Longitudinal erythronychia is a relatively common

Transcription

1 Longitudinal erythronychia: Suggestions for evaluation and management Nathaniel J. Jellinek, MD East Greenwich, Rhode Island Longitudinal erythronychia is a frequent nail presentation with a limited differential diagnosis. This clinical entity may be divided into cases that involve one (localized) or multiple (polydactylous) nails. The different presentations have distinct differential diagnoses and workups yet often share a common pathogenesis. Localized longitudinal erythronychia most commonly represents onychopapilloma, yet malignancies may present identically. Therefore biopsy may be required. Polydactylous longitudinal erythronychia usually coincides with a regional or systemic cause. Occasionally, it may herald an important underlying disease. A thorough understanding of the pathogenesis, clinical presentations, and possible diagnoses is necessary for successful evaluation and management. ( J Am Acad Dermatol 2011;64:167.e1-11.) Longitudinal erythronychia is a relatively common finding. For purposes of evaluation and management, longitudinal erythronychia can be divided into two groups, depending upon whether it involves one or multiple nails. Both groups, 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 glomus bodies and other benign vascular proliferations, 4 a solitary lesion of lichen planus, 5 Bowen s disease, 1,2,6,7 melanoma in situ, 8 and basal cell carcinoma 9 (Table I, Figs 1-13; Figs 1-4, 7-9, 12 are available online at PLE has been associated most commonly with lichen planus 10 and Darier s disease 11 and occasionally with systemic amyloidosis, 10,12 hemiplegia, 13 graftversus-host disease, 14,15 acantholytic epidermolysis bullosa, 16 or with no association 17 (Table II, Figs [Figs available online at de Berker, Perrin, and Baran 2 have proposed a pathogenesis that unifies most of the different From the Department of Dermatology, The Warren Alpert Medical School at Brown University and the Division of Dermatology, University of Massachusetts Medical School. Funding sources: None. Conflicts of interest: None declared. Accepted for publication October 8, Reprint requests: Nathaniel J. Jellinek, MD, 1672 South County Trail, Suite 101, East Greenwich, RI winenut15@ yahoo.com. Published online August 16, /$36.00 ª 2010 by the American Academy of Dermatology, Inc. doi: /j.jaad Abbreviations used: LLE: localized longitudinal erythronychia PLE: polydactylous longitudinal erythronychia diagnoses: a disease process limited to the distal matrix (either inflammatory, neoplastic, space occupying, or nonspecific), results in the production of a thinned longitudinal strip of ventral plate. The opposing nail bed swells to fill the concavity in the ventral plate. This longitudinal section of nail bed is compressed by the normal nail plate at the margins. This lateral pressure leads to an engorged nail bed trapped in the ventral groove, predisposed to splinter hemorrhages. The thinned plate is more transparent, further enhancing any underlying erythema (Fig 18). As this plate grows distally and reaches the free margin, it is subject to trauma from activities of daily living, fragments more easily, resulting in distal chipping and onycholysis. The underlying hyponychium reacts with secondary inflammation, hyperkeratosis, and occasional giant cell formation. Clinically, cases show some or all of the following: longitudinal pink-red nail discoloration, often associated with or composed entirely of splinter hemorrhages; a lucency in the distal matrix (visualized as the lunula on the thumbs and index fingers in most patients); distal V-shaped chipping, splitting, and onycholysis of the nail plate at the free edge, with reactive distal nail bed and hyponychial hyperkeratosis (see Figs 1-4 [available online at eblue.org).these changes are appreciated upon direct examination but amplified with a magnifier, 73 loupe, or dermatoscopy (see Figs 5-10 [Figs 7-9 available online]). Dermatoscopy nicely highlights 167.e1

