Nail Psoriasis

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Fingernail psoriasis reconsidered: A case-control study

Haike M. J. van der Velden, MD, Karlijn M. G. Klaassen, MD, Peter C. M. van de Kerkhof, MD, PhD,
and Marcel C. Pasch, MD, PhD
Nijmegen, The Netherlands

Background: Literature concerning clinical signs and frequency of nail psoriasis is incomplete. Recent
studies focus only on signs included in the Nail Psoriasis Severity Index (NAPSI).

Objective: We sought to describe clinical characteristics of fingernail psoriasis in comparison with healthy
controls.

Methods: We collected data on 49 patients with fingernail psoriasis who visited our outpatient department
and 49 control subjects, through questionnaires and clinical examination. The disease severity was
measured by the NAPSI.

Results: Mean NAPSI score in patients and control subjects was 26.6 and 3.6, respectively. Most items
included in the NAPSI were specific for nail psoriasis. Onycholysis and splinter hemorrhages were most
frequently observed. Leukonychia was more frequent in control subjects. Longitudinal ridges and Beau
lines are not included in the NAPSI but are significantly more frequently seen in patients than in control
subjects.

Limitations: Limited sample size was a limitation.

Conclusion: The NAPSI was able to discriminate patients with fingernail psoriasis from healthy control
subjects. Onycholysis and splinter hemorrhages were the most prevalent fingernail changes in psoriatic
patients. Leukonychia was more frequently observed in control subjects, which raises the question of
whether leukonychia should remain in the NAPSI. On the other hand, longitudinal ridges and Beau
lines occurred more frequently in psoriasis but are not included in the NAPSI. ( J Am Acad Dermatol
2013;69:245-52.)

Key words: Beau lines; epidemiology; leukonychia; nail psoriasis; Nail Psoriasis Severity Index; nails;
onycholysis; psoriasis; splinter hemorrhages.

T he prevalence of nail psoriasis varies be-


tween 10% and 79%.1-6 It is reported that 80%
to 90% of psoriatic patients develop nail
psoriasis some time during their disease.1-4,6
Abbreviations used:
NAPSI:
PASI:
PsA:
Nail Psoriasis Severity Index
Psoriasis Area and Severity Index
psoriatic arthritis
However, psoriatic nail lesions rarely appear as the
only clinical manifestation of psoriasis, as psoriasis
limited to the nails occurs in only 1% to 10% of Previous research states that nail psoriasis is associ-
patients with psoriasis.1,3,4 It is mentioned that pso- ated with both longer duration and greater extent of
riatic arthritis (PsA) is associated with higher rates of skin disease.2
nail psoriasis, and the severity of nail disease corre- The clinical spectrum of nail psoriasis is hetero-
lates with indicators of severity of joint disease.7 geneous, depending on the involvement of nail bed,

From the Department of Dermatology, Radboud University Internal Postal Code 370, PO Box 9101, Nijmegen 6500 HB, The
Nijmegen Medical Center. Netherlands. E-mail: H.vanderVelden@derma.umcn.nl.
Funding sources: None. Published online March 29, 2013.
Conflicts of interest: None declared. 0190-9622/$36.00
Accepted for publication February 11, 2013. Ó 2013 by the American Academy of Dermatology, Inc.
Reprint requests: Haike M. J. van der Velden, MD, Department of http://dx.doi.org/10.1016/j.jaad.2013.02.009
Dermatology, Radboud University Nijmegen Medical Center,

