r-. 111 (;)()1..

tCI\ d l11d111

NAIL LICHEN PLANUS

How Do I Manage?

MATILDE IORIZZO, MD PhD FMH

Pnvote Dormotology Practice - aer,nzona & Lugono (Switzorlond)
Locturor US/ Mod, Faculty of Biomod,co/ ~ncos Lugono (Switzorland)
motddo,onuo@gmaJ/ com
 t-..dil S<..xid v c1I Ind1d

Clinical presentations
• classic / typical
• trachyonychia
• idiopathic atrophy of the nails
• Yellow Nail Syndrome-like
• bullous/erosive
 · ~dil 6ocicl ,. ol lndi~1

Clinical features (Qol)

• Depends on the site of involvement (matrix, bed, both)
• Combination of findings usually occur
• Different degrees of severity are possible

Gavilanes-Coloma MC. Evalua/Jofl of cln,c--Oflfmo.copic (,nd/nQS III nJ /lchMI plarus Propo.al kx a nqw clas"r,cat,on JMD 2017 76(6/. A8128.
c«carell M el Bl O.Scr,ption of the mow ,e119,e signs In nail lichen planu,.; A wategy to contnbutu to th• dlagno;;ls o f ~ sewre stage IJD 2022.611124-30
 • Ndil SoLich

Dl•r11111tolog~ Pl•arl,

Nail Lichen Planus Severity Index (NALSI): A Novel Severity Score for Nail
Lichen Planus

Chander Grover,
Geetall Kharghorla 1
Dt•1_1or111,,.,,, of D,nn,rro/n6,'
011d STD !.,11n-n Colll'f" of
M<1d,eal SaCl<t!II ,md Guru Ter
&,/,adur ffO!J)llal Sn, Del111
'D,parn,,,.,,, ofOmM1ol"f:\
anJ 1~'1lffl'IOII0!1\, All /,,d/:,
J,.,,,,u1,.
of .11.-iioJI S< ,..,..,.,,,
.\.-w !Nl/11. /n:!,a
 • ~dil SoL iclv o f lndkt

Dl'rnrntolog~ Pl·nrl,

Nail Lichen Planus Severity Index (NALSI): A Novel Severity Score for Nail
Lichen Planus

Chander Grover,
Geetall Kharghorla 1
Dq,arm,,.,,, of Dm,wolofl\
,111J STD c,,,,,.=,n Collf'I'• of
.\f,,J,ca/ Snolft'S a,id Gun, 7i,r
S,,harfur Horp110I .Vn, Del/11
'INpom,•mJ of0,'nltlllolOfl\

Table 1: Parameter, to he Recorded lo '.\ ail l.icbcn Plaou, SeHr il) lode~ ('\ \LSI) Scoring
'\111 M 11ri, Ohea,i' (0 I ) '\1111 8 ,d 0 1-«-a,l' (0 I) !\all f11ld Ol,r1M· (0 I ) Trrl\cr-lhll' C ha nge, (T)
l ong11udmnl ndgmg th~unng Crythem., (d11Tu-..! or long11udmal) P1gmcnu111on Dor.al p1eryg1um
Longi1uduul mdanon) cb1a D1,colora11on ()cllo,, 1\h. diffu~ ~\\dims Atropb)·
n1elanonych1a <h ~hrom1a)
Tbmrung tluckc:nmg ol thc: On} ,hl,I) ~1, (nod or ~ubungu:il ( ,~~•c LP lc:,1on~ o, c:r Anon}dun
nail plate: byperl.cmto~1, 1en11ng) the n:ul fold
Tmch)OD} clun Rullou, crtN\ e le-.mn, Ragged cuudc hang 111111 01s.nptX-nnng nn1l bed
Red mottled lunula
Treatment options
• Treatment is challenging and characterized by a
high rate of failures, relapses and recurrences
• No specific medications exist (unknown molecular
targets)
...•
•
• Clinicians reluctant to prescribe systemic treatments
when the disease is localized / mild / affects only the nails
,111 Dr 1.1,,1,1<1t- lori.uo f
• Quality of Life might be severely compromised
• Permanent nail plate destruction is possible (NLP as a nail emergency?)
Pimcon, BM er o/ Nail llchDn planin fNpotlff to l/'8lllmoot and long loml lolloW· UI) Eur J Oennatol 2010:20.489-96
G<»ttmann S ., al NLP .,,,clem,oiog,cal, cl.n•ul, p11/hclogical lhenpeut,c Mid progno.i• slud'f ol 67 c-• JEADV 2012 10.13CU-9
Lipner S NIM lreh,,n p/llll(JS • true na, 9"Hlf98"CY J Am Aud Dennatol 2019.al 77-8
 I] 1'dil 0 1.xic h

~
0nl ACITltmN
Matrix -, 0,2 - 0,l me/kc
NLP FEWER THAN LONI
...

