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Multiple linear red bands on the fingernails: Idiopathic polydactylous longitudinal erythronychia

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Multiple linear red bands on the fingernails: Idiopathic polydactylous longitudinal erythronychia
Philip R Cohen MD
Dermatology Online Journal 18 (2): 6

The University of Houston Health Center, University of Houston, Houston, Texas
The Department of Dermatology, The University of Texas MD Anderson Cancer Center, Houston, Texas
The Department of Dermatology, University of Texas-Houston Medical School, Houston, Texas


Abstract

Longitudinal erythronychia refers to a linear red band that extends from the proximal nail fold or lunula to the distal tip of the nail. Multiple digits with one or more longitudinal red streaks, presenting in an individual without an associated skin condition, systemic disease, or subungual tumor, characterizes idiopathic polydactylous longitudinal erythronychia (IPLE). The nail findings in a woman with asymptomatic IPLE since childhood are described and the salient features of this condition in this individual and the 10 previously reported patients are summarized. IPLE is a nail dyschromia of undefined pathogenesis for which affected persons can be reassured of the benign nature of the condition and whose nails only require clinical monitoring.



Introduction

Idiopathic polydactylous longitudinal erythronychia (IPLE) is a benign, seldom recognized, usually asymptomatic condition that presents with one or more linear red bands on the nails of individuals without any associated skin disorder, systemic disease, or subungual malignant tumor. This condition was originally described by Baran et al [1] in 2006 and only 10 individuals have been reported [1, 2, 3, 4]. A woman with longitudinal red streaks on all digits of both hands is reported and the salient features of IPLE are summarized.


Case report


Figure 1
Figure 1. Idiopathic polydactylous longitudinal erythronychia presenting as multiple asymptomatic longitudinal red bands on the right and left thumbnails of a 57-year-old woman.

A 57-year-old woman presented with asymptomatic linear red streaks on the nails of all digits of both hands that had been present since childhood. She was being seen for follow up of chemotherapy-associated inflammation of actinic keratoses on her arms while receiving antineoplastic agents [premetrexed x 4 cycles and either cisplatin (cycle 1) or carboplatin (cycles 2-4)] for non-small cell lung cancer. This had been initially excised and subsequently treated with 50 Gray (in 25 fractions) of radiation therapy and currently showed no evidence of residual or metastatic cancer.

Examination of the nails showed clear acrylic nail polish that had been applied 1 month earlier and whose proximal border had advanced from the nail fold. Longitudinal narrow (<2 mm) red bands (longitudinal erythronychia) extended from the proximal nail fold to the distal tip of the nail. Multiple linear red streaks were present on both thumbnails (Figure 1). Between 2 to more than 5 longitudinal red bands were also observed on all of the fingernails of both hands (Figure 2). None of the toenails had linear red streaks.


Figure 2aFigure 2b
Figures 2a and 2b.The woman’s right hand (2a) and left hand (2b) also have two or more asymptomatic linear red streaks of idiopathic polydactylous longitudinal erythronychia that extend from the proximal nail fold to the distal edge of all fingernails.

Discussion

The presence of a linear red band, typically extending from the proximal nail fold to the distal edge of the nail plate, is referred to as longitudinal erythronychia [3, 5]. The condition is classified based upon the number of nails with red streaks (monodactylous versus polydactylous) and the number of red bands per nail (one versus multiple) [3, 4]. In contrast to monodactylous erythronychia, which usually appears as a solitary linear red band on one digit (Type IA), IPLE always involves multiple nails and presents with either one (Type IIA) or multiple (Type IIB) linear red bands on each nail [3, 4, 5].

Monodactylous longitudinal erythronychia and polydactylous longitudinal erythronychia can be idiopathic. Monodactylous longitudinal erythronychia can also be caused by either a benign (glomus tumor, onychopapilloma or warty dyskeratoma) or malignant (melanoma or squamous cell carcinoma) subungual tumor, or observed in patients with either hemiplegia on the thumbnail of the paralyzed arm) or prior distal digit surgery (as a consequence of the scar). In contrast, non-idiopathic polydactylous longitudinal erythronychia can either be associated with dermatologic conditions (acantholytic dyskeratotic epidermal nevus, acantholytic epidermolysis bullosa, acrokeratosis of Hopf, Darier disease or lichen planus) or systemic diseases (amyloidosis, graft-versus-host disease, or pseudobulbar syndrome) [3, 5].

The incidence of IPLE has recently been observed to be 1 percent; the condition was noted in 3 men from a study group of 134 men and 112 women [4]. IPLE has only been reported in 10 patients and the gender (male) was provided for 5 of the individuals [1, 2, 3, 4]. Hence, the patient in this report may be the first woman in whom this condition has been described.

IPLE is usually asymptomatic. The patients are often unaware of the nail finding; alternatively, they present for evaluation because of cosmetic concerns [1, 2, 3, 4]. However, one man – a 40-year-old blind concert pianist – presented with pain “in most of the distal digits when he was playing piano” [1].

The width of the linear red band ranged from less than 1 mm to 6 mm; thinner bands were often found as multiple lesions on the same nail, whereas broader bands were usually solitary. Similar to patients with monodactylous longitudinal erythronychia, distal subungual hyperkeratosis, fissuring at the free end of the nail plate, and splinter hemorrhages were longitudinal erythronychia-associated nail changes observed in some of the individuals with IPLE. Pseudolongitudinal erythronychia, in which the intensity of the distal red linear band is diminished when the digit is elevated above the level of the heart, was also observed in 3 individuals. In addition, red lunula and leukonychia were noted in one man [1, 2, 3, 4].

IPLE is favored to represent a benign nail variant for which the pathogenesis remains to be determined. Indeed, IPLE does not appear to be associated with any adverse sequellae. Although 3 of the individuals, including the currently described woman, in whom this nail condition has been observed have one or more systemic malignancies – either bladder cancer (1 man) or lung cancer (1 woman) or mantle cell lymphoma and prostate cancer (1 man) – this observation is likely secondary to the patient population seen at the investigator’s clinic and not related to the etiology of the linear red bands [4].


Conclusions

One or multiple linear red bands on several digits of the hands characterize IPLE. This benign nail condition of undefined pathogenesis has been observed in 1 percent of individuals. However, future studies are warranted to confirm the incidence of this uncommonly reported finding. Similar to patients with one or more dark (brown or black) linear bands on multiple digits (polydactylous longitudinal melanonychia striata) in which the nail finding is also associated with a benign clinical behavior, for individuals with IPLE it is reasonable to discuss the likely benign nature of the finding and to clinically monitor their nails.

References

1. Baran R, Dawber RPR, Perrin C, Drape JL: Idiopathic polydactylous longitudinal erythronychia: a newly described entity. [Letter] Br J Dermatol 2006 Jul;155(1):219-221. [PubMed]

2. Baran R: The red nail—always benign? Actas Dermosifiliogr 2009 Nov;100 Suppl 1:106-113. [PubMed]

3. 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 Aug;12(4):217-231. [PubMed]

4. Cohen PR: Idiopathic polydactylous longitudinal erythronychia. J Clin Aesthet Dermatol 2011 Apr;4(4):22-28. [PubMed]

5. Jellinek NJ: Longitudinal erythronychia: suggestions for evaluation and management. J Am Acad Dermatol 2011 Jan;64(1):167.e1-11.Epub 2010 Aug. [PubMed]

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