2 167.e2 Jellinek JAM ACAD DERMATOL JANUARY 2011 the splinter hemorrhages observed in most cases. In addition, some cases remain ambiguous until examined by dermoscopy (see Fig 7; available online) or when examining the nail plate from several angles. Given the disparate differential diagnoses, the discussion of evaluation and management of LLE and PLE is addressed separately. As a general rule, however, the workup of LLE focuses on the possibility of a local neoplastic process, whereas that of PLE is centered on a search for underlying regional or systemic causes. CAPSULE SUMMARY LOCALIZED LONGITUDINAL ERYTHRONYCHIA LLE most commonly presents on the thumbs in middle age, 2 although any digit may be affected. Involvement of the toenails is unusual (see Fig 10). Symptoms range from none to pain and tenderness. Patients commonly complain of catching the onycholytic split free edge on clothes and fabrics. The most common cause of LLE is onychopapilloma 1,2,18 (Table I, seefigs 1-10 [Figs 1-4 and 7-9 available online]). Onychopapilloma is an idiopathic benign tumor, and there is some debate about its etiology and pathogenesis. 2,9 It presents as a classic band of LLE, often with prominent distal hyperkeratosis that may resemble a distinct papule at the hyponychium, seemingly emanating from the distal nail bed (see Fig 2 [available online]). Rarely, two bands may be present in one digit 1 (see Fig 9 [available online]). Dermatoscopic findings occasionally highlight pink-red erythema of the lunula criss-crossed with minute telangiectases (see Figs 8 and 9 [available online]). I have also observed a single case of onychopapilloma presenting as longitudinal leukonychia. 19 Upon total or partial nail plate avulsion, an onychopapilloma shows variable, subtle pink erythematous swelling in the distal matrix, a longitudinal ridge in the nail bed, and distal bed hyperkeratosis (see Fig 10, B and C ). Histologically, it is characterized by variable and d d d d Longitudinal erythronychia is a common nail presentation, with a limited differential diagnosis. When limited to one digit (localized longitudinal erythronychia), the etiology is usually neoplastic, with onychopapilloma being the most common diagnosis. However, glomus tumor, Bowen s disease, melanoma in situ, and basal cell carcinoma have all been reported in this presentation. When identified on multiple digits (polydactylous longitudinal erythronychia), there is usually an underlying regional or systemic cause. The most common include lichen planus and Darier s disease. Other important, less common causes include systemic amyloidosis, graft-versus-host disease, and hemiplegia. In certain circumstances, biopsy is indicated. A partial nail plate avulsion coupled with a longitudinal biopsy of matrix and bed is the recommended technique for diagnosis in most circumstances. nonspecific matrix findings but diagnostic nail bed changes: there is prominent nail bed acanthosis and papillomatosis, visualized most clearly in transverse sections. The upper cell layers in the nail bed epithelium exhibit abundant eosinophilic cytoplasm closely resembling that of the nail matrix keratogenous zone, thought to indicate matrix metaplasia of the nail bed epithelium (Fig 19). In the first series reported by Baran and Perrin, 18 multinucleated cells were observed in the hyponychium in 4 of 4 cases. However, a subsequent report noted multinucleate cells in only 2 of the 14 cases; it is likely that they represent a nonspecific histologic sign. 1,2 Other causes of LLE range from the more common and benign (wart, glomus tumor) to the rare and malignant. Of the malignancies, Bowen s disease is by far the most frequently observed. However, there is no published or widely accepted algorithm for the workup of LLE. When a patient presents with LLE, a detailed history and physical examination can sometimes narrow the differential diagnosis. Any history of a painful nail, particularly exacerbated by cold, associated with point tenderness and radiation of pain up the finger, hand, and arm, are keys to the diagnosis of glomus tumor. In this instance, imaging is unnecessary (classic presentation by history and exam, with symptoms that require intervention) and the patient can be taken to surgery for exploration and excision. Occasionally the presentation is more subtle and physical examination findings and bedside maneuvers are nonspecific. When the clinical suspicion for glomus tumor remains high, radiologic imaging with magnetic resonance or ultrasound, or even occasionally plain film x-ray, may be diagnostic Subungual warts and warty dyskeratomas are usually more subtle clinical diagnoses. A history of periungual warts or immunosuppression may lead the clinician to suspect human papillomavirus as the cause. Other unusual and variably nonspecific

3 JAM ACAD DERMATOL VOLUME 64, NUMBER 1 Jellinek 167.e3 Table I. Differential diagnosis of LLE More common Onychopapilloma Glomus tumor Bowen s disease Wart Less common Wart Warty dyskeratoma Benign vascular proliferation (increased glomus bodies, cirsoid aneurysm) Lichen planus (isolated lesion) Nail melanoma Basal cell carcinoma LLE, Localized longitudinal erythronychia. Table II. Differential diagnosis of PLE Lichen planus Darier disease Primary amyloidosis Graft-versus-host disease Hemiplegia Acantholytic epidermolysis bullosa Idiopathic Fig 2. Onychopapilloma presenting as LLE and demonstrating distal onycholysis and hyperkeratosis of the distal nail bed and hyponychium. PLE, Polydactylous longitudinal erythronychia. Fig 3. Onychopapilloma presenting as wedge-shaped LLE and demonstrating nail bed splinter hemorrhages and distal onycholysis. Fig 1. Onychopapilloma presenting as LLE and demonstrating V-shaped distal onycholysis. diagnoses have been observed upon biopsy of LLE, including a range of benign vascular proliferations. As one possible confounder, a single suspected occurrence of onychopapilloma has been reported, simulated by a solitary lesion of nail lichen planus. 5 In most cases, however, there are no history or physical examination findings that point to a particular diagnosis, or the finding of LLE is completely incidental. In such instances, it is important to assess whether the lesion is stable or evolving. Any evolving or new band of LLE usually indicates that a biopsy should be considered. Any stable band of LLE, documented by a good historian or repeated examinations without change, must at the very least be followed up as a lesion of indeterminate significance, as one would follow up an unusual pigmented lesion presumed, but unproven, to be

