THE ITCHY VULVA

LICHEN SCLEROSUS (LS)

L.J. Margesson, MD + G. Davis, MDL.J. Margesson, MD + G. Davis, MD

Introduction Lichen Sclerosus

A common chronic vulvar diseaseA common chronic vulvar disease

An inflammatory skin conditionAn inflammatory skin condition

Prevalence 1 in 300 to 1 in 1,000Prevalence 1 in 300 to 1 in 1,000

Most commonly found in middle-age Most commonly found in middle-age women, but it can be seen in very young women, but it can be seen in very young children and the elderlychildren and the elderly

Etiology of LS

UnknownUnknown

MultifactorialMultifactorial - genetic - genetic

- autoimmune- autoimmune

- environmental factors- environmental factors

NOTE: Often associated with autoimmune NOTE: Often associated with autoimmune conditions, e.g. thyroid disease, vitiligo, etc.conditions, e.g. thyroid disease, vitiligo, etc.

Familial cases have been reportedFamilial cases have been reported

Clinical Findings LS

SYMPTOMS:SYMPTOMS:Most common Most common

- pruritus- pruritus- can be severe, intolerable- can be severe, intolerable

Can have soreness and burningCan have soreness and burningOften asymptomaticOften asymptomatic

Scratching results in open areas causing Scratching results in open areas causing dysuria, pain, dyspareunia, etc.dysuria, pain, dyspareunia, etc.

Clinical Findings LS

PHYSICAL EXAMINATION:PHYSICAL EXAMINATION:

Ivory white papules or confluent plaquesIvory white papules or confluent plaques

Cellophane-like sheen to surfaceCellophane-like sheen to surface

Patchy or generalized - anywhere Patchy or generalized - anywhere

vulva, perineum, perianalvulva, perineum, perianal

Disease is not in the vaginaDisease is not in the vagina

Clinical Findings LS PHYSICAL EXAMINATION PHYSICAL EXAMINATION Secondary changesSecondary changes

- scratches, purpura, erosion- scratches, purpura, erosion- crusting, thickening (lichenification)- crusting, thickening (lichenification)

- scarring with loss of normal - scarring with loss of normal

architecture; fusion of labia minoraarchitecture; fusion of labia minora

-phimosis-phimosis

-introital stenosis-introital stenosis

NOTE: LS can be associated with squamous cell carcinoma of NOTE: LS can be associated with squamous cell carcinoma of the vulva. Any open, raw, non-healing lesions must be assessed the vulva. Any open, raw, non-healing lesions must be assessed and biopsied.and biopsied.

Case History LS

35-year-old woman with vulvar itching for 35-year-old woman with vulvar itching for 2½ years2½ years

- treated repeatedly for yeast infections- treated repeatedly for yeast infections

- topical products burn her- topical products burn her

- was told the area looked thinned and - was told the area looked thinned and pale; questionable pre-menopausepale; questionable pre-menopause

Case LS

PHYSICAL EXAMINATION:PHYSICAL EXAMINATION:

35-year-old woman35-year-old woman

No other skin rashesNo other skin rashes

Bilaterally symmetrical white rashBilaterally symmetrical white rash

Extends from supra-clitoral to perianal areaExtends from supra-clitoral to perianal area

Slight scarring around the clitorisSlight scarring around the clitoris

Diagnosis LS

Clinical PatternClinical Pattern

BiopsyBiopsy

Histopathology LS

Thinned epidermis +/- Thinned epidermis +/- hyperkeratosishyperkeratosis

In reticular dermis, a band of In reticular dermis, a band of homogenized collagenhomogenized collagen

Lymphocytic infiltrate under Lymphocytic infiltrate under the bandthe band

In individuals who scratch In individuals who scratch chronically, histologic chronically, histologic changes of squamous cell changes of squamous cell hyperplasia can be seen in hyperplasia can be seen in addition to LS changesaddition to LS changes