245
246 van der Velden et al J AM ACAD DERMATOL
AUGUST 2013

nail matrix, or both. Widely accepted manifestations chloramphenicol and cycloheximide and 1 without
attributed to psoriatic inflammation of the nail matrix cycloheximide. None of the patients tested positive.
are pitting, red spots in the lunula, leukonychia, and In addition, a total of 49 age- and gender-matched
nail plate crumbling. Nail bed manifestations are oil- control subjects were included. They were healthy
drop discoloration, onycholysis, splinter hemor- individuals older than 18 years without (skin) dis-
rhages, and subungual hyperkeratosis.5,6 Additional eases or medication associated with nail manifesta-
symptoms found in literature include Beau lines, tions and were recruited from patients, visitors, and
onychomadesis, nailfold in- employees of the above-
volvement, and longitudinal mentioned outpatient clinic.
ridging.6 These are not in- CAPSULE SUMMARY
cluded in the widely used Survey details
d The clinical spectrum of nail psoriasis is
Nail Psoriasis Severity Index The survey of the psoriatic
heterogeneous.
(NAPSI) scoring system, patients consisted of a ques-
which is limited to the first 8 d We describe the clinical characteristics of tionnaire taken by interview
mentioned nail changes. fingernail psoriasis in comparison with and clinical examination
Using this instrument each healthy controls. conducted by the same in-
quadrant of each fingernail d
Knowledge of the complete spectrum of vestigator. The interview
is evaluated for presence or clinical changes in nail psoriasis will help included items concerning
absence of nail bed or nail clinicians to recognize this disease. demographic patient charac-
matrix disease. The sum of teristics, general health,
the scores for all the finger- psoriasis-related data, and
nails is the NAPSI score for the patient at that time specific nail psoriasis questions. Diagnosis of con-
(see Fig 1 for an example of calculation).8 In most comitant PsA was made through physical examina-
recent studies concerning nail psoriasis only the total tion by a rheumatologist. Nail manifestations in both
NAPSI score is used. Literature concerning the fre- groups, ie, fingernail psoriasis and healthy control,
quency of each specific clinical sign of nail psoriasis were documented and photographed.
is limited.2,7,9-17
The aim of this study was to describe the epide- Severity of disease measurement
miologic characteristics and the frequency of clinical The severity of psoriasis was assessed by the
signs of fingernail psoriasis and compare them with Psoriasis Area and Severity Index (PASI) (range
healthy controls. 0-72).18 Higher scores indicate more severe psoriasis
of the skin. The body surface area represents the
percentage of body surface affected and classifies the
METHODS severity of the condition. The severity of fingernail
Study population psoriasis was assessed by the NAPSI score (Fig 1).8
A total of 49 patients with fingernail psoriasis were Additional nail changes (eg, Beau lines, onychoma-
recruited consecutively in this case-control study desis, longitudinal ridging, and nailfold involve-
from the outpatient clinic at the Department of ment) were also assessed for each fingernail. The
Dermatology of the Radboud University Nijmegen fingernails of the healthy control subjects were
Medical Center, Nijmegen, The Netherlands. Patients scored for the presence of the same manifestations
were included from December 2009 through July of nail psoriasis and a NAPSI score was measured.
2012. Included patients were older than 18 years
with clinically diagnosed fingernail psoriasis (NAPSI Statistical analysis
score $ 1). Patients who used artificial nails in the Data were entered in a Statistical Package for
past 6 months or had presence of a skin disease Social Sciences (SPSS 18.0, SPSS Inc, Chicago, IL)
(other than psoriasis), which is associated with nail database and subsequently statistical analyses were
manifestations, were excluded, as were patients with performed. Descriptive statistics were provided us-
toenail involvement exclusively, because of the in- ing mean (SD) and median (range) for normally and
creased risk of coexistence of onychomycosis. To nonparametric distributed numeric values, respec-
exclude patients with concomitant onychomycosis, tively. Frequencies and percentages were used for
clippings and subungual scrapings of affected fin- categorical variables. Missing values were not in-
gernails were collected for direct microscopic exam- cluded to calculate percentages. Comparison of
ination (10% potassium hydroxide preparations) and numeric variables were analyzed with the unpaired
fungal culture. Cultures were carried out on 2 differ- t tests or the Mann-Whitney U test and the x2 or
ent types of Sabouraud dextrose agar: 1 with Fisher exact test was used for differences between
J AM ACAD DERMATOL van der Velden et al 247
VOLUME 69, NUMBER 2

