Longitudinal erythronychia: Suggestions forevaluation and management

Nathaniel J. Jellinek, MDEast Greenwich, Rhode Island

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Longitudinal erythronychia is a frequent nail presentation with a limited differential diagnosis. This clinicalentity may be divided into cases that involve one (localized) or multiple (polydactylous) nails. The differentpresentations have distinct differential diagnoses and workups yet often share a common pathogenesis.Localized longitudinal erythronychia most commonly represents onychopapilloma, yet malignancies maypresent identically. Therefore biopsy may be required. Polydactylous longitudinal erythronychia usuallycoincides with a regional or systemic cause. Occasionally, it may herald an important underlying disease. Athorough understanding of the pathogenesis, clinical presentations, and possible diagnoses is necessary forsuccessful evaluation and management. ( J Am Acad Dermatol 2011;64:167.e1-11.)

ongitudinal erythronychia is a relatively com-

Abbreviations used:

LLE: localized longitudinal erythronychiaPLE: polydactylous longitudinal erythronychia

L mon finding. For purposes of evaluation andmanagement, longitudinal erythronychia can

be divided into two groups, depending uponwhether it involves one or multiple nails. Bothgroups, localized longitudinal erythronychia (LLE)and polydactylous longitudinal erythronychia (PLE),are associated with a limited differential diagnosis.LLE 1,2 may be caused by onychopapilloma, wart,warty dyskeratoma,3 glomus tumor, increased glo-mus bodies and other benign vascular prolifera-tions,4 a solitary lesion of lichen planus,5 Bowen’sdisease,1,2,6,7 melanoma in situ,8 and basal cell car-cinoma9 (Table I, Figs 1-13; Figs 1-4, 7-9, 12 areavailable online at http://www.eblue.org). PLE hasbeen associated most commonly with lichen pla-nus10 and Darier’s disease11 and occasionally withsystemic amyloidosis,10,12 hemiplegia,13 graft-versus-host disease,14,15 acantholytic epidermolysisbullosa,16 or with no association17 (Table II, Figs 14-17 [Figs 14-16 available online at www.eblue.org]).

de Berker, Perrin, and Baran2 have proposed apathogenesis that unifies most of the different

the Department of Dermatology, The Warren Alpert Medical

hool at Brown University and the Division of Dermatology,

niversity of Massachusetts Medical School.

ing sources: None.

licts of interest: None declared.

pted for publication October 8, 2009.

int requests: Nathaniel J. Jellinek, MD, 1672 South County

ail, Suite 101, East Greenwich, RI 02818. E-mail: winenut15@

hoo.com.

ished online August 16, 2010.

-9622/$36.00

10 by the American Academy of Dermatology, Inc.

0.1016/j.jaad.2009.10.047

diagnoses: a disease process limited to the distalmatrix (either inflammatory, neoplastic, space occu-pying, or nonspecific), results in the production of athinned longitudinal strip of ventral plate. The op-posing nail bed swells to fill the concavity in theventral plate. This longitudinal section of nail bed iscompressed by the normal nail plate at the margins.This lateral pressure leads to an engorged nail bedtrapped in the ventral groove, predisposed to splin-ter hemorrhages. The thinned plate is more trans-parent, further enhancing any underlying erythema(Fig 18). As this plate grows distally and reaches thefree margin, it is subject to trauma from activities ofdaily living, fragments more easily, resulting in distalchipping and onycholysis. The underlying hypo-nychium reacts with secondary inflammation, hy-perkeratosis, and occasional giant cell formation.

Clinically, cases show some or all of the following:longitudinal pink-red nail discoloration, often asso-ciated with or composed entirely of splinter hemor-rhages; a lucency in the distalmatrix (visualized as thelunula on the thumbs and index fingers in mostpatients); distal V-shaped chipping, splitting, andonycholysis of the nail plate at the free edge, withreactive distal nail bed and hyponychial hyperkera-tosis (see Figs 1-4 [available online at http://www.eblue.org) .These changes are appreciated upondirect examination but amplified with a magnifier,73 loupe, or dermatoscopy (see Figs 5-10 [Figs 7-9available online]). Dermatoscopy nicely highlights

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the splinter hemorrhages observed in most cases. Inaddition, some cases remain ambiguous until exam-ined by dermoscopy (see Fig 7; available online) orwhen examining the nail plate from several angles.