Treatment of LS I

Thorough assessmentThorough assessmentBiopsy to confirm diagnosisBiopsy to confirm diagnosisStop irritantsStop irritantsEducationEducationSuperpotent steroid ointment clobetasol Superpotent steroid ointment clobetasol

0.05% ointment bid x 1 month, then qhs x 2 0.05% ointment bid x 1 month, then qhs x 2 months. Apply in a thin film. Maintenance months. Apply in a thin film. Maintenance dose is 1 to 3 times per week indefinitelydose is 1 to 3 times per week indefinitely

Treat secondary infection, particularly yeastTreat secondary infection, particularly yeast

Treatment of LS

Stop scratchingStop scratching

Consider sedation, if necessaryConsider sedation, if necessary

If very thick, consider intralesional If very thick, consider intralesional triamcinolone acetonidetriamcinolone acetonide

If not responding, reassess, rebiopsy If not responding, reassess, rebiopsy to R/O SCC, R/O contact dermatitisto R/O SCC, R/O contact dermatitis

Set up long-term followupSet up long-term followup

References LS

Dalziel K, Shaw S. Dalziel K, Shaw S. Lichen sclerosus.Lichen sclerosus. BMJ. BMJ. 2010;340:c731. 2010;340:c731.

Lynch PJ, Moyal-Barrocco M, Bogliatto F, Lynch PJ, Moyal-Barrocco M, Bogliatto F, Micheletti L, Scurry J. 2006 ISSVD classification Micheletti L, Scurry J. 2006 ISSVD classification of vulvar dermatoses: pathologic subsets and of vulvar dermatoses: pathologic subsets and their clinical correlates. Journal of their clinical correlates. Journal of Reproductive Medicine.Reproductive Medicine. 2007;52(1):3-9. 2007;52(1):3-9.

References LS (cont’d)

Lynch, PJ. 2006 International Society for the Lynch, PJ. 2006 International Society for the Study of Vulvovaginal Disease Study of Vulvovaginal Disease Classification of Vulvar Dermatoses: A Classification of Vulvar Dermatoses: A Synopsis. J Low Gen Tract Dis Synopsis. J Low Gen Tract Dis 2007;11(1):1-2.2007;11(1):1-2.

Saunders NA, Haefner HK. Vulvar lichen Saunders NA, Haefner HK. Vulvar lichen sclerosus in the elderly: pathophysiology sclerosus in the elderly: pathophysiology and treatment update. Drugs & Aging. and treatment update. Drugs & Aging. 22009;6(10):803-12.22009;6(10):803-12.

References LS (cont’d)

Smith SD, Fischer G. Paediatric vulval Smith SD, Fischer G. Paediatric vulval lichen sclerosuslichen sclerosus Australasian Journal of Australasian Journal of Dermatology. Dermatology. 2009;50(4):243-8. 2009;50(4):243-8.

van der Avoort IA, Tiemes DE, van Rossum van der Avoort IA, Tiemes DE, van Rossum MM, van der Vleuten CJ, MM, van der Vleuten CJ, Massuger LF, de Massuger LF, de Hullu JA. Hullu JA. Lichen sclerosus: treatment Lichen sclerosus: treatment and follow-up at the departments of and follow-up at the departments of gynaecology and dermatology. Journal gynaecology and dermatology. Journal of Lower Genital Tract Disease. of Lower Genital Tract Disease. 2010;14(2):118-23. 2010;14(2):118-23.