Table I. Sociodemographic and clinical


characteristics
Nail Control
psoriasis group
group (n = 49) (n = 49) P value
Gender
Female 23 (46.9%) 23 (46.9%) 1.000 (NS)
Male 26 (53.1%) 26 (53.1%)
Age, y, mean 6 SD 48 6 13.4 48 6 13.4 .958 (NS)
Age at psoriasis 29 6 14.1
onset, y,
mean 6 SD
Duration of 19 6 13.6
psoriasis, y,
mean 6 SD
Age at nail psoriasis 38 6 12.6
onset, y,
mean 6 SD
Duration of nail 10 6 8.1
psoriasis, y,
mean 6 SD
Localization of nail
psoriasis
Fingernails 10 (20.4%)
Toenails 0 (0%)
Fig 1. Fingernail psoriasis. Grading with Nail Psoriasis Both 39 (79.6%)
Severity Index (NAPSI).8 The nail is divided into 4 NAPSI score, 26.6 6 14.5 3.6 6 3.3 \.001
quadrants. Each quadrant is evaluated for presence of mean 6 SD
any features of nail bed psoriasis (0-4) and nail matrix Modified target 10.4 6 7.3
psoriasis (0-4). Score is 0 if no sign of nail bed involve- NAPSI score,
ment is present in any quadrant. Score is 4 if signs of nail mean 6 SD
bed involvement are present in all 4 quadrants. Score for PASI score, median 3.7 (0.0-29.5)
matrix is calculated in a similar way. In this example, (range)
pitting and nail plate crumbling are present in quadrants BSA, mean 6 SD 6.6% 6 11.2
A, B, and C, and therefore score for nail matrix psoriasis
is 3. We see onycholysis, splinter hemorrhages, and nail BSA, Body surface area; NAPSI, Nail Psoriasis Severity Index; NS, not
bed hyperkeratosis in quadrants C and D, and therefore significant; PASI, Psoriasis Area and Severity Index.
score for nail bed psoriasis is 2. Sum of these scores is
total score for that nail (in this example 5), with included psoriatic patients was 48 years (6SD 13.4).
minimum of 0 (representing no nail psoriasis) and The mean age at onset of psoriatic skin manifesta-
maximum of 8, with higher scores indicating more severe tions was 29 years (6SD 14.1) and the mean duration
nail psoriasis. Sum of all fingernails (not in this figure) of skin disease was 19 years (6SD 13.6). Median PASI
would be total NAPSI score, ranging from 0 to 80. Beau score was 3.7 (range 0.0-29.5) with a mean body
lines are also present, but they are not included in NAPSI
surface area of 6.6% (6SD 11.2). At study enrollment
score.
19 patients (38.8%) were actively treated with sys-
temic therapy.
categorical variables. Spearman rank test was used to Nail psoriasis features started predominantly after
explore the relationship between continuous varia- the onset of the cutaneous lesions, with a mean delay
bles. P values less than .05 were considered to be of 9 years and appeared most frequently simulta-
statistically significant. All tests were 2-sided. neously on fingernails and toenails (79.6%). Mean
age of onset of fingernail psoriasis was 38 years
RESULTS (6SD 12.6) and mean duration of nail disease was 10
A total of 49 psoriatic patients and 49 healthy years (6SD 8.1). The mean number of fingernails
control subjects were included in the study. The presenting with psoriasis-specific nail changes in
descriptive demographic patient characteristics have patients with nail psoriasis was 8.3 (6SD 2.2). The
been summarized in Table I. No statistical difference mean number of involved fingernails for the left
was observed between psoriatic patients and control hand was 4.1 (6SD 1.1) and for the right hand was
subjects for gender and age. The mean age of the 4.2 (6SD 1.3).
248 van der Velden et al J AM ACAD DERMATOL
AUGUST 2013

