nails is illustrated in the algorithm (algorithm 1). The dermoscopic evaluation and differential diagnosis of longitudinal melanonychia are discussed
separately (algorithm 2). (See "Dermoscopy of nail pigmentations", section on 'Differential diagnosis of nail pigmentations'.)
When to biopsy — The clinical differentiation of a benign longitudinal melanonychia from early nail melanoma may be extremely difficult. Thus, a
diagnostic biopsy is often necessary to rule out melanoma. The clinical features of longitudinal melanonychia that may suggest early nail melanoma and warrant
a biopsy of the nail matrix are discussed below. (See 'Melanoma' below.)
Monitoring — There is no consensus on the frequency and modalities of follow-up for pigmented nail bands. A "wait and see" approach may be
appropriate in adults and children when clinical and dermoscopic features indicate a low risk of melanoma [41]. Some experts recommend clinical and
dermoscopic examination every six months for lesions that have subtle irregular features that do not require immediate biopsy [32,40]. However, melanonychia
should be totally excised when worrisome features (eg, wide band, presence of the Hutchinson sign, or irregular dermoscopic features) are noted.
Longitudinal erythronychia — Longitudinal erythronychia is a pink to red longitudinal streak in the nail plate that corresponds to a band of thinned, more
transparent nail plate [42,43]. Longitudinal erythronychia is caused by a focal reduction of function in the distal matrix. The nail bed underlying the band is less
compressed than the adjacent portions so that blood pools and the engorged tissue swells and fills the ventral groove of the nail plate.
Splinter hemorrhages are commonly found in longitudinal erythronychia. The thinned portion of nail plate extends to the distal free margin and is easily
traumatized during the daily activities resulting in splitting and v-shaped chipping. Reactive hyperkeratosis of the underlying hyponychium may also occur.
Localized (monodactylous) longitudinal erythronychia involves one nail and may be associated with benign or malignant nail tumors, including (picture 18A-B)
[42-46]:
● Onychopapilloma (picture 19A-D)
● Warty dyskeratoma
● Glomus tumor (see 'Glomus tumor' below)
● Squamous cell carcinoma (SCC), including SCC in situ (Bowen disease) (see 'Squamous cell carcinoma' below)
● Amelanotic melanoma
Longitudinal erythronychia involving multiple nails (polydactylous longitudinal erythronychia) is most often associated with lichen planus or Darier disease, but
systemic amyloidosis, hemiplegia, graft versus host disease, acantholytic epidermolysis bullosa, and idiopathic cases have also been reported [46-50]. (See
"Darier disease", section on 'Nail changes'.)
The necessity of biopsy depends upon the number of involved digits and associated conditions or symptoms. Biopsy is necessary for the diagnosis of
monodactylous longitudinal erythronychia. For patients with polydactylous longitudinal erythronychia and a known associated condition, biopsy is not generally
required for diagnosis. Patients with polydactylous longitudinal erythronychia without associated cutaneous or systemic symptoms are generally observed over
time for development of cutaneous or systemic symptoms of associated conditions [43].
Biopsy indications and techniques for longitudinal erythronychia are discussed separately. (See "Principles and overview of nail surgery", section on
'Longitudinal erythronychia'.)
Splinter hemorrhages — Splinter hemorrhages appear as red to black small thin longitudinal lines under the nail plate. In general, they are more commonly
located in the distal nail plate and represent rupture of the longitudinally oriented nail bed capillaries (picture 20A-B). The most common causes of splinter
hemorrhages are trauma and nail psoriasis [51]. However, splinter hemorrhages may be associated with other chronic dermatoses involving the nail (eg, lichen
planus, Darier disease) (picture 21) and, rarely, with systemic illness such as infective endocarditis (picture 22), connective tissue disease, antiphospholipid
syndrome, chronic renal failure, and trichinellosis [51-56]. (See "Lichen planus", section on 'Nail lichen planus' and "Darier disease", section on 'Nail changes'
and "Clinical manifestations and evaluation of adults with suspected native valve endocarditis".)
Splinter hemorrhages associated with systemic diseases are often proximal in the nail, whereas those due to trauma usually are distal. However, all splinter
hemorrhages grow out distally (picture 22).
Onycholysis — Onycholysis is defined as distal or distolateral separation of the nail plate from the underlying nail bed and/or lateral supporting structures such
as the hyponychium and lateral nail folds (picture 20B) [57,58]. The onycholytic portion of the nail plate appears white due to air beneath the nail plate.
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Onycholysis may be associated with many conditions, including trauma, wet work, psoriasis, lichen planus, medications, onychomycosis, allergic or irritant
contact dermatitis, or yellow nail syndrome. For cases of chronic unexplained onycholysis, subungual squamous cell carcinoma should be included in the
differential diagnosis [58].
Onycholysis is a predisposing condition for secondary subungual infections from dermatophytes (eg, Trichophyton rubrum), yeasts (eg, Candida albicans), or
bacteria (including Pseudomonas aeruginosa and Staphylococcus aureus). If infection is suspected, cultures should be obtained and appropriate antifungal or
antibacterial treatment started.
The management of onycholysis involves the identification and treatment of the underlying condition. General nail care measures for onycholysis include [57,58]:
● Keeping nails trimmed short
● Avoiding trauma
● Avoiding contact irritants
● Keeping nails dry (avoiding wet work)
● Avoiding all nail cosmetics
● Protecting hands from cold or windy weather
Retronychia — Retronychia is a form of incomplete nail shedding that leads to the embedding of the proximal nail plate into the proximal nail fold with
subsequent inflammation (picture 23) [59-61]. It occurs predominantly in young adults with a female predominance, but has been reported also in children
[62,63]. In most cases, the great toes are affected, likely due to repetitive minor trauma to the free nail margin that leads to incomplete separation of the proximal
nail plate from the matrix. The new nail growing from the matrix pushes the old one upward, causing inflammation of the proximal nail fold and interrupting the
longitudinal growth of the nail plate. Simple avulsion is usually helpful, but the condition can recur in the setting of chronic distal onycholysis and continued minor
trauma/pressure on the distal nail plate.
Subungual hyperkeratosis — Subungual hyperkeratosis is due to an abnormal keratinization of the distal nail bed and hyponychium with accumulation of
scales under the distal nail plate (picture 20D). The most common causes include psoriasis, onychomycosis, trauma, and eczema.
Onychogryphosis — Onychogryphosis, also called "ram's horn nail," is an acquired nail disorder usually affecting the great toenail, which appears thickened,
yellow-brown in color, increased in length, and distorted (picture 24). It is common in older adult and neglected individuals [64,65].
INFECTIONS
Fungal infection — Onychomycosis, a fungal infection of the nail unit, is characterized by nail discoloration, thickening, and deformity (picture 25A-E) [66]. The
clinical presentation, diagnosis, and treatment of onychomycosis are discussed separately. (See "Onychomycosis: Epidemiology, clinical features, and
diagnosis".)
Bacterial infection
Acute paronychia — Acute paronychia is the most common infection of the hand and typically results from local injuries to the nail fold (picture 26A-B) [67].
It may also occur as a complication of chronic paronychia. Most acute nail unit infections are caused by S. aureus. Other bacteria, including Streptococcus or P.
aeruginosa, may cause acute paronychia. (See "Paronychia".)
Blistering distal dactylitis — Blistering distal dactylitis is a localized infection involving the volar pad of the distal phalanx of the digits. It occurs in children
and adults. Tense, nontender oval bullae filled with thin, seropurulent fluid usually lie over the anterior fat pad and may extend dorsally to involve the proximal or
lateral nail fold (picture 27A-C). Cultures from the blister fluid most commonly grow group A beta-hemolytic Streptococcus and less often S. aureus [68,69].
Felon (pulp space infection) — Felon is an acute infection of the fingertip pulp space usually involving the thumb and index finger (picture 28). Abscess
forms in the small compartments of the fingertip pulp separated by vertical fibrous septa (figure 3). Penetrating trauma is the most common cause of felon, but it
can also be a complication of untreated paronychia. (See "Overview of hand infections", section on 'Pulp space infections'.)
Green nail syndrome — P. aeruginosa, a water-borne bacterium, may secondarily infect injured or onycholytic nails [70]. The infection is characterized by a
blue-greenish color of the nail plate due to the deposition of pyocyanin, a blue-green pigment produced by this bacterium (picture 29). The pyocyanin
pigmentation can persist for months despite adequate treatment. (See "Pseudomonas aeruginosa skin and soft tissue infections", section on 'Green nail
syndrome'.)
Viral infection
Warts — Human papilloma virus infection is the most common viral infection of the nail. Warts usually occur in the nail fold and, less frequently, in the nail bed
(picture 30). Warts involving the proximal nail fold result in longitudinal ridging and nail plate dystrophy. Nail bed warts may cause onycholysis. Periungual and
subungual warts are especially resistant to treatment, and overzealous treatment in the area of the nail matrix can cause permanent nail dystrophy [70]. (See
"Cutaneous warts (common, plantar, and flat warts)".)
Herpetic whitlow — Herpetic whitlow is a herpes simplex virus (HSV) infection of the hand that involves the skin or the periungual area of a finger (picture
31). The infection is acquired by direct inoculation of the virus following a minor local trauma [67,71]. Herpetic whitlow typically occurs in children who suck their
finger during a primary herpetic gingivostomatitis, but is also an occupational hazard for medical and dental personnel. (See "Overview of hand infections",
section on 'Herpetic whitlow'.)
BENIGN TUMORS
Fibroma — Nail fibromas are benign tumors of the connective tissue that most commonly originate in the nail matrix. However, they can also arise in the nail
bed or in the proximal nail fold and extend to the nail plate surface [72-74]. There are several types of nail fibromas, including:
● Acquired periungual fibrokeratoma – Acquired periungual fibrokeratoma is an acquired tumor presenting as a small, asymptomatic fleshy growth with a
keratotic distal tip (picture 32C) [75]. It usually arises following local trauma.
● Dermatofibroma – A dermatofibroma is a flesh-colored, pea-shaped growth that usually develops spontaneously (picture 32A-B).