THE ITCHY VULVA

SQUAMOUS CELL HYPERPLASIALICHEN SIMPLEX CHRONICUS (LSC)

L. J. Margesson, MD + G. Davis, MDL. J. Margesson, MD + G. Davis, MD

Squamous Cell HyperplasiaSquamous Cell Hyperplasia

IsIs

Lichen Simplex ChronicusLichen Simplex Chronicus

Introduction - LSCWith this condition there is chronic, With this condition there is chronic,

intense itching that results in intense itching that results in repetitive scratching and rubbingrepetitive scratching and rubbing

The skin responds by thickening The skin responds by thickening (lichenification). The thickening of (lichenification). The thickening of the skin is caused by the scratchingthe skin is caused by the scratching

An itch-scratch-itch cycle starts and An itch-scratch-itch cycle starts and perpetuates the problemperpetuates the problem

Etiology LSC I

LSC develops in several itchy skin LSC develops in several itchy skin conditions:conditions:

Atopic dermatitis (eczema)Atopic dermatitis (eczema)

Contact dermatitisContact dermatitis

Lichen sclerosusLichen sclerosus

Contact dermatitis can start this condition or Contact dermatitis can start this condition or be the main long-term promoting factorbe the main long-term promoting factor

Etiology LSC II

Scratching and rubbing damage the skin so Scratching and rubbing damage the skin so it loses its protective coating / barrierit loses its protective coating / barrier

Result is:Result is: susceptibility to infectionsusceptibility to infection

ease of irritationease of irritation

more itchingmore itching

Clinical Findings LSC

SYMPTOMS:SYMPTOMS:Relentless pruritusRelentless pruritusYears of itchYears of itchItch eventually developing into burning Itch eventually developing into burning

painpainWorse with heat, stress, periods and Worse with heat, stress, periods and

tight synthetic clothingtight synthetic clothingWake up at night scratchingWake up at night scratching

Clinical Findings LSCPHYSICAL EXAMINATION:PHYSICAL EXAMINATION:Thick, lichenified skin so labia are enlarged, rugose Thick, lichenified skin so labia are enlarged, rugose

+/- edematous+/- edematousBilateral or unilateralBilateral or unilateralLocalized or generalizedLocalized or generalizedColor - variably pink, red, violaceous to ruddy brown; Color - variably pink, red, violaceous to ruddy brown;

often a white appearance will be present when often a white appearance will be present when there is a thick keratin layer deposited on the there is a thick keratin layer deposited on the surface of the epitheliumsurface of the epithelium

Secondary changes - erosions, ulcers, oozing, Secondary changes - erosions, ulcers, oozing, fissuring, honey-colored or serosanguineous fissuring, honey-colored or serosanguineous crustingcrusting

CASE HISTORY: LSC

26-year-old woman with intense vulvar 26-year-old woman with intense vulvar pruritus for 2½ yearspruritus for 2½ years

Wakes up at night scratchingWakes up at night scratching

Nothing helpsNothing helps

Most creams burn herMost creams burn her

Has allergic rhinitis and asthmaHas allergic rhinitis and asthma

Case LSC

PHYSICAL EXAMINATION:PHYSICAL EXAMINATION:A healthy, 26-year-old womanA healthy, 26-year-old womanNo other skin changesNo other skin changesOn the inner labia majora, interlabial On the inner labia majora, interlabial

sulcus and perineum is a bilateral, sulcus and perineum is a bilateral, symmetrical, pink, lichenified symmetrical, pink, lichenified eruptioneruption

Some excoriations seenSome excoriations seen

DIAGNOSIS LSC

ClinicalClinical

Do biopsy to R/O underlying conditionDo biopsy to R/O underlying condition

Culture for secondary infection Culture for secondary infection

- yeast and bacteria- yeast and bacteria

Consider patch testingConsider patch testing

Histopathology LSC

HyperkeratosisHyperkeratosis

AcanthosisAcanthosis

Lengthened broad Lengthened broad rete ridgesrete ridges

Chronic Chronic inflammatory inflammatory infiltrateinfiltrate

Treatment LSC I

Stop the itch-scratch-itch cycleStop the itch-scratch-itch cycle

Sitz baths and soaks, no irritantsSitz baths and soaks, no irritants

Reduce inflammation with superpotent steroids, Reduce inflammation with superpotent steroids, i.e., clobetasol or halobetasol ointment i.e., clobetasol or halobetasol ointment

- bid for two weeks, - bid for two weeks, - once a day for two weeks and - once a day for two weeks and - M / W / F for two weeks- M / W / F for two weeks