Given the disparate differential diagnoses, thediscussion of evaluation and management of LLE andPLE is addressed separately. As a general rule,

CAPSULE SUMMARY

d Longitudinal erythronychia is a commonnail presentation, with a limiteddifferential diagnosis.

d When limited to one digit (localizedlongitudinal erythronychia), the etiologyis usually neoplastic, withonychopapilloma being the mostcommon diagnosis. However, glomustumor, Bowen’s disease, melanoma insitu, and basal cell carcinoma have allbeen reported in this presentation.

d When identified on multiple digits(polydactylous longitudinalerythronychia), there is usually anunderlying regional or systemic cause.The most common include lichen planusand Darier’s disease. Other important,less common causes include systemicamyloidosis, graft-versus-host disease,and hemiplegia.

d In certain circumstances, biopsy isindicated. A partial nail plate avulsioncoupled with a longitudinal biopsy ofmatrix and bed is the recommendedtechnique for diagnosis in mostcircumstances.

however, the workup of LLEfocuses on the possibility of alocal neoplastic process,whereas that of PLE is cen-tered on a search for under-lying regional or systemiccauses.

LOCALIZEDLONGITUDINALERYTHRONYCHIA

LLE most commonly pre-sentson the thumbs inmiddleage,2 although any digit maybe affected. Involvement ofthe toenails is unusual (seeFig 10). Symptoms rangefrom none to pain and ten-derness. Patients commonlycomplainof catching theony-cholytic split free edge onclothes and fabrics.

The most common causeof LLE is onychopapil-loma1,2,18 (Table I, see Figs 1-10 [Figs 1-4 and 7-9 availableonline]). Onychopapilloma isan idiopathic benign tumor,and there is some debateabout its etiology and patho-genesis.2,9 It presents as aclassic band of LLE, often

with prominent distal hyperkeratosis that may resem-ble a distinct papule at the hyponychium, seeminglyemanating from the distal nail bed (see Fig 2 [availableonline]). Rarely, two bands may be present in onedigit1 (see Fig 9 [available online]). Dermatoscopicfindings occasionally highlight pink-red erythema ofthe lunula criss-crossed with minute telangiectases(see Figs 8 and 9 [available online]). I have alsoobserved a single case of onychopapilloma presentingas longitudinal leukonychia.19

Upon total or partial nail plate avulsion, anonychopapilloma shows variable, subtle pink ery-thematous swelling in the distal matrix, a longitudi-nal ridge in the nail bed, and distal bedhyperkeratosis (see Fig 10, B and C ).Histologically, it is characterized by variable and

nonspecific matrix findings but diagnostic nail bedchanges: there is prominent nail bed acanthosis andpapillomatosis, visualized most clearly in transversesections. The upper cell layers in the nail bedepithelium exhibit abundant eosinophilic cytoplasmclosely resembling that of the nail matrix keratoge-nous zone, thought to indicate matrix metaplasia of

the nail bed epithelium (Fig19). In the first series re-ported by Baran andPerrin,18 multinucleated cellswere observed in the hypo-nychium in 4 of 4 cases.However, a subsequent re-port noted multinucleatecells in only 2 of the 14 cases;it is likely that they representa nonspecific histologicsign.1,2

Other causes of LLE rangefrom the more common andbenign (wart, glomus tumor)to the rare and malignant. Ofthe malignancies, Bowen’sdisease is by far the most fre-quently observed. However,there is no published orwidely accepted algorithmfor the workup of LLE.

When a patient presentswith LLE, a detailed historyand physical examinationcan sometimes narrow thedifferential diagnosis. Anyhistory of a painful nail, par-ticularly exacerbated bycold, associated with pointtenderness and radiation ofpain up the finger, hand, and

arm, are keys to the diagnosis of glomus tumor. Inthis instance, imaging is unnecessary (classic pre-sentation by history and exam, with symptoms thatrequire intervention) and the patient can be taken tosurgery for exploration and excision. Occasionallythe presentation is more subtle and physical exam-ination findings and bedside maneuvers are nonspe-cific. When the clinical suspicion for glomus tumorremains high, radiologic imaging with magneticresonance or ultrasound, or even occasionally plainfilm x-ray, may be diagnostic.20-24

Subungual warts and warty dyskeratomas areusually more subtle clinical diagnoses. A history ofperiungual warts or immunosuppression may leadthe clinician to suspect human papillomavirus as thecause. Other unusual and variably nonspecific

Table I. Differential diagnosis of LLE

More commonOnychopapillomaGlomus tumorBowen’s diseaseWart

Less commonWartWarty dyskeratomaBenign vascular proliferation (increased glomus bodies,

cirsoid aneurysm)Lichen planus (isolated lesion)Nail melanomaBasal cell carcinoma

LLE, Localized longitudinal erythronychia.