● Koenen tumor (periungual fibroma) – Koenen tumors are periungual or subungual fibromas that develop in approximately 50 percent of patients with
tuberous sclerosis during childhood or adolescence. Koenen tumors occur more commonly on the toenails than on the fingernails. They present as
erythematous, polypoid, digitated growths, are often multiple, and may produce a longitudinal groove in the nail plate due to matrix compression (picture
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32D). (See "Tuberous sclerosis complex: Genetics, clinical features, and diagnosis", section on 'Dermatologic features'.)
Onychomatricoma — Onychomatricoma is a rare benign fibroepithelial tumor that originates from the nail matrix. Clinical manifestations of onychomatricoma
include (picture 33) [76,77]:
● Yellow longitudinal bands of variable width
● Splinter hemorrhages of the proximal portion of the nail plate
● Prominent longitudinal ridging associated with woodworm-like cavities
● Increased transverse curvature of the nail plate
Less frequent presentations of onychomatricoma include pincer nail deformity (picture 34), cutaneous horn, melanonychia, nail bleeding, or pterygium (the
extension and adherence of the proximal nail fold to the nail bed secondary to scarring of the nail matrix) (picture 35) [78-86]. Nail plate avulsion in
onychomatricoma reveals finger-like projections originating from a villous tumor of the nail matrix.
It has been suggested that a diagnosis of onychomatricoma can be made by histologic examination of a distal clipping of the free edge of the nail plate [87]. The
specimen is submitted for routine hematoxylin and eosin staining and sectioned in the transverse plane. The finding of increased nail thickness with cavitations
filled with serous material and surrounded by a layer of epithelium suggests the diagnosis of onychomatricoma.
Onychomatricoma may mimic subungual fibroma, fibrokeratoma, or squamous cell carcinoma (including squamous cell carcinoma in situ [Bowen disease])
[83,84]. (See "Clinical features and diagnosis of cutaneous squamous cell carcinoma (SCC)", section on 'SCC in situ (Bowen's disease)'.)
The surgical removal of onychomatricoma is discussed separately. (See "Principles and overview of nail surgery", section on 'Onychomatricoma'.)
Digital myxoid cyst or myxoid pseudocyst — A digital myxoid cyst (DMC) or myxoid pseudocyst typically presents as a translucent nodule on the dorsum of
the digit between the distal interphalangeal joint and the proximal nail fold (picture 36A) [88]. DMCs are most frequently located on the radial fingers [72].
DMCs result from mucoid degeneration of the connective tissue and/or joint fluid leaking from an osteoarthritic distal interphalangeal joint by way of a
communicating canal. Although DMC is commonly referred to as a cyst, on histologic examination the collection of mucinous fluid is not surrounded by an
epithelial lining. Thus, a more appropriate term is myxoid pseudocyst.
A DMC located distally in the proximal nail fold may exert pressure on the underlying nail matrix, resulting in a longitudinally-oriented, depressed groove in the
nail plate (picture 36B) (see 'Longitudinal grooves' above). Occasionally, a DMC may discharge its mucinous content and reduce the pressure on the nail matrix,
resulting in an irregular longitudinal depression in the nail plate [88]. Subungual DMC variants may present as subungual tumors associated with a red lunula,
transverse nail plate overcurvature, and ingrowing nail plate [72,89].
High-resolution ultrasonography or magnetic resonance imaging (MRI) may confirm the diagnosis in cases that are clinically ambiguous [72]. The
communicating canal between the DMC and the distal interphalangeal joint are visible on MRI in over 80 percent of cases [90].
A wide range of therapies are used to treat DMCs, including digital compression, cryotherapy, sclerosant injection, or surgical excision. Spontaneous discharge
of a DMC is a risk factor for septic osteoarthritis of the distal interphalangeal joint and is an indication for DMC removal [72]. The surgical treatment of digital
myxoid cysts is discussed separately. (See "Principles and overview of nail surgery", section on 'Digital myxoid cyst'.)
Pyogenic granuloma — A pyogenic granuloma is a benign vascular tumor presenting as a rapidly evolving sessile or polypoid nodule composed of red, friable
granulation tissue that bleeds easily (picture 37) [91]. Pyogenic granuloma may involve the nail fold or be subungual and penetrate the nail plate. Subungual
pyogenic granulomas arise from the nail matrix and produce a localized deformity of the nail plate. Local trauma or medications have been associated with the
development of periungual or subungual pyogenic granuloma. (See "Pyogenic granuloma (Lobular capillary hemangioma)".)
Glomus tumor — Glomus tumor is a rare benign tumor composed of cells resembling the smooth cells of the normal glomus body [92]. It presents as a red to
purple or blue lesion under the nail plate (picture 38A-D) [92-94]. Symptoms include paroxysmal pain, cold sensitivity, and tenderness. The diagnosis is
suspected based upon clinical appearance and history of paroxysmal pain and cold sensitivity [94,95]. (See "Overview of benign lesions of the skin", section on
'Glomus tumor'.)
Imaging studies with MRI or high-variable frequency ultrasonography may be helpful for confirming the clinical suspicion and assessing the size and location of
the tumor preoperatively [96-98]. The treatment of glomus tumors is surgical. Histologic examination of the excised tumor is necessary to confirm the diagnosis.
The surgical treatment of glomus tumor is discussed separately. (See "Principles and overview of nail surgery", section on 'Glomus tumors'.)
Subungual exostosis — Subungual exostosis is a benign osteocartilaginous tumor that most commonly occurs on the dorsomedial aspect of the tip of the great
toe in adolescents or young adults [99]. In its early stage, the tumor typically presents as a firm, porcelain-white, telangiectatic nodule with an overlying collarette
of scale that extends from beneath the distal nail causing reddish onycholysis. With time, the tumor becomes hyperkeratotic (picture 39). Pain is variable and
can be absent in some patients. Plain radiographs may confirm the clinical diagnosis [99]. Treatment is surgical excision.
The surgical treatment of subungual exostosis is discussed separately. (See "Principles and overview of nail surgery", section on 'Subungual exostosis'.)
Onychopapilloma — Onychopapilloma is a rare benign tumor of the nail bed or distal part of the nail matrix that most commonly presents as a longitudinal
erythronychia (picture 19A-D) [100,101]. (See 'Longitudinal erythronychia' above.)
Less common presentations include leukonychia, melanonychia, or splinter hemorrhages (picture 19D) [101-104]. A fissure of the distal nail plate associated
with a V-shaped notch can be seen in some patients. The lesion is usually asymptomatic.
On dermoscopic examination, onychopapilloma shows a homogeneous brown or gray band in the nail plate originating in the lunula, with splinter hemorrhages
and a characteristic gray keratotic mass at the free distal edge (picture 40). Characteristic histopathologic features include papillomatous acanthosis of the distal
matrix and nail bed; in the distal portion of the nail bed there are layers of hyperkeratosis containing fusiform cells with eosinophilic cytoplasm resembling those
of the keratogenous zone of the matrix (matrix metaplasia of the nail bed) [100].
The differential diagnosis of onychopapilloma includes other causes of localized (monodactylous) longitudinal erythronychia, such as glomus tumor, squamous
cell carcinoma, or amelanotic melanoma. (See 'Glomus tumor' above and 'Malignant tumors' below.)
Treatment of onychopapilloma is surgical excision.
MALIGNANT TUMORS
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Squamous cell carcinoma — Squamous cell carcinoma (SCC), including squamous cell carcinoma in situ (Bowen disease), is the most common malignant
tumor of the nail occurring most often on the fingernails, within the nail bed or lateral nail grooves. Nail SCC usually affects older adults, predominantly males
[105]. Trauma, radiation exposure, smoking, and infection with human papilloma virus types 16 and 18 are predisposing factors for SCC development. (See
"Epidemiology and risk factors for cutaneous squamous cell carcinoma", section on 'Risk factors'.)
The clinical features of periungual or subungual SCC are generally nonspecific, often causing prolonged delay in the diagnosis. SCC of the nail bed or lateral
nail groove or nail fold may present as hyperkeratosis, persistent onycholysis, longitudinal erythronychia, verruca, paronychia, nail plate dystrophy, or a
subungual mass (picture 18B-E). SCC should be suspected when a verrucous or keratotic lesion of the lateral nail groove or fold is persistent or recurs after
cryotherapy or other treatment for common warts.
Keratoacanthoma, a rare clinical variant of SCC, typically presents with painful onycholysis, digital erythema and swelling, or painful paronychia [91].
Keratoacanthomas usually grow rapidly, and osteolysis of the bone is commonly observed on radiography. Patients with incontinentia pigmenti may develop
multiple subungual keratoacanthomas at a young age [106,107].
A lesion suspicious for SCC or keratoacanthoma should be biopsied using the punch, excisional, or tangential (shave) excision techniques. (See "Nail biopsy:
Indications and techniques".)
Mohs micrographic surgery is the preferred treatment for SCC without bone involvement [108,109]. Alternative treatments include wide surgical excision or digit
amputation if the bone is involved. The recurrence rate is low after wide excision or amputation [110]. (See "Mohs surgery".)
Melanoma — Melanoma of the nail apparatus is a form of acral melanoma that arises from the nail matrix. Nail melanoma is rare. It accounts for 1 to 3 percent
of melanomas occurring in white populations and 15 to 30 percent of melanomas occurring in dark-skinned populations [111].
In two-thirds of cases, nail melanoma presents as a brown to black longitudinal stripe in the nail plate, known as longitudinal melanonychia (picture 41A-B) [26].
In one third of cases, nail melanoma is amelanotic and presents as a nail bed mass or nail plate abnormality (picture 42A-B). (See 'Longitudinal melanonychia'
above.)
Many patients with nail melanoma are initially misdiagnosed as having a benign condition and the diagnosis of melanoma may be delayed [30]. Because of the
diagnostic delay, subungual melanoma has a poor prognosis with reported 5- and 10-year survival rates of 30 and 13 percent, respectively.
Early nail melanoma may first appear as narrow longitudinal melanonychia with subtle variegation of color [40]. Additional clinical features that may suggest
early nail melanoma and warrant a biopsy of the nail matrix in patients with longitudinal melanonychia include (algorithm 1) [32,38,112]:
● Melanonychia that develops during adulthood, involves a single digit (in particular the thumb, index finger, or great toe), and enlarges rapidly
● Longitudinal melanonychia >3 mm in width (picture 12) with variegated pigmentation or proximal widening (triangular shape) (picture 41A)
