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Longitudinal melanonychia

Longitudinal melanonychia
Author:
Phoebe Rich, MD
Section Editor:
Erik Stratman, MD
Deputy Editor:
Rosamaria Corona, MD, DSc
Literature review current through: Jan 2024.
This topic last updated: May 16, 2022.

INTRODUCTION — Longitudinal melanonychia, also called "melanonychia striata," describes a pigmented, brown to black, longitudinal streak of the nail plate due to increased activity of melanocytes or melanocytic hyperplasia in the nail matrix, with increased melanin deposition in the nail plate [1,2]. The most common type of longitudinal melanonychia due to melanocytic activation is physiologic melanonychia, which typically presents in individuals with darkly pigmented skin as multiple bands affecting multiple nails. Other causes of physiologic melanocytic activation include pregnancy, chronic local trauma or inflammation, systemic conditions, and drugs (table 1). Nail lentigo, nail melanocytic nevus, and nail melanoma are causes of longitudinal melanonychia due to melanocytic hyperplasia.

This topic will discuss the pathophysiology, clinical presentation, diagnosis, differential diagnosis, and management of longitudinal melanonychia. Other nail disorders are discussed separately. The dermoscopic examination of nail pigmentations, nail biopsy and nail surgery techniques, and surgical management of nail melanoma are also discussed separately.

(See "Overview of nail disorders".)

(See "Dermoscopy of nail pigmentations".)

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

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

EPIDEMIOLOGY — The prevalence of longitudinal melanonychia ranges from approximately 1 percent in populations with lightly pigmented skin to more than 70 percent in populations with darkly pigmented skin [3-5]. In a cross-sectional study of nearly 2500 Chinese outpatients, longitudinal melanonychia was noted in 0.6 percent of patients aged 20 to 29 years and 1.7 percent of those aged ≥50 years [4]. In a Japanese population, the prevalence of longitudinal melanonychia was 11 percent. In another study of 482 Brazilian patients with skin phototypes IV to VI, longitudinal melanonychia was present in 58 patients (12 percent).

PATHOPHYSIOLOGY — The number of melanocytes in the nail unit is low compared with normal skin. Melanonychia usually originates in the distal nail matrix because melanocytes located in the proximal matrix are dormant, while approximately one-half of those located in the distal matrix have the potential to become activated. Longitudinal melanonychia results from either activation of previously dormant melanocytes or by proliferation (hyperplasia) of melanocytes in the distal nail matrix [1,6-8].

Melanocytic activation occurs physiologically in individuals with darkly pigmented skin; hormonally driven during pregnancy; or in response to a variety of stimuli, including inflammation or infection of the nail unit (eg, nail psoriasis, nail lichen planus, onychomycosis), exposure to certain drugs, due to minor repetitive trauma (frictional melanonychia), or in the setting of systemic disorders (eg, Addison disease, Laugier-Hunziker syndrome, Peutz-Jeghers syndrome) (table 1). Melanocytic activation (also termed "functional melanonychia," "melanocytic stimulation," or "hypermelanosis") causes an increase in melanic pigmentation of the nail matrix epithelium without a concurrent increase in the number of melanocytes [7].

Melanocytic hyperplasia (matrix melanocyte proliferation) is a less frequent cause of longitudinal melanonychia and occurs in only three situations: nevus, lentigo, and melanoma. Benign melanocytic hyperplasia results in lentigines and nevi of the nail matrix. Malignant melanocytic hyperplasia includes in situ and invasive melanoma of the nail apparatus.

CLINICAL PRESENTATION

General features — Longitudinal melanonychia presents with one or more longitudinal, pigmented bands running from the proximal nail fold to the distal free edge of the nail plate (picture 1) [2]. Occasionally, the band can be wide or involve the entire nail plate (picture 2 and picture 3). The color varies, from gray to light or dark brown to black. It occurs more frequently on the thumbnail or the big toenail but may involve other nails or multiple nails (picture 4 and picture 5A-B).

Melanonychia due to melanocytic activation — Melanonychia due to melanocytic activation, also called "functional melanonychia," is the most common type of melanonychia seen in both adults and children. It presents with regular, homogeneous, thin bands of brown color involving one or more nails.