Table II. Differential diagnosis of PLE

Lichen planusDarier diseasePrimary amyloidosisGraft-versus-host diseaseHemiplegiaAcantholytic epidermolysis bullosaIdiopathic

PLE, Polydactylous longitudinal erythronychia.

Fig 1. Onychopapilloma presenting as LLE and demon-strating V-shaped distal onycholysis.

Fig 2. Onychopapilloma presenting as LLE and demon-strating distal onycholysis and hyperkeratosis of the distalnail bed and hyponychium.

Fig 3. Onychopapilloma presenting as wedge-shaped LLEand demonstrating nail bed splinter hemorrhages anddistal onycholysis.

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diagnoses have been observed upon biopsy of LLE,including a range of benign vascular proliferations.As one possible confounder, a single suspectedoccurrence of onychopapilloma has been reported,simulated by a solitary lesion of nail lichen planus.5

In most cases, however, there are no history orphysical examination findings that point to a

particular diagnosis, or the finding of LLE is com-pletely incidental. In such instances, it is important toassess whether the lesion is stable or evolving. Anyevolving or new band of LLE usually indicates that abiopsy should be considered. Any stable band ofLLE, documented by a good historian or repeatedexaminations without change, must at the very leastbe followed up as a lesion of indeterminate signif-icance, as one would follow up an unusual pig-mented lesion presumed, but unproven, to be

Fig 4. Onychopapilloma presenting as a fine band of LLE.

Fig 5. Onychopapilloma presenting as LLE with a tear-drop-shaped origin in the lunula, and distal splinterhemorrhages (A). B, Direct dermatoscopy using water-soluble jelly shows splinter hemorrhages and a pinklucency in the lunula. Fig 6. Onychopapilloma presenting as LLE (A and B). C,

Direct dermatoscopy using water-soluble jelly shows moresubtle clinical features, splinter hemorrhages and V-shaped distal onycholysis.

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Fig 7. A, Poorly defined band of LLE mimicking longitudi-nal melanonychia. B, Dermatoscopy highlights erythrony-chia and splinter hemorrhages.Histopathologic examinationshowed onychopapilloma.

Fig 8. A and B, Onychopapilloma presenting as LLE. C,Marked telangiectasia on lunula highlighted bydermatoscopy.

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benign. In my practice, patients with such lesions ofLLE are often followed up at regular intervals, aretold to watch the lesion for any change, and to returnsooner if changes occur.

One proposed algorithm for this evaluation isfound as Fig 20.

POLYDACTYLOUS LONGITUDINALERYTHRONYCHIA

PLE commonly presents in adulthood, if onlybecause the associated diseases are unusual in chil-dren. Like LLE, PLE may be an incidental finding orpresenting complaint (see Figs 14-17 [Figs 14-16available online]). In some circumstances, such asunderlying hemiplegia, multiple myeloma, orDarier’s disease, the nail manifestation is an obviousassociated finding of another disease. In certaincases, however, the nail changes constitute the soleclinical manifestation, without signs of an underlyingdisease. In these circumstances, a workup for

Fig 9. A, Onychopapilloma presenting with two bands ofLLE and marked unilateral onycholysis. B, Dermatoscopydemonstrates marked funnel-shaped red dyschromia andtelangiectasia on the lunula, a feature not apparent ondirect examination or with a 73 loupe.

Fig 10. A, An unusual case of an onychopapillomapresenting as LLE on the great toe with a thin band ofdistal true leukonychia. B and C, Trap-door avulsionshows red discoloration of the distal matrix and a subtlelongitudinal ridge of nail bed.

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etiology is advisable. In Darier’s disease, which canpresent rarely as isolated nail disease, there is thepossibility of transmitting the autosomal dominantgenodermatosis to subsequent generations who maydevelop more generalized signs of disease. PLE inlichen planus may predate other cutaneous, muco-sal, hair, or progressive scarring nail disease. PLEmay also trigger a workup for hematologic malig-nancy if the diagnosis of amyloidosis is made.

One proposed algorithm for this evaluation isfound as Fig 21.