4 167.e4 Jellinek JAM ACAD DERMATOL JANUARY 2011 Fig 4. Onychopapilloma presenting as a fine band of LLE. Fig 5. Onychopapilloma presenting as LLE with a teardrop-shaped origin in the lunula, and distal splinter hemorrhages (A). B, Direct dermatoscopy using watersoluble jelly shows splinter hemorrhages and a pink lucency in the lunula. Fig 6. Onychopapilloma presenting as LLE (A and B). C, Direct dermatoscopy using water-soluble jelly shows more subtle clinical features, splinter hemorrhages and V- shaped distal onycholysis.

5 JAM ACAD DERMATOL VOLUME 64, NUMBER 1 Jellinek 167.e5 Fig 7. A, Poorly defined band of LLE mimicking longitudinal melanonychia. B, Dermatoscopy highlights erythronychia and splinter hemorrhages. Histopathologic examination showed onychopapilloma. benign. In my practice, patients with such lesions of LLE are often followed up at regular intervals, are told to watch the lesion for any change, and to return sooner if changes occur. One proposed algorithm for this evaluation is found as Fig 20. POLYDACTYLOUS LONGITUDINAL ERYTHRONYCHIA PLE commonly presents in adulthood, if only because the associated diseases are unusual in children. Like LLE, PLE may be an incidental finding or presenting complaint (see Figs [Figs available online]). In some circumstances, such as underlying hemiplegia, multiple myeloma, or Darier s disease, the nail manifestation is an obvious associated finding of another disease. In certain cases, however, the nail changes constitute the sole clinical manifestation, without signs of an underlying disease. In these circumstances, a workup for Fig 8. A and B, Onychopapilloma presenting as LLE. C, Marked telangiectasia on lunula highlighted by dermatoscopy.

6 167.e6 Jellinek JAM ACAD DERMATOL JANUARY 2011 Fig 9. A, Onychopapilloma presenting with two bands of LLE and marked unilateral onycholysis. B, Dermatoscopy demonstrates marked funnel-shaped red dyschromia and telangiectasia on the lunula, a feature not apparent on direct examination or with a 73 loupe. etiology is advisable. In Darier s disease, which can present rarely as isolated nail disease, there is the possibility of transmitting the autosomal dominant genodermatosis to subsequent generations who may develop more generalized signs of disease. PLE in lichen planus may predate other cutaneous, mucosal, hair, or progressive scarring nail disease. PLE may also trigger a workup for hematologic malignancy if the diagnosis of amyloidosis is made. One proposed algorithm for this evaluation is found as Fig 21. BIOPSY TECHNIQUES FOR LONGITUDINAL ERYTHRONYCHIA Because the relevant pathology involves the distal matrix and commonly extends onto the nail bed, a longitudinal biopsy is perfectly suited. 25,26 A longitudinal fusiform biopsy is performed, starting at the mid to distal matrix, extending through the bed to the Fig 10. A, An unusual case of an onychopapilloma presenting as LLE on the great toe with a thin band of distal true leukonychia. B and C, Trap-door avulsion shows red discoloration of the distal matrix and a subtle longitudinal ridge of nail bed.

7 JAM ACAD DERMATOL VOLUME 64, NUMBER 1 Jellinek 167.e7 Fig 11. Glomus tumor presenting as LLE, with distal nail plate 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 splinter hemorrhages. B, After a longitudinal partial nail plate avulsion; band is visualized with splinter hemorrhages. C, After a narrow longitudinal excision. D, Short-term (10-week) follow-up, without obvious residual erythronychia.