Longitudinal melanonychia due to melanocytic activation is common in individuals with darkly pigmented skin (picture 5A-B). The number and width of the bands increases with age [7]. Of note, a periungual hyperpigmentation (pseudo-Hutchinson sign) may be present [9]. (See 'Hutchinson sign and pseudo-Hutchinson sign' below.)

Longitudinal melanonychia can be seen in patients with chronic nail trauma (eg, nail biting, onychotillomania), onychomycosis, and inflammatory skin diseases involving the nails (eg, psoriasis, lichen planus (picture 6)) as well as in patients with a number of systemic diseases (eg, Addison disease, Cushing syndrome, Peutz-Jeghers syndrome) (table 1). Exposure to certain drugs and, in particular, to chemotherapeutic agents (table 2) may also cause melanonychia due to melanocytic activation [10]. In all these situations, multiple nails are typically involved.

Melanonychia due to melanocytic hyperplasia

Nail lentigo and nevus — Nail lentigines and nevi present as single-digit bands, usually <5 mm in width, and light brown to dark brown or black in color (picture 7A-B) [7,11]. Nail lentigines are seen more often than nevi in adults, while nail nevi are the most common cause of longitudinal melanonychia in children [12,13]. In a review of 40 patients younger than 16 years with longitudinal or total melanonychia, the histopathologic diagnosis was nevus in 19 patients, lentigo in 12 patients, and melanocytic activation in 9 patients [12].

Nevi usually occur on the fingernails, with the thumbnail being most commonly affected. In some cases, the pigmented band may be visible through the thin cuticle, resulting in an apparent periungual pigmentation (pseudo-Hutchinson sign (picture 8)). (See 'Hutchinson sign and pseudo-Hutchinson sign' below.)

Nail melanoma — Nail unit melanoma, also called subungual melanoma, is rare, accounting for 1 to 3 percent of all melanomas occurring in populations with lightly pigmented skin and 15 to 30 percent of melanomas occurring in populations with darkly pigmented skin [14]. In approximately two-thirds of cases, nail melanoma presents as a brown to black, longitudinal band involving a single nail [15]. The band is generally wider than 3 mm; shows proximal widening and irregular or blurred, lateral borders; and may be associated with nail plate dystrophy (picture 9A-C) [2]. A periungual pigmentation (Hutchinson sign) may be present and supports the clinical diagnosis of melanoma (picture 9D and picture 9C). (See 'Hutchinson sign and pseudo-Hutchinson sign' below.)

Early nail melanoma may appear as a narrow melanonychia band with subtle variegation of color that is difficult to differentiate clinically and dermoscopically from a benign lentigo or nevus [16]. (See 'Dermoscopic examination (onychoscopy)' below.)

In approximately one-third of cases, nail melanoma is hypomelanotic or amelanotic and presents as a nail bed mass or nail plate abnormality (picture 10).

Hutchinson sign and pseudo-Hutchinson sign — The Hutchinson sign is the periungual spread of pigment into the proximal or lateral nail folds [9,17]. It has been historically considered a pathognomonic sign of subungual melanoma (picture 11). However, a periungual pigmentation may be seen in approximately one-third of cases of nail lentigines or nevi and in other benign conditions, such as melanonychia in individuals with darkly pigmented skin (picture 5A), melanonychia associated with Laugier-Hunziker syndrome or Peutz-Jeghers syndrome, and melanonychia associated with the use of certain medications (table 3) [18,19]. In these circumstances, it is referred to as "pseudo-Hutchinson sign" [7,9,17]. Of note, in some cases and especially in children with melanocytic nevi of the nail matrix, the cuticle pigmentation is illusory due to the pigmented band that shows through the transparency of the cuticle (picture 8).

PATIENT EVALUATION AND DIAGNOSIS — Longitudinal melanonychia involving multiple nails is in most cases diagnosed clinically, based on physical examination and history. In contrast, the diagnosis of longitudinal melanonychia involving a single nail may be challenging, especially when the onset is in adulthood or when the patient reports a change (eg, widening, darkening) in a previously stable band. In these cases, a nail matrix biopsy is required for accurate diagnosis.