Table II. Frequency of fingernail changes and 1.1 (6SD 1.8). Overall, 44 psoriatic patients
Nail
(89.8%) showed both nail matrix and nail bed
psoriasis Control changes; the remaining patients had matrix changes
group group only. In the control group 40 individuals (81.6%)
(n = 49) (n = 49) P value
showed any fingernail changes, most of them were
Nail signs included in NAPSI minor.
Subungual 23 (46.9%) 1 (2.0%) \.001 Of the 49 patients with psoriatic fingernail disease,
hyperkeratosis
9 patients (18.4%) displayed nail changes as the
Oil-drop 33 (67.3%) 0 (0%) \.001
exclusive manifestation of psoriasis. Also 9 psoriatic
discoloration
Splinter 46 (93.9%) 18 (36.7%) \.001 patients (18.4%) were given the diagnosis of PsA by a
hemorrhages rheumatologist, of which 5 patients mentioned ar-
Onycholysis 46 (93.9%) 9 (18.4%) \.001 thritis of the distal interphalangeal joints. NAPSI and
Nail plate crumbling 21 (42.9%) 0 (0%) \.001 PASI scores of patients with and without PsA were
Red spotted lunula 1 (2.0%) 0 (0%) 1.000 (NS) not significantly different (P = .079).
Leukonychia 20 (40.8%) 32 (65.3%) .015
Pitting 36 (73.5%) 5 (10.2%) \.001 Correlations
Nail signs not included in NAPSI No significant correlation was found between
Longitudinal ridges 20 (40.8%) 8 (16.3%) .007 NAPSI and PASI scores (Spearman r = 0.042,
Nailfold involvement 8 (16.3%) 0 (0%) .003
P = .784). The age of onset of fingernail psoriasis
Beau lines 5 (10.2%) 0 (0%) .027
was positively correlated to age of onset of cutane-
Onychomadesis 1 (2.0%) 0 (0%) 1.000 (NS)
ous manifestations of psoriasis (Spearman r = 0.718,
NAPSI, Nail Psoriasis Severity Index; NS, not significant. P \.001).

DISCUSSION
The observed frequency of fingernail changes in We collected data on 49 adult psoriatic patients
the studied population is displayed in Table II. All 8 with fingernail involvement to describe the features
nail manifestations included in NAPSI were seen: of fingernail psoriasis and their prevalence. We
onycholysis (93.9%), splinter hemorrhages (93.9%), compared these results with data collected on 49
pitting (73.5%), oil-drop discoloration (67.3%), sub- age- and gender-matched control subjects without
ungual hyperkeratosis (46.9%), nail plate crumbling fingernail disease to determine which of the ob-
(42.9%), leukonychia (40.8%), and red spots in the served features are disease-specific for fingernail
lunula (2.0%) (Fig 2). Onycholysis was predomi- psoriasis. Patients with concomitant onychomycosis
nantly seen in the distal quadrants of the fingernail were excluded from this study, because Gupta et al19
(60.0%). Median number of pits per fingernail was found a higher prevalence of onychomycosis in
5.5 (range 1-26); 74.2% of patients had less than 10 psoriatic patients and discussed that fungal infection
pits. As expected, the frequency of most nail changes might have a role in the maintenance or aggravation
was statistically significantly lower in the control (Koebnerization) of nail psoriasis. Diagnosis of nail
group (Table II). However, leukonychia was signif- psoriasis was made clinically and not confirmed
icantly more frequent in healthy control subjects histologically, which may have influenced our study
(65.3% vs 40.8%, P = .015). results. Furthermore, we enrolled 19 patients who
Nail changes not included in the NAPSI, but seen were actively treated with systemic therapy, which
in psoriatic patients, were longitudinal ridges (40.8%, could affect the nail signs we have observed.
mean 4.3 fingers affected), nailfold involvement Reports of prevalence of clinical features of nail
(16.3%, mean 4.3 fingers affected), Beau lines psoriasis vary considerably in the literature, which
(10.2%, mean 1.8 fingers affected), and onychoma- may partly be a result of differences in descriptions
desis (2.0%, mean 1.0 finger affected). Of these, only and definitions used in previous studies. Grover
longitudinal ridges were seen in the control group et al9 reported onycholysis as the most prevalent nail
(16.3%). change in 76.0% of patients. Gisondi et al15 studied
The mean NAPSI score in patients and control nail involvement in psoriatic patients using ultraso-
subjects was 26.6 (6SD 14.5) and 3.6 (6SD 3.3), nography and also found onycholysis to be the most
respectively (P \ .001). When separated, in both common clinical sign. Other studies reported pit-
study groups nail matrix NAPSI scores were higher ting,2,10-12,14,17 oil-drop discoloration,7 or subungual
than nail bed NAPSI scores, in psoriatic patients, hyperkeratosis13,16 to be the most prevalent nail
respectively, 14.6 (6SD 11.6) and 12.8 (6SD 8.0), change in psoriatic patients. The most prevalent
and in control subjects, respectively, 2.5 (6SD 2.8) fingernail changes found in our fingernail psoriasis
J AM ACAD DERMATOL van der Velden et al 249
VOLUME 69, NUMBER 2