BIOPSY TECHNIQUES FORLONGITUDINAL ERYTHRONYCHIA

Because the relevant pathology involves the distalmatrix and commonly extends onto the nail bed, alongitudinal biopsy is perfectly suited.25,26 A longi-tudinal fusiform biopsy is performed, starting at themid to distal matrix, extending through the bed to the

Fig 11. Glomus tumor presenting as LLE, with distal nailplate splitting and onycholysis.

Fig 12. Amelanotic melanoma in situ presenting as LLE.Note prominent distal onycholysis.

Fig 13. A, Preoperative LLE, demonstrating a narrow red band, distal onycholysis, and splinterhemorrhages. B, After a longitudinal partial nail plate avulsion; band is visualized with splinterhemorrhages. C, After a narrow longitudinal excision. D, Short-term (10-week) follow-up,without obvious residual erythronychia.

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Fig 14. Systemic amyloidosis with PLE, splinter hemor-rhages, onychorrhexis, onychoschizia, hyperkeratosis ofthe cuticle, and a flaccid vesicle on dorsal aspect of onefinger.

Fig 15. Nails with classic Darier’s disease, with ‘‘candy-cane’’ PLE, distal onycholysis, splinter hemorrhages, andhyperkeratosis of hyponychium distal to red bands.

Fig 16. Nail lichen planus, with PLE, nail plate thinning,onychorrhexis, and mild onycholysis.

Fig 17. Graft-versus-host disease with PLE, distal ony-cholysis, nail plate atrophy, and onychorrhexis. (Courtesyof Bianca Maria Piraccini, MD.)

Fig 18. Pathogenesis of longitudinal erythronychia, asproposed by de Berker. Distal matrix disease processresults in production of a thinned ventral nail plate. Nailbed swells to fill the concave nail plate, is pinched by thesides. causing splinter hemorrhages, and cracks and splitsdistally with activities of daily living (ADL). Secondaryreactive hyperkeratosis of distal nail bed and hyponych-ium results.

Fig 19. Nail bed biopsy in setting of onychopapilloma.Epithelium shows maturation with development of kera-togenous zone, indicative of matrix metaplasia of nail bed.This is a histologic hallmark of onychopapilloma.

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Fig 20. Proposed algorithm for evaluation of LLE.

Fig 21. Proposed algorithm for evaluation of PLE.

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hyponychium, and can be executed after one ofseveral nail avulsion techniques2,26,27 (see Figs 10and 13) This biopsy technique may or may notrequire suturing to repair the distal matrix. The distalhalf of the matrix produces only the ventral 20% ofthe nail plate28 and may not result in a split nail if leftto heal by second intention. Nevertheless, the like-lihood of this outcome can be minimized throughwide undermining and side-to-side suturing of thematrix with absorbable suture, or by performing oneof several local matrix sliding flaps.29 The nail bedmay heal by second intention with little risk ofpostoperative dystrophy.

The longitudinal specimen is excised at the levelof periosteum and sectioned according to the spe-cific instructions of the surgeon. Longitudinal pro-cessing demonstrates contiguous pathology in thissetting and in most cases is preferred by the pathol-ogists with whom I work. Transverse sectioning,however, better demonstrates the marked papillo-matosis of the nail bed in onychopapilloma.

For those uncomfortable with performing thelongitudinal biopsy, a 3-mm punch biopsy may beutilized—of the distal matrix and involved bed—toobtain diagnostic specimens. The defects left bythese round biopsies neither facilitate nor requiresuturing. In the setting of an onychopapilloma,however, it is important to note that excision of thedistal hyperkeratosis in isolation will result in promptrecurrence of the lesion.1,2 In other instances, asingle bed punch biopsy may miss the diagnosisaltogether.2 This fact underscores the indication forlongitudinal biopsy.

For those cases with a high suspicion of glomustumor, the surgeon may proceed with one of severalacceptable techniques.30-32 These are generally di-vided between those that require partial or total plateavulsion and dissection through the nail epithelia(transungual) and those that are executed withoutdisruptionof thenail plateor epithelia (subepithelial).

As in cases of other nail tumors, complete excisionis preferred at the time of biopsy.33,34 And while thisis occasionally impossible because of lesion width,tumor biology,8 or poor demarcation, when success-ful, the most common diagnoses (onychopapilloma,glomus tumor) are both diagnosed and treated in thesame procedure.2

I thank Antonio Cruz, MD, for flowchart creation andJeffrey D. Bernhard, MD, for editing assistance.

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