Dermoscopy, which allows for the visualization of morphologic features that are not visible to the naked eye, may help the clinician in the decision on whether to perform a nail matrix biopsy. (See 'Dermoscopic examination (onychoscopy)' below and 'When to biopsy' below.)

The approach to the clinical diagnosis and management of longitudinal melanonychia involving one or multiple nails is illustrated in the algorithm (algorithm 1).

Clinical examination and history — The diagnosis of longitudinal melanonychia starts with a careful examination of all nails to assess [2]:

Number of involved nails

Color and width of the band(s)

Color homogeneity

Presence of signs of trauma

Presence of periungual pigmentation

Presence of nail dystrophy or subungual nail lesions

Information on onset, progression, and possible triggers of melanonychia should be obtained from the patient. Medical and drug history, history of digital trauma, and history of exposure to exogenous substances (eg, tar, tobacco, dyes) can provide clues to the etiology of melanonychia [8].

Physiologic longitudinal melanonychia (associated with darkly pigmented skin or pregnancy); melanonychia associated with signs of local trauma; and melanonychia associated with concomitant systemic diseases, dermatologic conditions (eg, fungal melanonychia), or drugs are diagnosed by clinical examination and history in most cases and do not require further examination (algorithm 1).

In contrast, unexplained longitudinal melanonychia involving a single nail, especially if presenting in an adult patient, can be a diagnostic challenge. The clinical features that raise suspicion of melanoma are reviewed below. (See 'Clinical features suspicious for nail melanoma' below and 'When to biopsy' below.)

Clinical features suspicious for nail melanoma — Clinical features that may suggest early nail melanoma and warrant a biopsy of the nail matrix in patients with longitudinal melanonychia include (algorithm 1) [1,20,21]:

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 11) with variegated pigmentation or proximal widening (triangular shape) (picture 9A, 9D)

Pre-existing longitudinal melanonychia that becomes darker or wider or demonstrates blurred, lateral borders

Longitudinal melanonychia associated with nail plate fissuring, splitting, or dystrophy (picture 9B)

Melanonychia extending to the nail folds (Hutchinson sign (picture 9C))

The ABCDEF mnemonic may also be helpful in recalling clinical features that raise suspicion of melanoma [22]:

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

Dermoscopic examination (onychoscopy) — Nail plate dermoscopy (onychoscopy) may help the clinician with at least minimal training in dermoscopy in recognizing benign lesions that do not require further histologic examination from lesions that require biopsy or regular follow-up. However, clinicians should be cognizant that dermoscopy is not a substitute for histopathologic diagnosis and that it is important to maintain a low threshold of suspicion for performing a biopsy for histopathologic examination:

Melanonychia due to melanocytic activation appears as a gray background with thin, gray, regular, parallel lines (picture 12). In melanocyte activation caused by chronic trauma, tiny, dark red to brown spots corresponding to extravasation of blood may also be seen [1]. (See "Dermoscopy of nail pigmentations", section on 'Benign lesions'.)

Nail lentigines generally appear as homogeneous, longitudinal, thin, gray or brown lines on a gray or light brown background; nail lentigines are more common in adults than in children.

Nail nevi, which are more common in children than in adults, appear as a band of regular lines of light brown to black color on a brown background (picture 13 and picture 14) [16,23].

Dermoscopic features associated with nail melanoma include (picture 15A-B) [16,23,24]:

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)

It is important to note that irregular lines that would be considered suspicious for melanoma in an adult can often be seen in children with nail matrix nevi (picture 16 and picture 17). (See "Dermoscopy of nail pigmentations", section on 'Benign lesions' and "Dermoscopy of nail pigmentations", section on 'Melanonychia in children'.)

The dermoscopic evaluation and differential diagnosis of longitudinal melanonychia are discussed in greater detail separately (algorithm 2). (See "Dermoscopy of nail pigmentations", section on 'Differential diagnosis of nail pigmentations'.)

When to biopsy — In most patients presenting with stable longitudinal melanonychia involving multiple nails, a biopsy is not required to confirm the clinical diagnosis (algorithm 1). (See 'Melanonychia due to melanocytic activation' above.)