~
A.lJTRtTINOIN
3 NAILS INVOLVED involvement 30mc
MILD • MODERATE

~
Oral ACITRmN
Bed 0,2 • 0,3 m&Jq
A.lJTR£TINOIN
Involvement
JOmc

NLP FEWER THAN

3 NAILS INVOLVED
MILD - MODERATE
Mat rix & a~ Involvement
...
.. '
~ I ACITllmN AZAnoo,111N£ 100mg/day .o1l n,"
,111 Dr ,.,,,rd,
--iii·~
-
. .itildt· lorizzo -
NLP FEWER THAN TIIIMICN...,._OIONI L 0,2 O,l mc/ka
AUTRmNOtN
MC::=~ 3mg/kg/day
_ __, 2-3gr/day
3 NAILS INVOLVED JOm1
SEVERE
.
NLP MORE THAN
3 NAILS INVOLVED
ANY PRESENTATION - '
 ~dil Socicl, 1..1! lndi~1

Treatment
• Topical treatment is generally NOT an option
(limited drug penetration, difficulty in maintaining patient
adherence, potential side effects due to long-term use)
• lntralesional I systemic steroids should be first line options
• Oral retinoids are second line choices
• lmmunsuppressants can be also considered

• PROMPTLY TREAT typical matrix NLP and bullous / erosive NLP

• DO NOT TREAT pterygium, anonychia, idiopathic atrophy

Pinlccir>, BAI et or NOii llchon plonu, ro~ponu to trootment and long term folJcN,, '-"' Eur J DermotOI 2010. 20·489 96

'°""
GotittmaM S et al Ni<// l,chen planus ep,,JOmio/or,c.il c£nicat. pathologicill theropaut,c and progfl0SI~ study of 87 case~. JEADV 2012. 10 1304.g
lonuo M, el al Isolated na"/lichen pta11us An 11xpe,t consensus on tre,Jl/nen/ of the cla»relll JAAD 2020 83 1717-23.
 • Ndil Si.x ich

lntralesional Treatment
lnlral.Nlanal Ethyl chloride spray, "talkesthesia," vibrating devices (for In
TRIAMCINOLONE Digital block anesthesia (for Intra Bed in/.)
ACETONIDE
l-10llllfflll Inject 0.1ml per quadrant with a 30G needle on a Luer Lo
Needle must be oblique to avoid Injecting too close to the
Inject until blanching from fluid load Is evident

--•'I:
'
I

'
I
a
•
•
Inject once a month for at least 6 months
Tapering is best performed by extending the period between injections
Also reducing the dose is fine

I
I ••
I
NO needleless injectors / beware of side effects
I
I I
••
t SUII not enough evidence regarding the optimal dosage, dilution,
I
30Gxh number and frequency of injections, and duration of treatment
0.301113mm
ye/lcw
Clitrl< A Jel 1r1111< NJ Intra 11<,onaJ /"J«f,on for /nflamm11fory Nltl Dt•e11•es DenT111tol Su,v 2016 , 2 257
Woon CY et ol. Hazards of steroid lnJociion auppurawe 11111onsor tendon rupture. Ind J PIDst Su,p 2010.,tJ·!n
Baran R Pro•mal nal fold ster'OKJ , nject,on responSJb/11 for Ho/gM syndrome. JEADV 20" 28·1563
Grover C, et el McolMJ syndrome follow,ng ,ntmmetnc/111 TA 1n1ect,on for NLP IDOJ 2017,8 350
 · ~dil Socicl,

WILEY A,- ...... )Hn(•-) 9 17(1U)

HCHNIQU U AND ,aoccouats
P!,OIUASIS (PSOl 16
lntralesional steroid injections for inflammatory nail M ILUettC., "-'' LS(MI) I•

dystrophies in the pediatric population

,.... __,,.udtrumt..a. 141(129 1........ llT_.jJ}

Mablde lonno MD PhD I I Nilton GioiA 0, Ch!Kchlo MD, PhDu

DRUCl:fflD
Nllton DI ChLKchlo MO, Ph0 2 I Chi..., Grover MO' I
Slw1 R. Lipner MO, PhD' I 8erVN Richert MO, PhD• I
,,_...,.
- -.._ -■ u.w-,
_ ,s...-i •
l,4
SIMia M.via Pkacdnl MD. Ph0 7 I Mlchd.1 St>rx. MO, PhD' Antonelb Tosd M0 1
, .... - ,... ..111---1 0 I ml 04.tl0 19ml)

Tew - ,... . . . - (...•) O.lal - Um1(07mO

•
3/30 patients < 12 years old

Beware of dose-related local adverse events

,,...,, -
,..,.,, .......
1'' " O F SESSIO!\"S

...,, ...
.......
II
7

'
Periungual skin of children thinner than adults AD\'l:MSll l:VL' ,11

• Max dose 0.2 ml per nail in <1 Syo ? ??