Intralesional steroids, if severeIntralesional steroids, if severe

Treatment LSC I

Intralesional steroidsIntralesional steroids

-5 mg of triamcinolone suspension in 2 ml of saline -5 mg of triamcinolone suspension in 2 ml of saline subcutaneouslysubcutaneously

-Use a 3-inch, 22-gauge spinal needle inserted in the -Use a 3-inch, 22-gauge spinal needle inserted in the lower mons pubis and then passed down to the lower mons pubis and then passed down to the perineum perineum

-As the needle is withdrawn, the solution is slowly -As the needle is withdrawn, the solution is slowly injected and then massaged into the tissueinjected and then massaged into the tissue

Systemic steroids are seldom neededSystemic steroids are seldom needed

Treatment LSC

Control infection - cefadroxil 500 mg Control infection - cefadroxil 500 mg bid for 7 days, fluconazole 150 mg bid for 7 days, fluconazole 150 mg repeated one week laterrepeated one week later

Sedation: doxepin or hydroxyzine Sedation: doxepin or hydroxyzine 25-10 mg qhs, SSRI like fluoxetine 20 25-10 mg qhs, SSRI like fluoxetine 20 mg q ammg q am

References LSC

O'Connell TX, Nathan LS, Satmary WA, O'Connell TX, Nathan LS, Satmary WA, Goldstein AT. Non-neoplastic epithelial Goldstein AT. Non-neoplastic epithelial disorders of the vulva. American Family disorders of the vulva. American Family Physician. 2008;77(3):321-6. Physician. 2008;77(3):321-6.

Burrows LJ, Shaw HA, Goldstein AT. The Burrows LJ, Shaw HA, Goldstein AT. The vulvar dermatoses. Journal of Sexual vulvar dermatoses. Journal of Sexual Medicine. 2008;5(2):276-83.Medicine. 2008;5(2):276-83.

THE ITCHY VULVA

LICHEN PLANUS (LP)

L. J. Margesson, MD + G. Davis, MDL. J. Margesson, MD + G. Davis, MD

Introduction LP

An inflammatory, mucocutaneous eruptionAn inflammatory, mucocutaneous eruption

With a distinctive pattern on:With a distinctive pattern on:

- skin, scalp, nails- skin, scalp, nails

- mucous membranes - mucous membranes oral, genital, esophageal, etc.oral, genital, esophageal, etc.

Etiology LP

UnknownUnknown

? Autoimmune triggered by exogenous ? Autoimmune triggered by exogenous antigens, possiblyantigens, possibly

- viral - viral

- bacterial (superantigen)- bacterial (superantigen)

- chemical- chemical

- drug- drug

- trauma- trauma

Clinical Findings LP

SYMPTOMSSYMPTOMSMost often there is irritation with burning and Most often there is irritation with burning and

sorenesssorenessCan be very itchy, and scratching flares itCan be very itchy, and scratching flares itWith scratching, the vulva gets very thick and With scratching, the vulva gets very thick and

scarredscarredStretching of scarring causes dyspareuniaStretching of scarring causes dyspareuniaSymptoms depend on extent of disease - e.g. Symptoms depend on extent of disease - e.g.

when vagina is involved with erosions, there when vagina is involved with erosions, there is discharge, burning, etc.is discharge, burning, etc.

Clinical LP

PHYSICAL EXAMINATION PHYSICAL EXAMINATION

Variable patternsVariable patterns

Lacey, reticulated pattern on the Lacey, reticulated pattern on the labia, vulvar trigone, perineum or labia, vulvar trigone, perineum or perianal area with or without scarring perianal area with or without scarring and erosions.and erosions.