In contrast, a clinician's threshold for biopsy should be low when examining a patient with a single digit longitudinal melanonychia, especially if (algorithm 1):

Onset occurred in adulthood

Lesion is located on the thumb, index finger, or big toenail

Lesion shows clinical features that suggest melanoma (see 'Clinical features suspicious for nail melanoma' above)

Lesion shows dermoscopic features suspicious for melanoma (see 'Dermoscopic examination (onychoscopy)' above)

Lesion is rapidly enlarging

In children, longitudinal melanonychia is in most cases due to a nevus of the nail matrix, while nail melanoma is exceedingly rare, with only a few cases reported in the literature [12,25,26]. Thus, some experts suggest to avoid nail matrix biopsy in children where possible, with the exception of cases in which the band enlarges and/or darkens rapidly or involves the whole nail [27].

The techniques for performing a nail matrix biopsy are described elsewhere. (See "Nail biopsy: Indications and techniques".)

Histopathologic diagnosis — Histopathologic examination is the gold standard for the diagnosis of longitudinal melanonychia. However, differentiating early melanoma of the nail matrix from benign melanocytic lesions may be challenging, even for the expert pathologist, as some features (eg, cellular atypia, pagetoid spread, nest formation) can be seen in lentigines, nevi, and in melanoma in situ:

Melanocytic activation – Melanocytic activation is characterized by nonspecific, melanic pigmentation of the matrix epithelium without an increase in the number of melanocytes. The Fontana-Masson stain is helpful when the pigmentation is barely visible. There are some melanocytes with pigmented dendrites in the suprabasal layer of the proximal matrix and basal layer of the distal matrix and pigmented keratinocytes; a few melanophages can be seen in the superficial dermis [28,29]. Cytologic atypia is absent.

Lentigo – Lentigo is characterized by a slight to moderate increase in the number of matrix melanocytes (10 to 31 per mm) that are individually aligned in the basal layer without confluence [28,29]. Cytologic atypia is absent or mild; pagetoid spread is rare or focal. The pigmentation is usually limited to the lower third of the nail epithelium but can be observed throughout its full thickness. Fontana-Masson staining shows fine granularity of the melanin pigment.

Nevus – On microscopic examination, a melanocytic nevus of the nail matrix is characterized by hyperplasia of melanocytes with nest formation. A lentiginous pattern can be seen at the center of the lesion, along with a suprabasal pagetoid spread tendency, mild nuclear pleomorphism, and nail plate involvement [29]. Periungual pigmentation can also be observed.

Melanoma – Melanoma in situ is characterized by an increased number of melanocytes in the basal cell layer (39 to 136 per mm), with a predominance of single melanocytes and a few nests [20,28]. Nuclear atypia and pagetoid spreading are present. Atypical melanocytes have large, hyperchromatic, pleomorphic nuclei; prominent nucleoli; increased mitoses; and long, branching dendrites [6]. A dermal lymphoid infiltrate is present. The detection of melanocytes in the nail plate corresponding to the matrix keratogenous zone is an important finding for malignancy [29].

Invasive melanoma is characterized by atypical melanocytes invading the dermis. The commonest histogenic subtype is acral lentiginous, followed by nodular and desmoplastic [28]. (See "Pathologic characteristics of melanoma", section on 'Acral lentiginous melanoma'.)

DIFFERENTIAL DIAGNOSIS — Several nonmelanic pigmentations of the nail plate may be confused with longitudinal melanonychia. These include (see "Overview of nail disorders"):

Subungual hematoma (picture 18)

Exogenous discoloration

Splinter hemorrhage (picture 19A-C)

Fungal melanonychia (picture 20)

Longitudinal erythronychia

Onychopapilloma (picture 21)

Pigmented onychomatricoma

MANAGEMENT — Longitudinal melanonychia is in most cases a benign condition. A wait-and-see approach with periodic clinical and, if available, dermoscopic monitoring may be appropriate in adults and children when clinical and dermoscopic features indicate a low risk of melanoma [2,13,30]. (See 'Monitoring' below.)

Melanonychia should be excised when worrisome features suspicious for melanoma (eg, wide band, presence of the Hutchinson sign, or irregular dermoscopic features) are noted. Additional surgery is generally needed if the initial biopsy shows melanoma. Wide surgical excision of the entire nail apparatus with margin control is a conservative option for subungual melanoma in situ; digit amputation is the traditional surgical approach for invasive melanoma [31,32]. (See "Surgical management of primary cutaneous melanoma or melanoma at other unusual sites", section on 'Subungual'.)