● Preexisting longitudinal melanonychia that becomes darker or wider or demonstrates blurred lateral borders
● Longitudinal melanonychia associated with nail plate fissuring, splitting, or dystrophy (picture 17)
● Melanonychia extending to the nail folds (Hutchinson sign) (picture 41B) (see 'Longitudinal melanonychia' above)
The ABCDEF mnemonic may also be helpful in recalling clinical features that raise the suspicion of melanoma [113]:
● Age of patient (peak incidence in the fifth to seventh decade)
● Band of Brown/Black color; Breadth greater than 3 mm; Border (irregular/blurred)
● Change in the band (rapid increase in size or growth rate)
● Digit involved (in order of decreasing frequency: thumb > hallux > index finger > single digit > multiple digits)
● Extension of pigment to the proximal or lateral nail fold (Hutchison sign) or free edge of nail plate
● Family history of melanoma
Nail plate dermoscopy may be helpful in the early diagnosis of nail melanoma. Dermoscopic features associated with nail melanoma include (picture 43)
[39,40,114]:
● Brown background hue
● Presence of irregular longitudinal lines (in their color, spacing, thickness, and parallelism)
● Micro-Hutchinson sign (pigmentation of the cuticle seen on dermoscopy but not with the naked eye)
(See "Dermoscopy of nail pigmentations", section on 'Melanoma'.)
However, histopathology is the gold standard for the diagnosis of nail apparatus melanoma. On histology, early nail melanoma is characterized by an increased
number of atypical melanocytes at the dermoepidermal junction, with a predominance of single cells over nests [38]. Suprabasal scatter involving the superficial
aspect of the matrix may be seen. Atypical melanocytes have large, hyperchromatic, pleomorphic nuclei, prominent nucleoli, increased mitoses, and long
branching dendrites [26]. Invasive nail unit melanoma displays many of the features that are observed elsewhere on the acral skin [115]. (See "Pathologic
characteristics of melanoma", section on 'Acral lentiginous melanoma'.)
The techniques for performing nail matrix biopsy are discussed separately. (See "Nail biopsy: Indications and techniques", section on 'Nail matrix biopsy'.)
The surgical treatment of subungual melanoma is discussed separately. (See "Initial surgical management of melanoma of the skin and unusual sites", section
on 'Subungual'.)
SKIN DISEASES WITH NAIL INVOLVEMENT
Psoriasis — Psoriasis is the most common dermatosis involving the nails [116,117]. (See "Nail psoriasis".)
Clinical features that suggest nail psoriasis include [116]:
● Nail plate pitting – Nail pitting is the most common sign of nail psoriasis. It results from focal areas of abnormal keratinization of the nail matrix that produce
foci of parakeratotic cells in the dorsal nail plate as it grows beyond the cuticle. Psoriatic pits are typically irregular, deep, and randomly distributed within the
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nail plate (picture 6A).
● Nail bed salmon patches – Nail bed salmon patches (also termed "oil drops") are irregular areas of yellow or pink discoloration in the nail bed visible
through the nail plate, resulting from psoriatic inflammation of the nail bed (picture 20C).
● Onycholysis – Onycholysis results from the distal separation of the nail plate from the inflamed underlying nail bed. An erythematous border and splinter
hemorrhages are often associated with onycholysis (picture 20B). Onycholysis and subungual hyperkeratosis may be the only manifestations of psoriasis of
the toenails (picture 20D).
Other signs of nail psoriasis include paronychia, leukonychia (white nails), erythema of the lunula, and trachyonychia (rough, lusterless nails) [116,117]. (See
'Leukonychia' above and 'Trachyonychia' above.)
Psoriasis of the nail folds resembles chronic paronychia and is often precipitated by treatment with systemic retinoids [118].
Mycology testing is indicated to differentiate psoriatic nail disease from onychomycosis, particularly if the disease is limited to the toenails. However,
onychomycosis and nail psoriasis may coexist in approximately 20 percent of patients with psoriasis [119].
The treatment of nail psoriasis is discussed separately. (See "Nail psoriasis", section on 'Treatment'.)
Parakeratosis pustulosa — Parakeratosis pustulosa is a nail disorder that occurs almost exclusively in children (picture 44) [120]. In most cases the disease is
limited to one nail, usually the thumb or the index finger, and presents with distal onycholysis, fingertip desquamation, and mild subungual hyperkeratosis.
Parakeratosis pustulosa begins with an acute eczematous inflammation of the periungual skin, causing hyperkeratosis and thickening of the free edges of the
nail. Scaling is more marked than pustulation. The course is often protracted and recurrences are the rule. Spontaneous regression may occur after puberty.
Lichen planus — Lichen planus is an inflammatory condition of unknown etiology that affects the skin, mucous membranes, hair follicles, and nails.
Approximately 10 to 25 percent of patients with lichen planus have nail involvement [116,117,121,122]. Lichen planus of the nails occurs more commonly in the
adult population and typically affects several or most nails.
The clinical features of lichen planus vary by site of involvement within the nail apparatus [116,117]. Lichen planus of the nail matrix results in longitudinal ridging,
nail plate thinning, longitudinal fissuring (picture 45A), trachyonychia (picture 45B), and erythema of the lunula [116]. Postinflammatory hyperpigmentation of the
proximal nail fold and longitudinal melanonychia can also occur with nail matrix involvement [123]. Nail bed lichen planus usually occurs in conjunction with nail
matrix involvement and is characterized by onycholysis with or without subungual hyperkeratosis.
Lichen planus of the nails can be rapidly progressive. Permanent nail dystrophy in the forms of anonychia and dorsal pterygium (the extension and adherence of
the proximal nail fold to the nail bed secondary to scarring of the nail matrix) (picture 45C and picture 35) may occur.
Lichen planus is usually diagnosed by clinical examination alone, but if the diagnosis is questionable, a 3 mm punch biopsy of the nail matrix and/or nail bed is
usually conclusive. Histology reveals a band-like infiltrate of the nail matrix and nail bed dermis, with hyperkeratosis, hypergranulosis, and acanthosis of the nail
matrix epithelium [124].
Lichen planus of the nails is generally treated with systemic or intralesional corticosteroids. Systemic corticosteroids are preferred in cases that are rapidly
progressive or involve more than three nails. Oral prednisone 0.5 to 1 mg/kg per day (maximum 60 mg per day) is given for four to six weeks and then tapered
over the next four to six weeks [125]. As an alternative, monthly intramuscular injections of triamcinolone acetonide at a dose of 0.5 mg/kg may be given for
three to six months [116,117,126].
Intralesional corticosteroid therapy is an option when less than three nails are involved. Triamcinolone acetonide diluted to 2.5 to 5.0 mg/mL can be injected into
the proximal and lateral nail folds at monthly intervals [126]. These injections are placed intradermally in the nail folds, from which the solution can diffuse to the
underlying matrix. Injection directly into the nail matrix is uncomfortable and unnecessary. Treatment is generally continued at monthly intervals until the proximal
one-half of the nail appears normal, at which time injections can be tapered.
In patients who do not respond to treatment, systemic or topical corticosteroids should be stopped after five to six months. Acitretin at a dose of 0.35 mg/kg per
day may be a treatment option in recalcitrant cases [126].
The treatment of nail lichen planus with topical or systemic corticosteroids has not been evaluated in randomized trials. Their use is based upon clinical
experience and limited evidence from small case series.
In a study of 27 patients with nail lichen planus treated with intramuscular or intralesional corticosteroids who were followed for more than 5 years (mean follow-
up 10 years), 9 patients did not respond to treatment, 18 were cured, and 11 relapsed [126]. In another study, 67 patients with histologically confirmed lichen
planus of the nails were treated with systemic and/or intralesional corticosteroids for six months [124]. Complete or substantial improvement was reported in 42
patients (63 percent).
Alopecia areata — Nail involvement occurs in approximately 50 percent of children and 20 percent of adults with alopecia areata [127]. Nail changes may not
occur at the same time as hair loss and are more common in males and severe cases [116,117,127-129]. Abnormalities may involve one or more nails and
include mottled erythema of the lunula ("spotted lunula"), longitudinal ridging or trachyonychia (picture 6C), and geometric pitting (multiple, small, superficial pits
regularly distributed along longitudinal and transverse lines on the nail plate) (picture 6B). (See "Clinical manifestations and diagnosis of alopecia areata".)
The diagnosis is usually made clinically, but a nail matrix biopsy may be performed in questionable cases. Pathology reveals a lymphocytic infiltrate with
spongiosis in the proximal nail fold, matrix, nail bed, and hyponychium [17].
Nail abnormalities are typically stable and improve spontaneously over a period of years. Topical corticosteroids or monthly intralesional injections of 2.5 to 3
mg/mL of triamcinolone acetonide into the proximal nail fold can be administered if spontaneous clearing of the nails does not occur [117].
Darier disease — Darier disease, also termed keratosis follicularis, is an autosomal dominant condition characterized by greasy hyperkeratotic papules in
seborrheic regions. Approximately 90 percent of patients with Darier disease have nail involvement [117]. (See "Darier disease".)
Fingernails are affected more often than toenails. Nail matrix involvement results in onychorrhexis (nail fragility), splitting, fragility, and red and white longitudinal
streaks in the nail plate (picture 46). Nail bed involvement is characterized by subungual hyperkeratosis and V-shaped notches in the distal nail plate at the free
edge. Keratotic papules may be present over the proximal nail fold.
Sarcoidosis — Nail involvement is rare in patients with sarcoidosis. Nails appear yellow and dystrophic, often with splinter hemorrhages, nail plate pitting and/or
crumbling, subungual hyperkeratosis, and painful paronychia (picture 47) [130,131]. (See "Cutaneous manifestations of sarcoidosis", section on 'Nail
sarcoidosis'.)
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NAIL SIGNS OF SYSTEMIC DISEASES — Nail abnormalities associated with systemic diseases usually involve most or all nails. Signs of temporary
disturbance in nail growth, such as Beau lines and onychomadesis, may occur in association with high fever, viral diseases (eg, hand, foot, and mouth disease),
or Kawasaki syndrome [9,10,132]. (See 'Transverse grooves (Beau lines and onychomadesis)' above.)
Permanent or prolonged abnormalities of nail shape, thickness, and color associated with systemic diseases include yellow nail syndrome, digital clubbing, half-
