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Nail lichen planus
Antonella TostiFredric Brandt Endowed Professor of
Dermatology&Cutaneous Surgery Miller School of Medicine, University of Miami
Antonella Tosti, MDF112 - Lichen Planus and Lichen Planopilaris: An Update
DISCLOSURESFotofinder :Consultant, Erconia Laser : PI, Springer & Verlag, CRC Press:
Author-Royalties , Karger : Editor in chiefThis presentation will discuss treatments that are not FDA approved
DISCLOSURE OF RELATIONSHIPS WITH INDUSTRY
PrevalenceNail lichen planus
rare adults > children both sexes equally affectedskin or mucosal LP (1-10% of patients)isolated nail involvement common
Tosti et al. J Am Acad Dermatol. 1993;28:724-30.
Tosti et al. Arch Dermatol. 2001;137:1027-32.
Different clinical varietiesNail lichen planus
“typical” nail matrix LPnail bed LPtrachyonychiaYNS-like featuresidiopathic atrophy of the nailsbullous-erosive LP
longitudinal ridging and fissuring : onychorrhexis
nail plate thinning
dorsal pterygium
“Typical” nail matrix LP
Dorsal pterygiummatrix destruction adhesion of PNF to nail bedrare (<5% of cases)usually 1 nailnot related to disease duration
“Typical” nail matrix LP
Onycholysis
Mild subungual hyperkeratosis
Pup tent sign
Usually associated with nail matrix LP
Nail bed LP
Clinically identical to T due to other conditions
Sandpapered nails
Benign course
Trachyonychia
Tickened yellowToenails
Patients usually have typical fingernalchanges
Yellow-nail syndrome-like changes
Tosti et al. Br J Dermatol 2000; 142:848-9.
rapid development nail atrophy with/without pterygium several/all nailsonset in young age frequent in Indians
Idiopathic atrophy of the nails
Tosti et al. Dermatology 1995; 190:116-8.
Extremely rare Painful nail erosions1-2 toenailsPathology needed for diagnosisScarring outcome
Bullous/erosive nail LP
Nail lichen planus in children
Not exceptional (11% of all patients with nail LP)Males > females‘Typical’ LP in half of patientsDorsal pterygium rare
Tosti et al. Arch Dermatol 2001; 137:1027-32.
Differential diagnosis
Nail lichen planus
age-related nail changessystemic amyloidosisgraft versus-host diseaselichen striatustraumatic nail lesionsbullous diseasespsoriasisyellow-nail syndrome
Age-related nail changesLPlongitudinal ridging but not fissuring
normal nail thickness
Systemic amyloidosisLP
longitudinal ridging and distal splittingsplinter hemorrhages
Graft versus host diseaselongitudinal ridging and distal splittinglongitudinal erythronychiadorsal pterygium
LP
Lichen striatus
Children
One nail
LP changes confined to oneside of the nail
Tosti A, Peluso AM, Misciali C, Cameli N. Nail lichen striatus: clinical features and long-term follow-up of five patients. J Am Acad Dermatol. 1997 Jun;36(6 Pt 1):908-13.
linear skin changes not always present
spontaneous regression
Lichen striatusLP
Traumatic nail dystrophies
Dorsal pterygium secondary to surgical removal of a band of melanonychia
pterygium due to surgery or acute nail traumas
Bullous diseasesnail destruction
LP
Epidermolysis bullosaBullous pemphigoid
Diagnosis is based on clinical history and pathology
Psoriasis LP
Diagnosis may require pathology
Psoriasis
PathologyThe site of biopsy should be chosen according to the clinical features
Nail matrix biopsy Nail bed biopsy
A longitudinal nail biopsy is always diagnostic
Treatment
TREAT DO NOT TREAT
“typical” nail matrix LP dorsal pterygium nail bed LP YNS-like featuresidiopathic atrophy of the nailsbullous-erosive LPtrachyonychia?????
Not all clinical varieties should be treated
Treatment
Systemic steroidsIntralesional steroidsAcitetrinBiologicsJak inhibitorsTopical tacrolimus
Treatment
“typical” nail matrix LPnail bed LPYNS-like featuresbullous-erosive LP
Systemic or intralesional steroids depending on number of nails involved
Lichen planus of the nails involving 1-3 digits is an indication for intralesional steroid treatment
Systemic steroids i.m. triamcinolone acetonide 0.5 mg/kg/monthfor 3-4 months then tapered off
Treatment of nail lichen planus with systemic steroids
cure: 44 (66%) improvement: 9 (13%)no response: 14 (21%)
67 patients
Patients who did nor respond systemic steroids did not improve with the addition of azathioprine nor with treatment with systemic retinoids
Piraccini BM, Saccani E, Starace M, Balestri R, Tosti A. Nail lichen planus: response to treatment and long term follow-up. Eur J Dermatol. 2010 Jul-Aug;20(4):489-96
Treatment of nail lichen planus
Irla N, Schneiter T, Haneke E, Yawalkar N. Nail Lichen Planus: Successful Treatment with Etanercept. Case Rep Dermatol. 2010 Oct 21;2(3):173-176.
Trachyonychia
Patients with severe nail disease can seek for treatment
Efficacy of tofacitinib recently reported
Treatment of nail lichen planusJak inhibitors
1: Jaller et al. Recovery of nail dystrophy potential new therapeutic indication of tofacitinib. Clin Rheumatol. 2017 Feb 16..
2: Ferreira al Remarkable Improvement of Nail Changes in Alopecia Areata Universalis with 10 Months of Treatment with Tofacitinib: A Case Report. Case Rep Dermatol. 2016 Oct 4;8(3):262-266.3: Dhayalan A, King BA. Tofacitinib Citrate for
the Treatment of Nail Dystrophy Associated With Alopecia Universalis. JAMA Dermatol. 2016 Apr;152(4):492-3.
Treatment of nail lichen planusJak inhibitors
Six patients published until now, none with trachyonychia due to LP
All of them also had trachyonychia and alopecia areata
Two required more than 5 mg twice a day
Nail improved even in patients who did nor regrow hair
Treatment of nail lichen planusJak inhibitors
Jak inhibitors
No information on long term benefit
Alopecia areata relapses after drug discontinuation and even during treatment
Seems aggressive approach for a benign disease
Treatment of nail lichen planus
Long term follow- up (>5 years) of patients with nail LP
cure: 12 pts (44%)mild thinning and ridging: 2 pts (7%) relapsed: 13 (48%)
Outcome27 patients
Associated diseases
Long term follow- up (>5 years) of patients with nail LP
4 patients developed onychomycosis(3 toenail DSO, 1 fingernail DSO) cured by systemic antifungals
Predisposing role of steroids or LP?
Nail lichen planus
Several clinical presentationsPathology needed to confirm diagnosisNail matrix LP requires immediate treatment to prevent scarringSystemic or intralesional steroids effectiveRelapses may occur
Take home message
Thank [email protected]