Small reddish papules may be seenSmall reddish papules may be seen

Clinical LPPHYSICAL EXAMINATION PHYSICAL EXAMINATION Thick, white, indurated plaques anywhere from Thick, white, indurated plaques anywhere from

clitoris to anus – as in lichen sclerosusclitoris to anus – as in lichen sclerosusSecondary changes: excoriations, crusting, Secondary changes: excoriations, crusting,

scarringscarringIf ulcerative: If ulcerative:

vulva - glazed erythema, erosions, ulcers vulva - glazed erythema, erosions, ulcers vagina - synechiae, scarring, stenosis, vagina - synechiae, scarring, stenosis,

telescoping, bleeding, dischargetelescoping, bleeding, discharge

Diagnosis LP

Look at rest of skin and mucous membranesLook at rest of skin and mucous membranes

Look in the mouthLook in the mouth

BiopsyBiopsy

- regular histopathology (H&E)- regular histopathology (H&E)

- immunofluorescence- immunofluorescence

Stop topical steroids for 1-2 weeks before Stop topical steroids for 1-2 weeks before biopsybiopsy

Case LP

A 42-year-old woman presented with A 42-year-old woman presented with severe itching and burning on her severe itching and burning on her vulva for 18 months vulva for 18 months

Topical treatment for yeast and herpes Topical treatment for yeast and herpes simplex burned hersimplex burned her

Topical cortisone-no helpTopical cortisone-no help

Initial dyspareunia now apareuniaInitial dyspareunia now apareunia

Case LP

PHYSICAL EXAMINATIONPHYSICAL EXAMINATION

Lacey white pattern of involvement in Lacey white pattern of involvement in interlabial sulciinterlabial sulci

Peri-clitoral whitish plaquePeri-clitoral whitish plaque

Ulceration of clitoris and interlabial sulciUlceration of clitoris and interlabial sulci

Early scarring of the clitorisEarly scarring of the clitoris

Histopathology LP

HyperkeratosisHyperkeratosis

Prominent granular layerProminent granular layer

Irregular acanthosis and sawtooth Irregular acanthosis and sawtooth patternpattern

Destruction of basal layerDestruction of basal layer

Band-like inflammatory infiltrateBand-like inflammatory infiltrate

Treatment LP Challenging - no single agent is universally Challenging - no single agent is universally

effectiveeffectiveStop irritation and traumaStop irritation and traumaTreat infectionTreat infectionRestore barrier function with Sitz bath or tub Restore barrier function with Sitz bath or tub

bath 1- 2 times a daybath 1- 2 times a dayReduce inflammation with topical superpotent Reduce inflammation with topical superpotent

corticosteroids corticosteroids clobetasol 0.05% clobetasol 0.05% ointment 1- 2 timesointment 1- 2 times

a daya day

Treatment LP

For the vagina - hydrocortisone For the vagina - hydrocortisone acetate foam (80 mg) at night or in a acetate foam (80 mg) at night or in a compounded suppository 100 mgcompounded suppository 100 mg

For localized disease consider For localized disease consider intralesional triamcinoloneintralesional triamcinolone

Treatment LPSevere Lichen Planus control:Severe Lichen Planus control:Prednisone 1 - 1.5 mg per kg per day for 2 weeks and Prednisone 1 - 1.5 mg per kg per day for 2 weeks and

tapering over 2-4 months ortapering over 2-4 months orIM triamcinolone 1 mg per kg q 4 weeks for maximum IM triamcinolone 1 mg per kg q 4 weeks for maximum

three injections not to exceed 80 mg per monththree injections not to exceed 80 mg per monthAdd cyclosporine 4 mg per kg per day and continue Add cyclosporine 4 mg per kg per day and continue

until the patient is clear, then wean onto Plaquenil until the patient is clear, then wean onto Plaquenil 200 mg bid and / or hydrocortisone acetate 200 mg bid and / or hydrocortisone acetate vaginallyvaginally

Other drugs to consider: doxycycline, metronidazole, Other drugs to consider: doxycycline, metronidazole, acitretin, methotrexate, azathioprine and tacrolimusacitretin, methotrexate, azathioprine and tacrolimus