8 167.e8 Jellinek JAM ACAD DERMATOL JANUARY 2011 Fig 14. Systemic amyloidosis with PLE, splinter hemorrhages, onychorrhexis, onychoschizia, hyperkeratosis of the cuticle, and a flaccid vesicle on dorsal aspect of one finger. Fig 17. Graft-versus-host disease with PLE, distal onycholysis, nail plate atrophy, and onychorrhexis. (Courtesy of Bianca Maria Piraccini, MD.) Fig 15. Nails with classic Darier s disease, with candycane PLE, distal onycholysis, splinter hemorrhages, and hyperkeratosis of hyponychium distal to red bands. Fig 18. Pathogenesis of longitudinal erythronychia, as proposed by de Berker. Distal matrix disease process results in production of a thinned ventral nail plate. Nail bed swells to fill the concave nail plate, is pinched by the sides. causing splinter hemorrhages, and cracks and splits distally with activities of daily living (ADL). Secondary reactive hyperkeratosis of distal nail bed and hyponychium results. Fig 16. Nail lichen planus, with PLE, nail plate thinning, onychorrhexis, and mild onycholysis. Fig 19. Nail bed biopsy in setting of onychopapilloma. Epithelium shows maturation with development of keratogenous zone, indicative of matrix metaplasia of nail bed. This is a histologic hallmark of onychopapilloma.

9 JAM ACAD DERMATOL VOLUME 64, NUMBER 1 Jellinek 167.e9 Fig 20. Proposed algorithm for evaluation of LLE. Fig 21. Proposed algorithm for evaluation of PLE.

10 167.e10 Jellinek JAM ACAD DERMATOL JANUARY 2011 hyponychium, and can be executed after one of several nail avulsion techniques 2,26,27 (see Figs 10 and 13) This biopsy technique may or may not require suturing to repair the distal matrix. The distal half of the matrix produces only the ventral 20% of the nail plate 28 and may not result in a split nail if left to heal by second intention. Nevertheless, the likelihood of this outcome can be minimized through wide undermining and side-to-side suturing of the matrix with absorbable suture, or by performing one of several local matrix sliding flaps. 29 The nail bed may heal by second intention with little risk of postoperative dystrophy. The longitudinal specimen is excised at the level of periosteum and sectioned according to the specific instructions of the surgeon. Longitudinal processing demonstrates contiguous pathology in this setting and in most cases is preferred by the pathologists with whom I work. Transverse sectioning, however, better demonstrates the marked papillomatosis of the nail bed in onychopapilloma. For those uncomfortable with performing the longitudinal biopsy, a 3-mm punch biopsy may be utilized of the distal matrix and involved bed to obtain diagnostic specimens. The defects left by these round biopsies neither facilitate nor require suturing. In the setting of an onychopapilloma, however, it is important to note that excision of the distal hyperkeratosis in isolation will result in prompt recurrence of the lesion. 1,2 In other instances, a single bed punch biopsy may miss the diagnosis altogether. 2 This fact underscores the indication for longitudinal biopsy. For those cases with a high suspicion of glomus tumor, the surgeon may proceed with one of several acceptable techniques These are generally divided between those that require partial or total plate avulsion and dissection through the nail epithelia (transungual) and those that are executed without disruption of the nail plate or epithelia (subepithelial). As in cases of other nail tumors, complete excision is preferred at the time of biopsy. 33,34 And while this is occasionally impossible because of lesion width, tumor biology, 8 or poor demarcation, when successful, the most common diagnoses (onychopapilloma, glomus tumor) are both diagnosed and treated in the same procedure. 2 I thank Antonio Cruz, MD, for flowchart creation and Jeffrey D. Bernhard, MD, for editing assistance. REFERENCES 1. Baran R, Perrin C. Longitudinal erythronychia with distal subungual keratosis: onychopapilloma of the nail bed and Bowen s disease. Br J Dermatol 2000;143: de Berker DA, Perrin C, Baran R. Localized longitudinal erythronychia: diagnostic significance and physical explanation. Arch Dermatol 2004;140: Baran R, Perrin C. Focal subungual warty dyskeratoma. Dermatology 1997;195: Chamberlain AJ, Millard PR, Pryce DW, Dawber RP. Acquired periungual arteriovenous tumour (cirsoid aneurysm). J Eur Acad Dermatol Venereol 2005;19: Richert B, Iorizzo M, Tosti A, Andre J. Nail bed lichen planus associated with onychopapilloma. Br J Dermatol 2007;156: Cogrel O, Beylot-Barry M, Doutre MS. [Subungual squamous cell carcinoma revealed by longitudinal erythronychia]. Ann Dermatol Venereol 2008;135: French. 7. Dalle S, Depape L, Phan A, Balme B, Ronger-Savle S, Thomas L. Squamous cell carcinoma of the nail apparatus: clinicopathological study of 35 cases. Br J Dermatol 2007;156: Harwood M, Telang GH, Robinson-Bostom L, Jellinek N. Melanoma and squamous cell carcinoma on different nails of the same hand. J Am Acad Dermatol 2008;58: Gee BC, Millard PR, Dawber RP. Onychopapilloma is not a distinct clinicopathological entity. Br J Dermatol 2002;146: Baran R, Dawber RPR, Richert B. Physical signs. In: Baran R, Dawber RPR, De Berker DAR, Haneke E, Tosti A, editors. Baran and Dawber s Diseases of the nails and their management. 3rd edition. Malden, MA: Blackwell Science; pp Zaias N, Ackerman AB. The nail in Darier-White disease. Arch Dermatol 1973;107: Derrick EK, Price ML. Primary systemic amyloid with nail dystrophy. J R Soc Med 1995;88:290P-1P. 13. Siragusa M, Schepis C, Cosentino FI, Spada RS, Toscano G, Ferri R. Nail pathology in patients with hemiplegia. Br J Dermatol 2001;144: Liddle BJ, Cowan MA. Lichen planus-like eruption and nail changes in a patient with graft-versus-host disease. Br J Dermatol 1990;122: Palencia SI, Rodriguez-Peralto JL, Castano E, Vanaclocha F, Iglesias L. Lichenoid nail changes as sole external manifestation of graft vs. host disease. Int J Dermatol 2002;41: Hoffman MD, Fleming MG, Pearson RW. Acantholytic epidermolysis bullosa. Arch Dermatol 1995;131: Baran R, Dawber RP, Perrin C, Drape JL. Idiopathic polydactylous longitudinal erythronychia: a newly described entity. Br J Dermatol 2006;155: Baran R, Perrin C. Localized multinucleate distal subungual keratosis. Br J Dermatol 1995;133: Criscione V, Telang G, Jellinek NJ. Onychopapilloma presenting as longitudinal leukonychia. J Am Acad Dermatol 2010;63: Al-Qattan MM, Al-Namla A, Al-Thunayan A, Al-Subhi F, El- Shayeb AF. Magnetic resonance imaging in the diagnosis of glomus tumours of the hand. J Hand Surg Br 2005;30: Matsunaga A, Ochiai T, Abe I, Kawamura A, Muto R, Tomita Y, et al. Subungual glomus tumour: evaluation of ultrasound imaging in preoperative assessment. Eur J Dermatol 2007;17: Takemura N, Fujii N, Tanaka T. Subungual glomus tumor diagnosis based on imaging. J Dermatol 2006;33: Koc O, Kivrak AS, Paksoy Y. Subungual glomus tumour: magnetic resonance imaging findings. Australas Radiol 2007;(51 Spec No);B Chen SH, Chen YL, Cheng MH, Yeow KM, Chen HC, Wei FC. The use of ultrasonography in preoperative localization of digital glomus tumors. Plast Reconstr Surg 2003;112:115-9; discussion 120.