There is very limited evidence to guide the management of biopsied lesions displaying atypical melanocytic hyperplasia, especially in children [33,34]. Some experts suggest that adult patients with melanocytic hyperplasia with moderate to severe melanocytic atypia on biopsy should undergo complete resection with margin control [33]. In children, given the extreme rarity of subungual melanoma, clinical and dermoscopic surveillance for enlargement or changes may be reasonable [34].

MONITORING — There is no consensus on the frequency and modalities of follow-up for pigmented nail bands. Some experts recommend clinical and dermoscopic examination every six months for lesions that have subtle, irregular features that do not require immediate biopsy [1,16]. Baseline medical photography of the nail in question, including dermoscopy photos when possible, can be very helpful when clinically monitoring over time the patient with longitudinal melanonychia.

SUMMARY AND RECOMMENDATIONS

Definition and pathogenesis – Longitudinal melanonychia, also called "melanonychia striata," describes a pigmented, brown to black, longitudinal streak of the nail plate resulting from increased melanin production and deposition within the nail plate. This may result from melanocytic activation, due to multiple causes, or from benign or malignant hyperplasia of melanocytes in the nail matrix (table 1). (See 'Introduction' above and 'Pathophysiology' above.)

Clinical presentation – Longitudinal melanonychia presents with one or more pigmented bands running from the proximal nail fold to distal margin of the nail plate (picture 1):

Melanonychia due to melanocytic activation – Also called "functional melanonychia," this is the most common type of melanonychia seen in adult patients. It includes melanonychia frequently seen in patients with darkly pigmented skin (picture 12); melanonychia due to repeated trauma (frictional melanonychia); and melanonychia associated with skin diseases, systemic diseases, or exposure to drugs. (See 'Melanonychia due to melanocytic activation' above.)

Melanonychia due to melanocytic hyperplasia – This type of melanonychia includes benign lesions (lentigines and nevi of the nail matrix) and nail melanoma:

-Lentigines/nevi – Nail lentigines and nevi present as single-digit bands, <5 mm in width, and light brown to dark brown or black in color (picture 7A-B). Lentigines are seen more often than nevi in adults, while nail nevi are the most common cause of longitudinal melanonychia in children. (See 'Nail lentigo and nevus' above.)

-Nail melanoma – Nail melanoma presents in most cases as a brown to black, longitudinal band involving a single nail, usually wider than 3 mm, that shows proximal widening and irregular or blurred, lateral borders (picture 9B and picture 9C). A periungual pigmentation (Hutchinson sign) may be present and supports the clinical diagnosis of melanoma (picture 9C and picture 9D and picture 11). However, early melanoma can be clinically indistinguishable from lentigo or nevus. (See 'Nail melanoma' above and 'Hutchinson sign and pseudo-Hutchinson sign' above.)

Diagnosis – The diagnosis of longitudinal melanonychia is based on clinical and dermoscopic features in many cases (see 'Patient evaluation and diagnosis' above and "Dermoscopy of nail pigmentations"):

When to biopsy – A nail matrix biopsy for histopathologic evaluation may be required in the following circumstances (algorithm 1):

-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 11) with variegated pigmentation or proximal widening (triangular shape)

-Pre-existing longitudinal melanonychia that becomes darker or wider or demonstrates blurred, lateral borders

-Longitudinal melanonychia associated with nail plate fissuring, splitting, or dystrophy (picture 9B)

-Melanonychia extending to the nail folds (Hutchinson sign (picture 9C))

Management – Longitudinal melanonychia is in most cases a benign condition. A wait-and-see approach with periodic clinical and dermoscopic monitoring may be appropriate in adults and children when clinical and dermoscopic features indicate a low risk of melanoma. Surgical excision of the entire lesion should be performed when there are worrisome features suspicious for melanoma. (See 'Management' above.)

The management of subungual melanoma is discussed separately. (See "Surgical management of primary cutaneous melanoma or melanoma at other unusual sites", section on 'Subungual'.)

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