Treatment LP

Tacrolimus 0.03% and 0.1% ointment can be Tacrolimus 0.03% and 0.1% ointment can be used topically but the Protopic brand can used topically but the Protopic brand can sting and must be sting and must be usedused only on areas under only on areas under control with topical steroids - used as steroid control with topical steroids - used as steroid sparerssparers

Custom formulas are available for suppository Custom formulas are available for suppository and intravaginal creams and ointmentsand intravaginal creams and ointments

Prognosis LP

38% complete resolution38% complete resolution

30% significant resolution30% significant resolution

32% ongoing problems32% ongoing problems

Lichen SclerosusLichen Sclerosus Lichen Planus Lichen Simplex Chronicus Lichen Planus Lichen Simplex Chronicus

Itch or burnItch or burn Itch or burn Itch or burn Severe itch Severe itchScarsScars Scars Scars No scarring No scarringOn the vulvaOn the vulva On vulva and On vulva and On the vulva On the vulva

in vaginain vagina

Oral lesionsOral lesions frequently seenfrequently seen

References LPReferences LP

Baldo M. Bailey A, Bhogal B, Groves RW, Ogg G, Baldo M. Bailey A, Bhogal B, Groves RW, Ogg G, Wojnarowska F. T cells reactive with the NC16A Wojnarowska F. T cells reactive with the NC16A domain of BP180 are present in vulval lichen domain of BP180 are present in vulval lichen sclerosus and lichen planus. Journal of the sclerosus and lichen planus. Journal of the European Academy of Dermatology & European Academy of Dermatology & Venereology. 2010;24(2):186-90. Venereology. 2010;24(2):186-90.

Byrd JA, Davis MD, Rogers RS 3rd. Recalcitrant Byrd JA, Davis MD, Rogers RS 3rd. Recalcitrant symptomatic vulvar lichen planus: response to symptomatic vulvar lichen planus: response to topical tacrolimus. Archives of Dermatology. topical tacrolimus. Archives of Dermatology. 2004;140(6):715-20.2004;140(6):715-20.

References LP (cont’d)

Cooper SM, Ali I, Baldo M, Wojnarowska F. The Cooper SM, Ali I, Baldo M, Wojnarowska F. The association of lichen sclerosus and erosive association of lichen sclerosus and erosive lichen planus of the vulva with autoimmune lichen planus of the vulva with autoimmune disease: a case-control study. Archives of disease: a case-control study. Archives of Dermatology. 2008;144(11):1432-5.Dermatology. 2008;144(11):1432-5.

Cooper SM, Wojnarowska F. Influence of Cooper SM, Wojnarowska F. Influence of

treatment of erosive lichen planus of the vulva treatment of erosive lichen planus of the vulva on its prognosis. Archives of Dermatology. on its prognosis. Archives of Dermatology. 2006;142(3):289-94.2006;142(3):289-94.

References LP (cont’d)

Goldstein AT, Metz A. Vulvar lichen planus. Goldstein AT, Metz A. Vulvar lichen planus. Clinical Obstetrics & Gynecology. Clinical Obstetrics & Gynecology. 2005;48(4):818-23.2005;48(4):818-23.

Kennedy CM, Peterson LB, Galask RP. Kennedy CM, Peterson LB, Galask RP. Erosive vulvar lichen planus: a cohort at Erosive vulvar lichen planus: a cohort at risk for cancer? Journal of Reproductive risk for cancer? Journal of Reproductive Medicine. 2008;53(10):781-4. Medicine. 2008;53(10):781-4.

References LP (cont’d)

Kennedy CM, Galask RP. Erosive vulvar Kennedy CM, Galask RP. Erosive vulvar lichen planus: retrospective review of lichen planus: retrospective review of characteristics and outcomes in 113 characteristics and outcomes in 113 patients seen in a vulvar specialty clinic. patients seen in a vulvar specialty clinic. Journal of Reproductive Medicine. Journal of Reproductive Medicine. 2007;52(1):43-7. 2007;52(1):43-7.