S)-
,.......
Nalllld ,.
0

......... Mil [old • .....,. •

2
rm.., 111111 r.w ~,...,.,.. 1111N 2
• Local anesthetic injections are one of the most 11.....- ....· , - I

anxiety-inducing stimuli in the pediatric patients TRI.Al \ltl>T U l IC.\C. Y

• Use should be discouraged 11.cMhod.•
l aprwYt:«ar:al •
19(91'$() 111'-LI')
9 (61'S0'3'•ll'I
w........ 2(11'50 l'Ll'I

·rso Cuo1bnclmhA'SklOIONAPSI 1·2- )-.1,P CrsnlPkustJP ■iJddj-

 i'.dil Socicl ,. of lnd1d

PAIN REUCED THANKS TO THE GATE CONTROL THEORY

When nerves receive non-painful signals (vibration/pressure) the brain closes the gate on pain signals
l,Mz tdr R wan PD Pam ~fl/"111$ II 11"W 1/leo,y Sot!t>Cfl 1965 150 971-9
Dupl,$t!a MJ. Florel K. Usmg an e/eelnc toothbrwh for Vlbtaoon anc~the!!ltl dunng padvl procedures Pad Dir Dormatol 2019.36 414-15

--~--
S«:11 A. et el EJrlcacy of v,brotJon on vv111puncturo p;,,n ,coro, 111 • p(ldotnc ame,pency dop,ytmont Pt1aatr Emerg CiJTII 2014.30:680-88

~ •-
-· ...
U,,Jtilrl.- lorozzo
,111 Dr U
 ~dil SoLich

Intramuscular Treatment
..........,
TRIAMCINOLONE
ACDONIDI!
•
•
•
lnJect once a month for at least 6 months
Higher dosages during the active treatment phase/severe disease
Tapering is best performed by extending the period between injections
Also reducing the dose is fine
O.S-1 nvKg
Rule out underlying conditions (diabetes, glaucoma, osteoporosis)
Discuss bone protrection (VitD & Calcium)

Deltoid Site VentroglutuJ Srte 21G I ½ • • 0,8 11 38 mm• grrien

Caplan A et or ProvetllJOII and

managem&nl ol glucocortK:oKJ.;nduaKI
side efTecJs A comptvllo,ns,ve ~ A
rov,ow of g/uoo(:MJcold pharmacology
and bone hall/th. JMD 2017·76:1-9
 · ~dil S1..xicb ol lnd1

Before and after 40 mg i.M. triamcinolone

· olo
 Nail 01..xich

Before and after 20mg/day ACITRETIN (0.2-0.Jmg/kg/day)

• AEs of retinoids include nail softening and brittleness which are unwanted in
NLP affected patients that already suffer from nail fragility
• Low dosages of acitretin are always recommended
• Best results in mild to moderate disease

Baran R Act,on /h.,apeu/Jque el corrf)l,ca/JOt1s du ret,nok» aromal,qw wr l'appared ungueal AM Dermalol Venereol 1982, 109.367-1
Baron R RellllO<d$ ond lhe Ml/$ J Dcnn/Jlol Trool 1990. 1'151-4
 ~dtl SOL id\

ALITRETINOIN: 9-cis-Retinoic acid

In NLP cellular immunity is impaired
(abnormal Ag presentation with recruitment of cytotoxic T-CDB+)
lsotretlnoln, acltretln
, An endogenous rebnoid 1.1 t.azarotone, trellnoln
, Ai,tret1n0111 bind1 to rerlllOld add receptors
iRAR) and ret notd X recei:1ors (RXR )
apart from other rellOOids !hat are spear,c
dg<>n IS to one rctnoid receptor' 7

' well-loleraled ]
only minimal side effects (headache)
teratogenic/ deaease TSH and T4
AJ,,.....,.
, Ai tret,, •0111 shows '4Ar4•11ifl•~~t
1mmunomodula:ory a!ld ,-..,.,.~oo,c ••tets
~rr-• tr«t1
.. ,~,.,.. ......,., ..,«t,
enu ~
elfucts" ""rl'Nil •'tectJ Of\ tltlill'\
:ir.lll~""tfl

It seems also to reduce the susceptibility of the epidermis to friction,

positively influencing the normal nail growth.