and-half nails (apparent leukonychia), and koilonychia.
Yellow nail syndrome — Yellow nail syndrome is an uncommon disorder characterized by the triad of pulmonary disease, lymphedema, and slow-growing,
yellow nails without a cuticle or lunula (picture 48) [133-135]. Nails progressively thicken, become opaque and curved with loss of the lunula and cuticle. Swelling
of the periungual tissue and onycholysis can occur. In most cases yellow nail syndrome is sporadic, but it may be inherited in autosomal dominant or recessive
fashion [136]. A number of pulmonary diseases are associated with this syndrome including pleural effusion, bronchiectasis, and chronic sinusitis [137]. (See
"Diagnostic evaluation of pleural effusion in adults: Additional tests for undetermined etiology", section on 'History'.)
Clubbing — Digital clubbing is characterized by increased distal finger tip mass and increased longitudinal and transverse nail plate curvature (picture 49A-C)
[130,133,138,139]. The Lovibond angle, the angle between the nail plate and the proximal nail fold when viewed from the side, is >180° in clubbed nails and
160° in normal nails (figure 4). Digital clubbing can be acquired or hereditary.
● Acquired bilateral clubbing is the most common form. It usually begins in the thumb and index fingers and is most often associated with pulmonary or
cardiovascular diseases, including lung cancer, interstitial pulmonary fibrosis, lung abscess, pulmonary tuberculosis, pulmonary lymphoma, congestive heart
failure, infective endocarditis, and cyanotic congenital heart disease [138]. Less frequently, digital clubbing may occur in patients with extrathoracic disease,
including inflammatory bowel disease, liver cirrhosis, and gastrointestinal neoplasms. (See "Approach to the adult with interstitial lung disease: Clinical
evaluation", section on 'Clubbing' and "Clinical presentation and diagnosis of inflammatory bowel disease in children", section on 'Extraintestinal
manifestations'.)
● Acquired unilateral or single-digit clubbing is commonly related to nearby vascular lesions such as a peripheral shunt, arteriovenous fistula, or aneurysm,
but Pancoast tumors, lymphadenitis, or erythromelalgia can also cause unilateral clubbing [130,133]. Single nail involvement is typically traumatic but may
be congenital. (See "Superior pulmonary sulcus (Pancoast) tumors".)
● Isolated congenital digital clubbing (MIM#119900) is considered an incomplete form of primary hypertrophic osteoarthropathy (PHO) [140]. PHO is an
autosomal recessive disorder due to mutations in the 15-hydroxyprostaglandin dehydrogenase (HPGD) gene [141]. It presents in otherwise healthy children
with clubbing, periostosis, and skin manifestations including thickening of the skin of the face and scalp, coarsening of facial features, hyperhidrosis, and
seborrhea. (See "Malignancy and rheumatic disorders", section on 'Hypertrophic osteoarthropathy'.)
Thyroid acropachy — Thyroid acropachy is a rare manifestation of Graves' disease characterized by digital clubbing, soft-tissue swelling of the hands and feet,
and periosteal reaction with new bone formation (picture 50). It is almost always associated with thyroid dermopathy and exophthalmos and usually becomes
apparent after the diagnosis and treatment of hyperthyroidism. (See "Overview of the clinical manifestations of hyperthyroidism in adults".)
Half-and-half nails — Half-and-half nails (also termed apparent leukonychia or Lindsay nails) are a manifestation of chronic renal insufficiency and uremia
[142,143]. A half-and-half nail typically exhibits a red, pink, or brown horizontal distal band that occupies 20 to 60 percent of the total length of the nail
[130,133,144]. The proximal portion of a half-and-half nail usually has a dull, white ground-glass appearance due to underlying nail bed changes (picture 51).
The nails may revert to normal following renal transplantation [145].
Koilonychia — Koilonychia, also called spoon nail, is the upward curving of the distal nail plate that results in a spoon-shaped nail that could hold a drop of
water on the surface (picture 52A-B). Koilonychia has been associated with iron deficiency and other systemic conditions in rare case reports; however, it is
more commonly seen as an occupational change in nails and may be idiopathic. Ruling out iron deficiency anemia in someone with koilonychia is the only work-
up necessary in this condition. (See "Iron deficiency in infants and children <12 years: Screening, prevention, clinical manifestations, and diagnosis".)
SUMMARY
● The nail unit is composed of the nail matrix, nail bed, proximal and lateral nail folds, and hyponychium (picture 1 and figure 1). Acquired nail disorders may
involve all the components of the nail unit. They include infections, tumors, disorders associated with skin or systemic diseases, and abnormal pigmentation.
(See 'Anatomy and physiology of the nail unit' above.)
● Common signs of nail disease include transverse and longitudinal grooves (picture 2A-C, 3A-B, 38D), onychorrhexis (picture 4), pitting (picture 6A-B),
trachyonychia (picture 6C), leukonychia (picture 8), longitudinal melanonychia (picture 11), longitudinal erythronychia (picture 18A-B), splinter hemorrhages,
onycholysis (picture 20B), and subungual hyperkeratosis (picture 20D). (See 'Signs of nail disease' above.)
● Longitudinal melanonychia is a longitudinal pigmented band in the nail caused by the presence of melanin in the nail plate. Most cases of longitudinal
melanonychia, including ethnic melanonychia and melanonychia associated with skin or systemic disease or drugs result from activation of nail matrix
melanocytes (picture 14A-B and table 3A-B).
Longitudinal melanonychia may also be the manifestation of melanocytic proliferation within the epithelium of the nail matrix. Differentiating a benign
pigmented lesion of the nail lesion (lentigo or nevus) (picture 15A-B) from early nail melanoma (picture 17) may be extremely difficult. Thus, a diagnostic
biopsy is often necessary to rule out melanoma. (See 'Longitudinal melanonychia' above and 'Melanoma' above and 'When to biopsy' above.)
● Common nail infections include onychomycosis (picture 25A-E); bacterial infections, including acute paronychia (picture 26A-B), blistering distal dactylitis
(picture 27A-C), pulp space infection (felon) (picture 28), and green nail syndrome (picture 29); and viral infections such as warts (picture 30) and herpetic
whitlow (picture 31). (See 'Infections' above.)
● Benign nail tumors include fibromas (picture 32A-D), onychomatricoma (picture 33), digital myxoid cyst (picture 36A-B), pyogenic granuloma (picture 37),
glomus tumor (picture 38A-D), and subungual exostosis (picture 39). (See 'Benign tumors' above.)
● Squamous cell carcinoma (SCC), including squamous cell carcinoma in situ, is the most common malignant tumor of the nail. It presents with nonspecific
symptoms, such as hyperkeratosis, persistent onycholysis, longitudinal erythronychia, verruca, paronychia, nail plate dystrophy, or a subungual mass
(picture 18B-E). (See 'Squamous cell carcinoma' above.)
● Nail melanoma is a rare form of acral melanoma. In two thirds of cases, nail melanoma presents as a brown to black longitudinal stripe in the nail plate,
known as longitudinal melanonychia (picture 41A-B) [26]. In one-third of cases, nail melanoma is amelanotic and presents as a nail bed mass or nail plate
abnormality (picture 42A). (See 'Melanoma' above.)
● Clinical features that may suggest early nail melanoma and warrant a biopsy of the nail matrix include (algorithm 1):
• Melanonychia that develops during adulthood, involves a single digit (in particular, the thumb, index finger, or great toe), and enlarges rapidly
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• Longitudinal melanonychia >3 mm in width (picture 12) with variegated pigmentation or proximal widening (triangular shape) (picture 41A)
• Preexisting longitudinal melanonychia that becomes darker or wider or demonstrates blurred lateral borders
• Longitudinal melanonychia associated with nail plate fissuring, splitting, or dystrophy (picture 17)
• Melanonychia extending to the nail folds (Hutchinson sign) (picture 41B)
● The nails may be involved in numerous skin diseases, including psoriasis (picture 6A, 20B-D), parakeratosis pustulosa (picture 44), lichen planus (picture
45A-C), alopecia areata (picture 6B-C), Darier disease (picture 46), and sarcoidosis (picture 47).
● Nail abnormalities associated with systemic diseases usually involve most or all nails. Signs of temporary disturbance in nail growth such as Beau lines and
onychomadesis may occur in association with high fever, viral diseases (eg, hand, foot, and mouth disease), or Kawasaki syndrome. Permanent or
prolonged abnormalities of nail shape, thickness, and color associated with systemic diseases include yellow nail syndrome (picture 48), clubbing (picture
49A-C), and half-and-half nails (apparent leukonychia) (picture 51). (See 'Nail signs of systemic diseases' above.)
ACKNOWLEDGMENT — The editorial staff at UpToDate would like to acknowledge Julie A Jefferson, MD, who contributed to an earlier version of this topic
review.
Use of UpToDate is subject to the Subscription and License Agreement.
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45. Baran R, Perrin C. Focal subungual warty dyskeratoma. Dermatology 1997; 195:278.
46. Cohen PR. Longitudinal erythronychia: individual or multiple linear red bands of the nail plate: a review of clinical features and associated conditions. Am J
Clin Dermatol 2011; 12:217.
47. Baran R, Dawber RP, Richert B. Physical signs. In: Baran and Dawber's Diseases of the nails and their management, 3rd ed, Baran R, Dawber RP, De Ber
ker DAR, Haneke E, Tosti A (Eds), Blackwell Science, Malden, MA 2001. p.48.
48. Zaias N, Ackerman AB. The nail in Darier-White disease. Arch Dermatol 1973; 107:193.
49. Siragusa M, Schepis C, Cosentino FI, et al. Nail pathology in patients with hemiplegia. Br J Dermatol 2001; 144:557.
50. Baran R, Dawber RP, Perrin C, Drape JL. Idiopathic polydactylous longitudinal erythronychia: a newly described entity. Br J Dermatol 2006; 155:219.
51. van der Velden HM, Klaassen KM, van de Kerkhof PC, Pasch MC. Fingernail psoriasis reconsidered: a case-control study. J Am Acad Dermatol 2013;
69:245.
52. Salem A, Al Mokadem S, Attwa E, et al. Nail changes in chronic renal failure patients under haemodialysis. J Eur Acad Dermatol Venereol 2008; 22:1326.
53. Silverman ME, Upshaw CB Jr. Extracardiac manifestations of infective endocarditis and their historical descriptions. Am J Cardiol 2007; 100:1802.
54. García-Carrasco M, Galarza C, Gómez-Ponce M, et al. Antiphospholipid syndrome in Latin American patients: clinical and immunologic characteristics and
comparison with European patients. Lupus 2007; 16:366.
55. Tunc SE, Ertam I, Pirildar T, et al. Nail changes in connective tissue diseases: do nail changes provide clues for the diagnosis? J Eur Acad Dermatol
Venereol 2007; 21:497.
56. Nakamura R, Broce AA, Palencia DP, et al. Dermatoscopy of nail lichen planus. Int J Dermatol 2013; 52:684.