Fig 2. Fingernail psoriasis. Nail changes in fingernail psoriasis observed in this study.
A, Nailfold involvement, nail plate crumbling, and splinter hemorrhage (arrow). B, Leuko-
nychia (asterisk), pitting, and onycholysis. C, Oil-drop discoloration, onycholysis, and splinter
hemorrhages (arrow). D, Beau lines, onycholysis, and splinter hemorrhages (arrows).
E, Longitudinal ridges and splinter hemorrhage (arrow). F, Subungual hyperkeratosis.

group were onycholysis (93.9%) and splinter hem- it to be less common, ranging from 1% to
orrhages (93.9%). Three previous studies found 28.9%.7,11,12,15,16 Onycholysis and splinter hemor-
splinter hemorrhages to be the second (20.4%10) or rhages are manifestations of nail bed psoriasis.
third (39.3%14-42%17) most common manifestation However, overall nail matrix NAPSI scores were
in fingernail psoriasis. However, others reported higher than nail bed NAPSI scores in our patients.
250 van der Velden et al J AM ACAD DERMATOL
AUGUST 2013

This result is in line with previous investigations.12,20 Chandran et al25 found excellent agreement on the
The red spotted lunula is an infrequent nail change in modified NAPSI24 among rheumatologists and der-
psoriasis. In our study population only 1 psoriatic matologists. Our study results made us reconsider
patient (2.0%) showed red spots in the lunula. In these (modified) NAPSI scoring systems. We ques-
previous studies this nail symptom was observed in tion whether leukonychia should remain in nail
0.4% by Aktan et al,16 in 1.3% by Kyriakou et al,7 and psoriasis disease severity scores and whether addi-
in 10.2% by Rich et al.12 In most previously published tional symptoms, such as Beau lines, should be
literature concerning nail psoriasis additional nail added. Baran21 proposed a different scoring system,
symptoms such as Beau lines and longitudinal ridg- which meets these criteria. However, to our knowl-
ing were not taken into account and scored, prob- edge reliability assessment of this scoring system has
ably because these features are not included in the not yet been done and assessing nail psoriasis
NAPSI score8 and may be even less specific for severity based on only 4 clinical features seems
psoriasis than the 8 above-mentioned features. questionable. Furthermore, evaluating subungual
Nevertheless, we found Beau lines in 10.2% of hyperkeratosis with a calliper is not convenient.
patients and in none of the control subjects (Fig 1). Different fingernail findings have been taken into
Longitudinal ridging was even present in 40.8% of account in the grading system used by Cannav o
patients with fingernail psoriasis but also in 16.3% of et al,26 but again additional symptoms, such as Beau
healthy control subjects, making this feature rather lines, were not taken into account and interrater
sensitive but not specific for fingernail psoriasis. reliability assessment of this scoring system has not
Both longitudinal ridging and Beau lines can be been performed yet. Additional studies assessing the
explained by the inflammatory process in the prox- reliability and sensitivity of the different scoring
imal nail matrix. Baran21 also has recognized the systems and larger observational case-control studies
relevance of Beau lines for estimating disease activity concerning clinical signs of nail psoriasis to decide
in nail psoriasis and included the number of Beau which symptoms should be part of an optimal nail
lines in his nail psoriasis severity scoring system. score are needed.
Various nail symptoms known to occur in nail We did not find a correlation between mean
psoriasis (splinter hemorrhages, onycholysis, pitting, NAPSI scores and mean PASI scores. This raises the
and subungual hyperkeratosis) were also observed question of whether nail psoriasis is a comorbidity of
in the control group without nail disease. However, psoriasis or an isolated disease expression. Rich
those nail changes were significantly less frequent in et al12 also did not find a correlation between
the control group compared with the fingernail baseline NAPSI and PASI scores. However, improve-