11 JAM ACAD DERMATOL VOLUME 64, NUMBER 1 Jellinek 167.e Zaias N. The longitudinal nail biopsy. J Invest Dermatol 1967; 49: Collins SC, Jellinek NJ. Matrix biopsy of longitudinal melanonychia and longitudinal erythronychia: a step-by-step approach. Cosmet Dermatol 2009;22: Collins SC, Cordova K, Jellinek NJ. Alternatives to complete nail plate avulsion. J Am Acad Dermatol 2008;59: De Berker D, Mawhinney B, Sviland L. Quantification of regional matrix nail production. Br J Dermatol 1996;134: Abimelec P, Dumontier C. Basic and advanced nail surgery (Part 1: Principles and Techniques). In: Scher RK, Daniel CRIII, editors. Nails: diagnosis, therapy, surgery. Philadelphia: Elsevier Saunders; pp McDermott EM, Weiss AP. Glomus tumors. J Hand Surg [Am] 2006;31: Ekin A, Ozkan M, Kabaklioglu T. Subungual glomus tumours: a different approach to diagnosis and treatment. J Hand Surg [Br] 1997;22: Vasisht B, Watson HK, Joseph E, Lionelli GT. Digital glomus tumors: a 29-year experience with a lateral subperiosteal approach. Plast Reconstr Surg 2004;114: Jellinek N. Nail matrix biopsy of longitudinal melanonychia: diagnostic algorithm including the matrix shave biopsy. J Am Acad Dermatol 2007;56: Tosti A, Piraccini BM, de Farias DC. Dealing with melanonychia. Semin Cutan Med Surg 2009;28:49-54.