57. Daniel CR III. Simple Onycholysis. In: Nails: Diagnosis, Therapy, and Surgery, Scher RK, Daniel CR III, et al (Eds), Elsevier Saunders, Oxford 2005. p.97.
58. Rich P, Scher RK. Nail signs and their definitions: non-specific nail dystrophies. In: An Atlas of Diseases of the Nail, Parthenon Publishing, New York 2003.
p.13.
59. Baumgartner M, Haneke E. Retronychia: diagnosis and treatment. Dermatol Surg 2010; 36:1610.
60. de Berker DA, Richert B, Duhard E, et al. Retronychia: proximal ingrowing of the nail plate. J Am Acad Dermatol 2008; 58:978.
61. Braswell MA, Daniel CR 3rd, Brodell RT. Beau lines, onychomadesis, and retronychia: A unifying hypothesis. J Am Acad Dermatol 2015; 73:849.
62. Piraccini BM, Richert B, de Berker DA, et al. Retronychia in children, adolescents, and young adults: a case series. J Am Acad Dermatol 2014; 70:388.
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64. Singh G, Haneef NS, Uday A. Nail changes and disorders among the elderly. Indian J Dermatol Venereol Leprol 2005; 71:386.
65. Cohen PR, Scher RK. Geriatric nail disorders: diagnosis and treatment. J Am Acad Dermatol 1992; 26:521.
66. Elewski BE. Onychomycosis. Treatment, quality of life, and economic issues. Am J Clin Dermatol 2000; 1:19.
67. Lawry M, Daniel CR III. Nonfungal Infections and Acute Paronychia. In: Nails: Diagnosis, Therapy, and Surgery, Scher RK, Daniel CR III, et al (Eds), Elsevi
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68. Scheinfeld NS. Is blistering distal dactylitis a variant of bullous impetigo? Clin Exp Dermatol 2007; 32:314.
69. Hays GC, Mullard JE. Blistering distal dactylitis: a clinically recognizable streptococcal infection. Pediatrics 1975; 56:129.
70. Rich P, Scher RK. Infectious causes of Nail Disorders. In: An Atlas of Diseases of the Nail, Parthenon Publishing, New York 2003. p.41.
71. Rubright JH, Shafritz AB. The herpetic whitlow. J Hand Surg Am 2011; 36:340.
72. Abimelec P, Dumontier C. Basic and Advanced Nail Surgery (Part 2: Indications and Complications). In: Nails: Diagnosis, Therapy, and Surgery, Scher RK,
Daniel RC III, et al (Eds), Elsevier Saunders, Oxford 2005. p.291.
73. Baran R, Perrin C, Baudet J, Requena L. Clinical and histological patterns of dermatofibromas of the nail apparatus. Clin Exp Dermatol 1994; 19:31.
74. Rich P, Scher RK. Nail Tumors. In: An Atlas of Diseases of the Nail, Parthenon Publishing, New York 2003. p.83.
75. Cahn RL. Acquired periungual fibrokeratoma. A rare benign tumor previously described as the garlic-clove fibroma. Arch Dermatol 1977; 113:1564.
76. Cañueto J, Santos-Briz Á, García JL, et al. Onychomatricoma: genome-wide analyses of a rare nail matrix tumor. J Am Acad Dermatol 2011; 64:573.
77. Baran R, Richert B. Common nail tumors. Dermatol Clin 2006; 24:297.
78. Gaertner EM, Gordon M, Reed T. Onychomatricoma: case report of an unusual subungual tumor with literature review. J Cutan Pathol 2009; 36 Suppl 1:66.
79. Perrin C, Goettmann S, Baran R. Onychomatricoma: clinical and histopathologic findings in 12 cases. J Am Acad Dermatol 1998; 39:560.
80. Fayol J, Baran R, Perrin C, Labrousse F. Onychomatricoma with misleading features. Acta Derm Venereol 2000; 80:370.
81. Raison-Peyron N, Alirezai M, Meunier L, et al. Onychomatricoma: an unusual cause of nail bleeding. Clin Exp Dermatol 1998; 23:138.
82. Goettmann S, Zaraa I, Moulonguet I. Onychomatricoma with pterygium aspect: unusual clinical presentation. Acta Derm Venereol 2006; 86:369.
83. Perrin C, Baran R. Onychomatricoma with dorsal pterygium: pathogenic mechanisms in 3 cases. J Am Acad Dermatol 2008; 59:990.
84. Perrin C, Baran R, Balaguer T, et al. Onychomatricoma: new clinical and histological features. A review of 19 tumors. Am J Dermatopathol 2010; 32:1.
85. Fraga GR, Patterson JW, McHargue CA. Onychomatricoma: report of a case and its comparison with fibrokeratoma of the nailbed. Am J Dermatopathol
2001; 23:36.
86. Lam C, Weyant GW, Billingsley EM. Longitudinal melanonychia of the toenail. JAMA Dermatol 2014; 150:449.
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87. Miteva M, de Farias DC, Zaiac M, et al. Nail clipping diagnosis of onychomatricoma. Arch Dermatol 2011; 147:1117.
88. Richert B. Surgery of the distal interphalangeal joint. In: Nail Surgery, Richert B, Di Chiacchio N, Haneke E (Eds), Informa Healthcare, New York 2011. p.16
5.
89. de Berker D, Goettman S, Baran R. Subungual myxoid cysts: clinical manifestations and response to therapy. J Am Acad Dermatol 2002; 46:394.
90. Drapé JL, Idy-Peretti I, Goettmann S, et al. MR imaging of digital mucoid cysts. Radiology 1996; 200:531.
91. Tosti A, Richert B, Massimiliano P. Tumors of the nail apparatus. In: Nails: Diagnosis, Therapy, and Surgery, Scher RK, Daniel RC, et al (Eds), Elsevier Sau
nders, Oxford 2005. p.195.
92. Gombos Z, Zhang PJ. Glomus tumor. Arch Pathol Lab Med 2008; 132:1448.
93. Richert B, Haneke E, Di Chiacco N. Surgery of the nail bed. In: Nail Surgery, Richert B, Di Chiacchio N, Haneke E (Eds), Informa Healthcare, New York 201
1. p.55.
94. Bhaskaranand K, Navadgi BC. Glomus tumour of the hand. J Hand Surg Br 2002; 27:229.
95. Cigna E, Carlesimo B, Bistoni G, et al. The value of clinical diagnosis of digital glomus tumors. Acta Chir Plast 2008; 50:55.
96. Marchadier A, Cohen M, Legre R. [Subungual glomus tumors of the fingers: ultrasound diagnosis]. Chir Main 2006; 25:16.
97. Wortsman X, Jemec GB. Role of high-variable frequency ultrasound in preoperative diagnosis of glomus tumors: a pilot study. Am J Clin Dermatol 2009;
10:23.
98. Drapé JL, Idy-Peretti I, Goettmann S, et al. Subungual glomus tumors: evaluation with MR imaging. Radiology 1995; 195:507.
99. Haneke E, Di Chiacco N, Richert B. Surgery of the bony phalanx. In: Nail Surgery, Richert B, Di Chiacchio N, Haneke E (Eds), Informa Healthcare, New Yo
rk 2011. p.149.
100. Tosti A, Schneider SL, Ramirez-Quizon MN, et al. Clinical, dermoscopic, and pathologic features of onychopapilloma: A review of 47 cases. J Am Acad
Dermatol 2016; 74:521.
101. Jellinek NJ, Lipner SR. Longitudinal Erythronychia: Retrospective Single-Center Study Evaluating Differential Diagnosis and the Likelihood of Malignancy.
Dermatol Surg 2016; 42:310.
102. Criscione V, Telang G, Jellinek NJ. Onychopapilloma presenting as longitudinal leukonychia. J Am Acad Dermatol 2010; 63:541.
103. Ito T, Uchi H, Yamada Y, et al. Onychopapilloma manifesting longitudinal melanonychia: A mimic of subungual malignancy. J Dermatol 2015; 42:1199.
104. Miteva M, Fanti PA, Romanelli P, et al. Onychopapilloma presenting as longitudinal melanonychia. J Am Acad Dermatol 2012; 66:e242.
105. Tang N, Maloney ME, Clark AH, Jellinek NJ. A Retrospective Study of Nail Squamous Cell Carcinoma at 2 Institutions. Dermatol Surg 2016; 42 Suppl 1:S8.
106. Baran R, Goettmann S. Distal digital keratoacanthoma: a report of 12 cases and a review of the literature. Br J Dermatol 1998; 139:512.
107. Montes CM, Maize JC, Guerry-Force ML. Incontinentia pigmenti with painful subungual tumors: a two-generation study. J Am Acad Dermatol 2004; 50:S45.
108. de Berker DA, Dahl MG, Malcolm AJ, Lawrence CM. Micrographic surgery for subungual squamous cell carcinoma. Br J Plast Surg 1996; 49:414.
109. Young LC, Tuxen AJ, Goodman G. Mohs' micrographic surgery as treatment for squamous dysplasia of the nail unit. Australas J Dermatol 2012; 53:123.
110. Dalle S, Depape L, Phan A, et al. Squamous cell carcinoma of the nail apparatus: clinicopathological study of 35 cases. Br J Dermatol 2007; 156:871.
111. Thai KE, Young R, Sinclair RD. Nail apparatus melanoma. Australas J Dermatol 2001; 42:71.
112. Saida T, Ohshima Y. Clinical and histopathologic characteristics of early lesions of subungual malignant melanoma. Cancer 1989; 63:556.
113. Levit EK, Kagen MH, Scher RK, et al. The ABC rule for clinical detection of subungual melanoma. J Am Acad Dermatol 2000; 42:269.
114. Thomas L, Dalle S. Dermoscopy provides useful information for the management of melanonychia striata. Dermatol Ther 2007; 20:3.
115. Shin HT, Jang KT, Mun GH, et al. Histopathological analysis of the progression pattern of subungual melanoma: late tendency of dermal invasion in the nail
matrix area. Mod Pathol 2014; 27:1461.
116. Tosti A, Piraccini BM. Dermatological Diseases. In: Nails: Diagnosis, Therapy, and Surgery, Scher RK, Daniel RC, et al (Eds), Elsevier Saunders, Oxford 20
05. p.105.
117. Rich P, Scher RK. Nail manifestations of cutaneous disease. In: An Atlas of Diseases of the Nail, Parthenon Publishing, New York 2003. p.51.
118. Baran R. Retinoids and the nails. J Dermatol Treat 1990; 1:151.
119. Klaassen KM, Dulak MG, van de Kerkhof PC, Pasch MC. The prevalence of onychomycosis in psoriatic patients: a systematic review. J Eur Acad Dermatol
Venereol 2014; 28:533.
120. Richert B, André J. Nail disorders in children: diagnosis and management. Am J Clin Dermatol 2011; 12:101.
121. Scher RK. Lichen planus of the nail. Dermatol Clin 1985; 3:395.
122. Tosti A, Peluso AM, Fanti PA, Piraccini BM. Nail lichen planus: clinical and pathologic study of twenty-four patients. J Am Acad Dermatol 1993; 28:724.
123. Juhlin L, Baran R. On longitudinal melanonychia after healing of lichen planus. Acta Derm Venereol 1990; 70:183.
124. Goettmann S, Zaraa I, Moulonguet I. Nail lichen planus: epidemiological, clinical, pathological, therapeutic and prognosis study of 67 cases. J Eur Acad
Dermatol Venereol 2012; 26:1304.
125. Cribier B, Frances C, Chosidow O. Treatment of lichen planus. An evidence-based medicine analysis of efficacy. Arch Dermatol 1998; 134:1521.
126. Piraccini BM, Saccani E, Starace M, et al. Nail lichen planus: response to treatment and long term follow-up. Eur J Dermatol 2010; 20:489.
127. Tosti A, Morelli R, Bardazzi F, Peluso AM. Prevalence of nail abnormalities in children with alopecia areata. Pediatr Dermatol 1994; 11:112.
128. Tosti A, Fanti PA, Morelli R, Bardazzi F. Trachyonychia associated with alopecia areata: a clinical and pathologic study. J Am Acad Dermatol 1991; 25:266.
129. Sharma VK, Dawn G, Muralidhar S, Kumar B. Nail changes in 1000 Indian patients with alopecia areata. J Eur Acad Dermatol Venereol 1998; 10:189.
130. Lawry M, Daniel CR III. Nails in Systemic Disease. In: Nails: Diagnosis, Therapy, and Surgery, Scher RK, Daniel CR III, et al (Eds), Elsevier Saunders, Oxf
ord 2005. p.147.
131. Santoro F, Sloan SB. Nail dystrophy and bony involvement in chronic sarcoidosis. J Am Acad Dermatol 2009; 60:1050.
132. Ciastko AR. Onychomadesis and Kawasaki disease. CMAJ 2002; 166:1069.
133. Rich P, Scher RK. Nail Signs of systemic disease. In: An Atlas of Diseases of the Nail, Parthenon Publishing, New York 2003. p.61.
134. SAMMAN PD, WHITE WF. THE "YELLOW NAIL" SYNDROME. Br J Dermatol 1964; 76:153.
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144. Lindsay PG. The half-and-half nail. Arch Intern Med 1967; 119:583.
145. Saray Y, Seçkin D, Güleç AT, et al. Nail disorders in hemodialysis patients and renal transplant recipients: a case-control study. J Am Acad Dermatol 2004;
50:197.
Topic 13682 Version 22.0
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GRAPHICS
Nail anatomy
Graphic 85578 Version 2.0
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Anatomy of the nail apparatus
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Nail growth: Direction of matrix cell differentiation and