psoriasis group. In contrast, leukonychia was even ment of PASI score did correlate increasingly over
more frequently seen in the control group. This time with the percentage improvement of NAPSI
finding suggests that leukonychia is not a nail score in a group of patients with psoriasis treated
psoriasisespecific symptom and raises the question with infliximab. Of course, this only shows that this
of whether leukonychia is the consequence of the anti-tumor necrosis factor-a treatment is effective
inflammatory process characterizing psoriasis or just against psoriasis of the skin and psoriasis of the nails
occurring in nail psoriasis because it has a very high but does not prove direct correlation between both.
prevalence in the general population. Leukonychia The same was found by Sanchez-Rega~ na et al20; as
was present in over 65% of our healthy control the skin lesions improve, the nail symptoms improve
subjects. Consequently, the NAPSI score and espe- as well. On the other hand Augustin et al27 found
cially the positioning of leukonychia in this score significantly higher PASI scores in psoriatic patients
should perhaps be reconsidered. The same applies with nail involvement compared with patients with-
to the red spotted lunula, because this sign was rarely out nail involvement. Further research with a larger
observed. However, the red spotted lunula, oil-drop sample size is needed to see whether there is a direct
discoloration, and nail plate crumbling were only relation between nail psoriasis and the cutaneous
observed in patients with fingernail psoriasis and manifestations of psoriasis.
therefore seem more specific for the diagnosis of nail Based on the questionnaire fingernail psoriasis
psoriasis. started predominantly after the onset of the skin
The NAPSI score is an objective tool for the lesions, with a mean delay of 9 years. Interestingly,
evaluation of nail psoriasis8 and the interobserver De Marco et al28 and Palmou et al14 found a similar
reliability is established.16,22 A modification of this delay in the onset of PsA. Those delays may be
scoring system, the modified (target) NAPSI score, explained by shared pathogenetic principles, which
using gradation systems for the different nail signs, differ from the pathogenesis of psoriatic skin disease.
was proposed by 2 separate study groups.23,24 Psoriatic disease, encompassing skin, joint, and nail
J AM ACAD DERMATOL van der Velden et al 251
VOLUME 69, NUMBER 2

involvement, has been considered as an autoim- parameters: a cross-sectional study. Dermatology 2011;223:
mune disorder with a major role for adaptive immu- 222-9.
8. Rich P, Scher RK. Nail psoriasis severity index: a useful tool for
nity. However, recently the view on the pathogenesis evaluation of nail psoriasis. J Am Acad Dermatol 2003;49:206-12.
of psoriatic joint and nail disease has been chal- 9. Grover C, Reddy BS, Uma Chaturvedi K. Diagnosis of nail
lenged.29-31 Genetic studies fail to demonstrate a psoriasis: importance of biopsy and histopathology. Br J
human leukocyte antigen-Cw6 (HLA-Cw6) associa- Dermatol 2005;153:1153-8.
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Fornara L, et al. Prevalence, severity and clinical features of
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Baseline nail disease in patients with moderate to severe
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nail involvement in our fingernail psoriasis study 1 year. J Am Acad Dermatol 2008;58:224-31.
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Red spots in the lunula appear to be an infrequent prospective clinical study. J Cutan Med Surg 2003;7:317-21.
sign. Various nail psoriasis symptoms, such as splin- 14. Palmou N, Marzo-Ortega H, Ash Z, Goodfield M, Coates LC,
Helliwell PS, et al. Linear pitting and splinter hemorrhages are
ter hemorrhages, also appeared in individuals with- more commonly seen in the nails of patients with established
out nail disease, however they were significantly less psoriasis in comparison to psoriatic arthritis. Dermatology
frequent. Remarkably, we showed that leukonychia 2011;223:370-3.
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