frm f112 - tosti - 12963 10434 - american … naillichen planus rare adults > children both sexes...

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 you!atosti@med.miami.edu