movement
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Key questions for the patient with abnormal nails
What do you think is the cause of your nail problem?
Were you born with this problem? If no, when did this problem begin?
Which nails were affected first?
Which nails are affected now?
How has your nail condition progressed?
Using general terms, describe your nails (hard, soft, brittle, etc).
Have you ever traumatized any of the involved nails?
Do you do anything to affect your nails or the tips of your fingers or toes?
Do any of your hobbies affect or traumatize your nails (ie, tennis, jogging, basketball, racquetball, painting, playing the piano, etc)?
What is your occupation?
Have you done any of the following: Picked at your nails, bit or sucked on your nails, torn your nails off, worn tight or pointed-toe shoes, or pushed the cuticle back? If yes,
when and how often?
Have you ever had ingrown nails?
Are your hands in water often?
Do your nails ever come in contact with any chemicals or irritants such as strong soaps, detergents, hair color/straightening chemicals, or dyes?
What nail cosmetics or conditioners do you use (ie, base coat, top coat, enamel, nail strengtheners, nail hardeners, cuticle treatment, gloss, nail conditioners, etc)?
Do you go to a manicurist/pedicurist? How often? What do you usually have done to your nails?
Have you ever had any of the following: Sculptured nails, false/artificial nails, gel nails, nail tips, nail wraps, acrylic nails, etc? If so, how often and when was the last time?
What instruments do you use when caring for your nails?
How do you use your nail care instruments?
How often do you use your nail care instruments?
Have you recently experienced swelling around the cuticles?
What treatments have you tried for your nail problem? Please list all pills, topical treatments, and surgical treatments and the dates.
Do you currently have, or have you ever had, any other skin or hair problems (ie, lichen planus, psoriasis, alopecia areata, ringworm, jock itch, athlete's foot, vaginal yeast
infection or other yeast infection, etc)?
Do you currently have, or have you ever had, any other medical problems (ie, diabetes mellitus, heart trouble, lung issues or difficulty breathing, kidney problems, thyroid
problems, gastrointestinal disorders, lower extremity swelling, etc)?
Is there any chance that you could be pregnant?
What prescription and nonprescription medications have you taken during the last year? Please include vitamins and herbal supplements.
Do you have any drug allergies?
Does anyone in your family have any of the following: Nail problems, thyroid problems, diabetes, hair and skin problems (ie, psoriasis, lichen planus, fungus, etc)?
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Transverse grooves of the nail plate (Beau lines)
Reproduced with permission from: www.visualdx.com. Copyright Logical Images, Inc.
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Beau line
The transverse groove on the nail plate is a Beau line.
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Beau lines
Transverse grooves representing Beau lines are present on the nail plates.
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Nail plate detachment from the matrix area (onychomadesis)
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Subungual glomus tumor with nail dystrophy
Nail dystrophy caused by a subungual glomus tumor. The red subungual patch indicates
the location of the tumor.
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Median nail dystrophy, also called median canaliform dystrophy