Cht,ng C. el al A/JlrolmOlfl a comprehori'1'rll ,v~,e,.. Expen Op,, lrN&WQ Drugs 2008, 17 437-43
RuzJCM T el al Eff,cecy end safety ol ebtre/,no,n ,n pe/,ools ...th se..,. chron,c hand eczema ,.fraclory to lopocal cort,costeroids 8.JD 2008 158 808· I 7
 • Ndil Socich

.
Novel Insights from Olnlcal Practice
Skin Appendage
D1sorder5 .,__......, ..,
- ~ - XDlllM17-4XI
00110.11-,0141 ..........,..,..11,..,
-.::..--. ,. lJllJ

Mycophenolate Mofetil in the Treatment

of Severe Corticosteroid-Dependent or
Corticosteroid-Resistant Nall Lichen Planus:
A Case Series of 5 Patients
Mycophenolate Mofetil 2-3mg/day
Alexandr, Ouplalne Olivier Cogrel
~dDonnoulloty liO!>r... SWC-..,0'\ldt_..,._,,onat
 Ndil S1..xich

JAK inhibitors in Nail Lichen Planus

• Elevated levels of IFNy, IL21 , CXCL9

and 10 are detected in LP tissues
• Targeting JAKs signaling, IFNy/lL21
mediated signaling is inhibited and
T-killer CD8+ and pro-inflammatory
cytokines recruitment reduced
• Keratinocytes (DEJ) are protected from
cytotoxic T cells induced apoptosis

Di Lemie V Tt1rpct1no rhe /FN-ylCXCL 10 pttthwey ,n lichen planus Med Hypot/HJstls 2016·92"60-1
Wenzel J. et 111 Golle expreUH){I PfO(H,11(} of LP reflects CXCL9+ rrHlcbled mnamma/JOtl llfld d•SlltlgUIIJwld th•• d.-11 .. from AD end PSO. J/0 2008. 128 87-78
Y..ng CC Ill el. Tofac/Jn b fo< the treatr11enl of hcht,r, planop,l.ms • cau Rnes O..nnalol Th11r 2018.31 012656
Pliml• J et et Tof110tm,b m rhtl rreetmont al lichen plnnopw,s • retrospoc;t/V9 ,ev,ew JAAD 2020. 83 1487-9
Domslry W. or o/ Treotmont of .soven, ~chen ploros M-1th rho JAK inhbror tofoor,nib JAi/argy Cl,n /mmunol 2020;1451708-10
 N...-iil Socicl, ol Ind tu

Case Reports > JAMA Dermatol 2021 Mar 1,157(3) 352-353

doi 101001/lamadermatol 2020 4555

Tofacitinib as Treatment for Nail Lichen Planus

Associated With Alopecia Universalis After 6 mo of
Smg/twice a day

,,.,
 • Ndil SoLicl ,. ~11 lnd1d

A. ~~ trf'lt~ffll 8 Compl~tf' d~aran.-~ lflff 6 months al trf'~l~ with bNIC'ltnb. 4 "'" di ly C, Sm.ti IKUfl'f'n(~ on th<- na,tt r. . fi,,r 4 nv,ths lfil'f
hlw1! cimm~ bir1trt1111b s dlily ~ to 2 ma (tol.ll of 14 rm nths of trr'ltmMI)

Piinche,. J Ulfftt• E TINlmenl ol S.-- Nu L.dien PtllnU$ With a.nu,n,b JAMA Derm.tol 2022 158 107-6

After 6 mo of baricitinib 4 mg/day

Baricitinib half life is 12.5 hours so a once a day intake might be sufficient
 ~dil SoLicl, ol lnd1d

I • I • citinib 15m I I • I I • I• I I I
 • Ndil Socicl, ol Ind kt

• Topical treatment is generally NOT an option

(limited drug penetration, difficulty in maintaining patient
adherence, potential side effects due to long-term use)
1,. , = -: ,. QI.I L--.,&,,,_._
2% Tofacitinib cream twice a day (compound)

Ho,Jong AM, et -1 Top,ca/ Janus kinase lnhibJcn

e ffW'- ol epp/JClllons ,n d&mlltlology JAAD
2018.1953544
t,..dil S1.xicl ,. ol lnd1<1
 i\dil s~xich ol lndid

TAKE HOME MESSAGE

• Fingernails respond better and quicker than toenails
The thumb nail responds less, and more slowly

• Not all patients, and not even all affected nails,

always respond to the given therapy

• The first prescribed successful treatment might not

be effective in case of recurrence/relapse

• Relapses of the nail lesions usually occur

1 to 4 years after clearing