of Heller
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Longitudinal nail grooves with distal splits (onychorrhexis)
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Onychoschizia
Onychoschizia is characterized by horizontal splits of the distal nail plate.
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Nail pits in psoriasis
Numerous pits are present on the nail of this patient with psoriasis.
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Nail pitting
Numerous pits are present in this nail from a patient with alopecia areata.
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Trachyonychia
Rough surfaces of nail plates with longitudinal ridging are present in this patient
with trachyonychia secondary to alopecia areata.
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Trachyonychia (twenty nail dystrophy)
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Leukonychia (white patches or striae of the nail plate)
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Total leukonychia
Milky porcelain-white nails in a 30-year-old woman with total leukonychia.
Reproduced with permission from DermIS: Dermatology Information System. www.dermis.net.

Copyright © 2013. All rights reserved.
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Leukonychia striata (Mees' line)
White bands running parallel to the nail base in a 40-year-old man with leukonychia
striata.
Reproduced with permission from DermIS: Dermatology Information System. www.dermis.net.

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Ethnic melanonychia commonly seen in dark-skinned

individuals
Multiple longitudinal melanonychia bands are commonly seen in dark-skinned

individuals.
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Nail melanoma with the Hutchinson sign
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Nail matrix nevus with pseudo-Hutchinson sign
The darkly pigmented band is visible through the thin, transparent cuticle ("pseudo-
Hutchinson sign") in this child with a congenital nail matrix nevus.
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Conditions associated with pseudo-Hutchinson sign
Benign
Physiologic melanonychia in dark-skinned individuals
Lauzier-Hunziker syndrome
Peutz-Jeghers syndrome
Radiation therapy
Malnutrition
Minocycline-induced dyschromia
AIDS
Congenital nevus
Chronic trauma
Subungual hematoma
Illusory - pigmentation within the nail bed and/or matrix seen through the transparency of the cuticle
Malignant nonmelanoma
Bowen disease
Source: André J, Lateur N. Pigmented nail disorders. Dermatol Clin 2006; 24:329.
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Longitudinal melanonychia
Multiple pigmented linear streaks on the nail are common in individuals (adults
> children) with dark skin. The wart on the proximal nail fold of the thumb on
the left is an unrelated finding.
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Longitudinal melanonychia
A biopsy of the nail matrix revealed a junctional melanocytic nevus.
Reproduced with permission from: Schaffer JV, Bolognia JL. The clinical spectrum of
pigmented lesions. Clin Plast Surg 2000; 27:391. Copyright © 2000 Elsevier.
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Melanonychia caused by hydroxyurea
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Drugs inducing longitudinal melanonychia
Chemotherapeuticals Others
Bleomycin sulfate ACTH
Busulfan Amodiaquine
Cyclophosphamide Amorolfine
Dacarbazine Arsenic
Daunorubicin hydrochloride Chloroquine
Doxorubicin Clofazimine
Etoposide Clomipramine
5-fluorouracile Cyclones
Hydroxyurea Fluconazole
Imatinib Fluorides
Melphalan hydrochloride Gold salts

Methotrexate Ibuprofen
Nitrogen mustard Ketoconazole
Nitrosourea Lamivudine
Tegafur Mepacrine
Mercury
MSH
Minocycline
PCB
Phenytoin
Phenothiazine
Psoralen
Roxithromycin
Steroids
Sulfonamide
Thallium
Timolol
Zidovudine
Reproduced from: André J, Lateur N. Pigmented nail disorders. Dermatol Clin 2006; 24:329. Table used with the permission of Elsevier Inc. All rights reserved.
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Longitudinal melanonychia: Conditions associated with melanocytic activation and hyperplasia
Melanocytic activation
Physiologic causes
Racial melanonychia
Pregnancy
Local and regional causes
Repeated local trauma from poor footwear or overriding toes
Onychotillomania
Nail biting
Occupational trauma
Carpal tunnel syndrome
Dermatologic causes
Psoriasis
Lichen planus
Amyloidosis
Chronic radiation dermatitis
Systemic lupus erythematosus
Localized scleroderma
Chronic paronychia
Onychomycosis
Onychomatricoma
Bowen disease
Myxoid pseudocyst
Basal cell carcinoma
Subungual fibrous histiocytoma
Verruca vulgaris
Systemic causes
Endocrine disorders (Addison disease, Cushing syndrome, Nelson syndrome, hyperthyroidism, acromegaly)
Alcaptonuria
Nutritional disorders
Hemosiderosis
Hyperbilirubinemia
Porphyria
Graft versus host disease (lichen planus-type changes accompanied by longitudinal melanonychia)
AIDS
Iatrogenic causes
Phototherapy
X-ray exposure
Electron beam therapy
Drug intake
Syndromes
Laugier-Hunziker syndrome
Peutz-Jegher syndrome
Touraine syndrome
Melanocytic hyperplasia
Lentigo
Nevus
Congenital nevi
Acquired nevi
Nail apparatus in-situ and invasive melanoma
Source: André J, Lateur N. Pigmented nail disorders. Dermatol Clin 2006; 24:329.
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Lentigo of the nail matrix
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Nevus of the nail matrix in a child
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Dermoscopic image of nail lentigo
Dermoscopy shows brown band with irregular lines.
Courtesy of Antonella Tosti, MD.
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Dermoscopic image of a nail matrix nevus
A nail matrix nevus typically presents as multiple regular hyperpigmented lines with a brown background.
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Dermoscopic image of a nail matrix nevus in a child
Dermoscopy shows a brown background and irregular lines. Note leukonychia due to trauma.
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Nail melanoma in situ
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Approach to the differential diagnosis and management of longitudinal melanonychia
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Approach to the dermoscopic differential diagnosis and management of longitudinal melanonychia
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Longitudinal erythronychia secondary to melanoma in

situ
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Longitudinal erythronychia secondary to squamous cell

carcinoma
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Onychopapilloma
Onychopapilloma with distal triangular onycholysis and splinter hemorrhage.
From: Jellinek NJ, Lipner SR. Longitudinal erythronychia: Retrospective single-center study evaluating differential
diagnosis and the likelihood of malignancy. Dermatol Surg 2016; 42:310. DOI: 10.1097/DSS.0000000000000594.
Copyright © 2016 American Society for Dermatologic Surgery. Reproduced with permission from Lippincott Williams
& Wilkins. Unauthorized reproduction of this material is prohibited.
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Onychopapilloma
Onychopapilloma with distal subungual keratosis and splinter hemorrhage.
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Onychopapilloma
Onychopapilloma with confluent distal hemorrhage resembling melanonychia.
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Onychopapilloma
Onychopapilloma composed nearly entirely of splinter hemorrhages.
Copyright © 2016 American Society for Dermatologic Surgery. Reproduced with permission from Lippincott
Williams & Wilkins. Unauthorized reproduction of this material is prohibited.
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Splinter hemorrhages of the great toe
Trauma-induced splinter hemorrhages are typically located in the distal nail plate.
Reproduced with permission from: Stedman's Medical Dictionary. Copyright © 2008 Lippincott
Williams & Wilkins.
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Nail psoriasis
Onycholysis with erythema of the nail bed and splinter hemorrhages in a patient with nail
psoriasis.
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Splinter hemorrhages in Darier disease
Distal splinter hemorrhages of the nail in a patient with Darier disease.
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Splinter hemorrhages in infective endocarditis
Splinter hemorrhages, linear reddish-brown lesions, are seen in the nail bed of
this patient with bacterial endocarditis due to group B Streptococcus.
Courtesy of Gene Beyt. The Skin and Infection: A Color Atlas and Text, Sanders CV,
Nesbitt LT Jr (Eds), Williams & Wilkins, Baltimore 1995.
http://www.lww.com
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Retronychia
Thick, yellowish, onycholytic nail with whitish maceration proximally. Granulation

tissue is seen protruding from under the proximal nail fold.
From: Baumgartner M, Haneke E. Retronychia: Diagnosis and treatment. Dermatol

Surg 2010; 36:1610. DOI: 10.1111/j.1524-4725.2010.01693.x. Copyright © 2010
American Society for Dermatologic Surgery. Reproduced with permission from
Lippincott Williams & Wilkins. Unauthorized reproduction of this material is prohibited.
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Nail psoriasis with subungual hyperkeratosis
Marked subungual hyperkeratosis is present on several nails of this patient with

psoriasis.
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Onychogryphosis
Onychogryphosis, also called "ram's horn nail," usually affects the great toenail of older adult and
neglected individuals. The nail appears thickened, yellow-brown in color, increased in length, and
distorted.
Image created by Ken Gross, MD. Reproduced with permission from: www.visualdx.com. Copyright Logical
Images, Inc.
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Distal subungual onychomycosis
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Distal subungual onychomycosis
Nail discoloration and subungual hyperkeratosis are present on the nail. The area
immediately adjacent to the cuticle is not yet involved.
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Proximal subungual onychomycosis
Proximal, white discoloration of the nail plate is noted. The nail fold is
uninvolved.
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Whitish discoloration originating under the surface of the proximal nail plate is
present.
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Whitish discoloration originating under the surface of the proximal nail plate is
present.
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Acute paronychia
Swelling, erythema, and a purulent collection are present in this patient with
acute paronychia.
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Acute paronychia
Acute paronychia is a bacterial infection of the lateral nail fold and can result from nail biting,
hangnail, poor manicure technique, minor trauma, and, in children, thumb sucking.
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Blistering distal dactylitis
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Felon of the fingertip
The patient presented with three days of increased swelling, redness, and
severe pain of the fingertip.
Reproduced with permission from: Anthony J Viera, MD. Copyright © Anthony J

Viera, MD.
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Cross section of felon
The fingertip pulp contains compartments of eccrine sweat glands and fat
globules separated by fibrous septae. An abscess within these compartments is
a felon. The locations of the neurovascular bundles are shown; these areas
should be avoided when incision and drainage are performed.
Adapted from Clark DC. Common acute hand infections. Am Fam Physician 2003;
68:2167.
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Nail infection from Pseudomonas aeruginosa (green nail

syndrome)
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Periungual warts
Courtesy of Beth G Goldstein, MD, and Adam O Goldstein, MD.
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Herpetic whitlow
In addition to erythema, swelling, and pain, herpetic whitlow is characterized by

the presence of vesicular or pustular lesions. Patients may also experience fever,
lymphadenitis, and epitrochlear or axillary lymphadenopathy.
Reproduced with permission from: Nikkels AF, Peirard GE. Treatment of

mucocutaneous presentations of herpes simplex virus infections. Am J Clin Dermatol
2002; 3:479. Copyright © 2002 Adis International.
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Acquired ungual fibrokeratoma
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Subungual fibroma
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Nail fibroma
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Periungual fibroma in tuberous sclerosis (Koenen tumor)
Reproduced with permission from: The Dermatology Online Atlas, www.dermis.net. Copyright
© 2012. All rights reserved.
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Onychomatricoma
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Pincer nail deformity secondary to onychomatricoma
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Pterygium in lichen planus of the nail
The proximal nail fold is adherent to the nail bed.
Reproduced with permission from Bethanee J Schlosser, MD, PhD.
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Digital myxoid (mucous) cyst
A clear, viscous, jelly-like material can be expressed from a myxoid cyst after
puncturing it with a sterile needle.
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Longitudinal depression of the nail plate caused by a digital myxoid

(mucous) cyst
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Periungual pyogenic granuloma
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Glomus tumor
A 13-year-old boy developed a painful nodule on his right great toe nail bed.
Biopsy revealed a glomus tumor.
Copyright © Brovedani Piergiorgio, Dermatlas; http://www.dermatlas.org.
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Subungual glomus tumor
Bluish-red flush of the nail induced by a glomus tumor of the nail bed.
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Subungual glomus tumor
Left: Red flush of the nail bed induced by a subungual glomus tumor.
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Subungual exostosis
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Dermoscopic image of onychopapilloma
(A) A brown homogeneous band is visible in the nail.

(B) Free edge dermoscopy shows a keratotic subungual mass.
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Invasive squamous cell carcinoma of the nail
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Periungual Bowen disease (squamous cell carcinoma in situ)
Reproduced with permission from: The Dermatology Online Atlas, www.dermis.net. Copyright
© 2012. All rights reserved.
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Periungual Bowen disease (squamous cell carcinoma in

situ)
Reproduced with permission from: The Dermatology Online Atlas, www.dermis.net.

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Acral lentiginous melanoma in situ
Acral lentiginous melanoma in situ on the left index finger of a 74-year-old man, presenting as a linear pigmented streak
(melanonychia striata) with Hutchinson sign (pigmentation of the proximal and lateral nail folds).
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Subungual acral lentiginous melanoma
Melanoma originating from the nail matrix. An area of hyperpigmentation of the

proximal nail fold (Hutchinson sign) is present.
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Amelanotic subungual melanoma
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Dermoscopic image of amelanotic melanoma of the nail bed
Amelanotic melanoma of the nail bed showing linear irregular vessels and milky red areas on dermoscopy.
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Dermoscopy of nail melanoma
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Oil drop sign and distal onycholysis in psoriasis
Discoloration of the nail plate and distal onycholysis (separation of the nail plate
from the nail bed) are present in this patient with psoriasis.
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Parakeratosis pustulosa
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Longitudinal nail fissuring caused by lichen planus
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Trachyonychia induced by lichen planus
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Lichen planus of the nails, permanent nail dystrophy leading to

pterygium and anonychia
Permanent nail dystrophy resulting in anonychia and pterygium (extension and adherence of
the proximal nail fold to the nail bed secondary to scarring of the nail matrix).
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Darier disease
Distal notching and linear, red and white bands are present on the nails.
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Sarcoidosis involving the nail
Sarcoidal dactylitis. Swelling and deformity of all fingers are present. Some
fingers demonstrate erythema and swelling of the nail folds and beds. Marked
onychodystrophy is present.
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Yellow nail syndrome
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Digital clubbing
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Hypertrophic osteoarthropathy - digital clubbing
Bulbous swelling of the distal phalanx of the finger with increased nail curvature
is present.
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Hypertrophic osteoarthropathy - digital clubbing
Bulbous swelling of the distal phalanges of the fingers with increased nail
curvature is present.
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Clubbing of the fingers
In a normal finger, the length of the perpendicular dropped from point A to point
B should be greater than a similar line from C to D. In clubbing, the
relationships are reversed – that is, the distance C-D is greater than the
distance A-B. The other important change is the angle described by A-C-E. In
the normal finger this is usually <180 degrees, whereas in clubbing it is >180
degrees.
Panels A and C redrawn from: DeRemee RA. Facets of the algorithmic synthesis. In:
DeRemee RA, (Ed), Clinical profiles of diffuse interstitial pulmonary disease, Mount
Kisco, NY, Futura Publishing Company, Inc, 1990, pp. 9-44.
Panel B redrawn from: Bates B. A Guide to Physical Examination and History Taking,
5th Ed. Philadelphia: J.B. Lippincott Company, 1991.
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Thyroid acropachy
Digital clubbing and soft-tissue swelling in a patient with Graves disease.
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Half-and-half nails in chronic renal failure
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Koilonychia (spoon nail) associated with iron deficiency
Photo courtesy of Moise L Levy, MD. Reproduced with permission from: Chung EK, Boom JA,
Datto GA, Matz PS. Visual Diagnosis in Pediatrics. Philadelphia: Lippincott Williams & Wilkins,
2006. Copyright © 2006 Lippincott Williams & Wilkins.
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Koilonychia (spoon nail) associated with iron deficiency
Reproduced with permission from: Stedman's Medical Dictionary. Copyright © 2008

Lippincott Williams & Wilkins.
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Contributor Disclosures
Phoebe Rich, MD Grant/Research/Clinical Trial Support: Abbvie [Psoriasis (Biologic)]; Allergan [Rosacea (Topical)]; Amgen [Psoriasis (Biologic)]; Dusa [Actinic
(Topical)]; Eli Lilly [Keratosis (Biologic)]; Galderma [Psoriasis (Topical)]; Janssen [AV, atopic (Biologic)]; Merck & Co [Psoriasis (Biologic)]; Novartis
[Onychomycosis (Biologic)]; Pfizer, Inc [Actinic (Topical)]; Valeant [Psoriasis (Topical)]; Viamet [AKs, tinea (Systemic)]. Consultant/Advisory Boards: Novartis
[Psoriasis (Biologic)]; Allergan [Acne (Systemic)]. Erik Stratman, MD Nothing to disclose Rosamaria Corona, MD, DSc Nothing to disclose
Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are addressed by vetting through a multi-level review
process, and through requirements for references to be provided to support the content. Appropriately referenced content is required of all authors and must
conform to UpToDate standards of evidence.
Conflict of interest policy
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Overview of nail disorders - UpToDate https://www.uptodate.com/contents/overview-of-nail-disorders/print?s...

Official reprint from UpToDate®

Overview of nail disorders

Author: Phoebe Rich, MD

● (See "Dermoscopy of nail pigmentations".)

● (See "Nail avulsion and chemical matricectomy".)

● (See "Principles and overview of nail surgery".)

● (See "Nail biopsy: Indications and techniques".)

● (See "Nail-patella syndrome".)

● (See "The genodermatoses", section on 'Pachyonychia congenita'.)

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Causes of transverse grooving and onychomadesis include [9-12]:

● Local cutaneous disease (eg, dermatitis, paronychia)

● Drugs (eg, retinoids, chemotherapy agents)

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● Onychopapilloma (picture 19A-D)

● Glomus tumor (see 'Glomus tumor' below)

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● Keeping nails trimmed short

● Avoiding contact irritants

● Keeping nails dry (avoiding wet work)

● Avoiding all nail cosmetics

● Protecting hands from cold or windy weather

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● Yellow longitudinal bands of variable width

● Splinter hemorrhages of the proximal portion of the nail plate

● Prominent longitudinal ridging associated with woodworm-like cavities

● Increased transverse curvature of the nail plate

Treatment of onychopapilloma is surgical excision.

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● Age of patient (peak incidence in the fifth to seventh decade)

● Band of Brown/Black color; Breadth greater than 3 mm; Border (irregular/blurred)

● Change in the band (rapid increase in size or growth rate)

● Family history of melanoma

● Brown background hue

(See "Dermoscopy of nail pigmentations", section on 'Melanoma'.)

SKIN DISEASES WITH NAIL INVOLVEMENT

Clinical features that suggest nail psoriasis include [116]:

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nail plate (picture 6A).

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• Melanonychia extending to the nail folds (Hutchinson sign) (picture 41B)

Use of UpToDate is subject to the Subscription and License Agreement.

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Topic 13682 Version 22.0

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Graphic 85578 Version 2.0

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Anatomy of the nail apparatus

Graphic 85616 Version 2.0

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Nail growth: Direction of matrix cell differentiation and

Graphic 85617 Version 2.0

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Key questions for the patient with abnormal nails

What do you think is the cause of your nail problem?

Which nails were affected first?

Which nails are affected now?

How has your nail condition progressed?

Have you ever traumatized any of the involved nails?

What is your occupation?