vulvar lp and more: all vulvar erosions are not created equal s052... · vulvar lp and more: all...

2/19/2018

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Vulvar LP and More:

All vulvar erosions are not created equal

Bethanee J. Schlosser, MD, PhD

[email protected]

Depts. of Dermatology and OB/GYN

Northwestern University

Chicago, IL

February 19, 2018

Disclosure of Relationships with Industry

Elorac, Galderma: Investigator, Fees to Institution

Decision Support in Medicine, UpToDate®: Author, Honoraria

Allergan: Advisory Board, Speaker, Honoraria

Off-label use of medication will be discussed.


S052: Gender Dermatology

Vulvar LP and More

Happiness = Results – Expectations

• Results are dependent upon:

1) Provider expertise, knowledge

2) Patient engagement

• How many buckets do you have?

• Does the patient “fit” into one of your buckets?

Know what you know.

Know what you don’t know.

(And work to fill the gaps.)

Generating a Differential Diagnosis: 1st Think Big

EROSION

• Inflammatory

• Infectious

• Neoplastic

• Symptoms: itch + pain OR pain alone

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Generating a Differential DiagnosisEROSION

• Inflammatory• ACD, ICD• Drug eruption (EM, SJS, TEN)

• Fixed drug eruption• Atopic dermatitis (2° erosion)

• Psoriasis (2° erosion)• Lichen sclerosus (2° erosion)• Lichen planus

• Plasma cell vulvitis• Hailey-Hailey disease

• Pemphigus vulgaris• Bullous pemphigoid• Mucous membrane pemphigoid

• Linear IgA disease• Dermatitis herpetiformis

• Estrogen deficiency• Necrolytic migratory erythema• Langerhans cell histiocytosis

• Crohn’s disease

• Infectious• Vulvovaginal candidiasis• Tinea cruris

• Pubic lice• Scabies

• Impetigo• HSV• VZV

• Neoplastic• VIN, SCC• Extramammary Paget’s disease

• BCC

X X

To Biopsy or Not to Biopsy?

• Biopsy to support clinical diagnosis

• Multiple biopsies if needed (path, tissue cx)

• Utility and necessity of DIF

• Clinicopathologic correlation is key

• If no lesion = no biopsy

– Consider microbial cultures (fungal, bacterial)

Vulvar Biopsy Tips and Tricks

• Buffered, warmed lidocaine, 30g needle

– Inject slowly within the dermis/submucosa

• Wait 10 min before performing biopsy

• Get help – retraction, hemostasis, patient

• 4-8mm punch suture closure vs AgNO3

• Exceptions: clitoris, urethra, vagina

Clinicopathologic Correlation

Narrenbeschwörung by Thomas Murner, 1512

Vulvovaginal Lichen Planus

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• Chronic, idiopathic

• T-cell mediated, autoimmune disorder

• Prevalence:~1 in 4000

– Less common than LS

• Disease onset: peri/post-menopause

– May present during reproductive years

– Rare in children


• Pain, pruritus, dyspareunia, irritation1

– Can be asymptomatic

• 74% present with erosions2

• 25% have purulent vaginal discharge1

• >50% have significant scarring1

• Vaginal involvement is common!!

– Vaginal LP can occur isolation

1 Cooper SM, Wojnarowska F. Arch Dermatol 2006; 142: 289.2 Cooper SM et al. Arch Dermatol 2008; 144: 1520.3 Santegoets LA et al. J Low Genit Tract Dis 2010; 14: 323.

Vaginal Exam with Speculum

• Introital narrowing, foreshortening

• Vaginal walls – erythema, erosion, ulcer

• Vaginal discharge

• Water as lubricant

• Defer if vaginal creams used within 72hr

Normal saline wet mount microscopy is the

poor man’s speculum exam.

Vaginal Evaluation: Normal NaCl Wet Mount

• Epithelial cells: mature, immature

• WBCs: <1 per epithelial cell (or <10/HPF)

• Lactobacilli

• Clue cells

Normal Normal Saline Wet Mount

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Courtesy of Dr. Libby Edwards

Abnormal Normal Saline Wet Mounts Vulovaginal Lichen Planus• 25% of vulvovaginal LP have concomitant skin involvement

• 50% of cutaneous LP have genital disease

Vulovaginal Lichen Planus• Oral and vulvovaginal mucosa travel together

Mucosal Lichen Planus

• Conjunctiva

• Nasal mucosa

• Auditory canal

• Esophagus

• Larynx

• Vulva and vagina

• Urethra

• Anus

Scarring

Obstruction

Loss of function

ROS for Mucosal Lichen Planus

• Ocular foreign body sensation, eye pain, change in vision

• Nasal sores, epistaxis

• Decreased hearing, itching inside ears

• Dysphagia, odynophagia, globus sensation

• Change in voice, stridor

• Dysuria, decreased urinary stream

• Dyschezia, hematochezia, constipation


• Topical corticosteroids

– Super/potent corticosteroid ointment

– Vaginal steroid suppositories

• Hydrocortisone acetate 25mg

• Compounded corticosteroid

• Compounded cyclosporine

• Intralesional triamcinolone (3-10mg/ml)

• Topical calcineurin inhibitors

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Vulvovaginal Lichen Planus• Systemic corticosteroids

– Acute flares

– Rapid recurrence upon discontinuation

• Steroid-sparing systemic agents

– Methotrexate

– Mycophenolate mofetil

– Azathioprine

– Cyclosporine

– Tacrolimus

– Hydroxychloroquine

– Acitretin

Adjuvant Treatment

• Intravaginal estrogen therapy

• Vaginal dilator use, pelvic floor PT

– American Physical Therapy Association – Women’s Health

(ww.apta.org)

• Surgical intervention for vaginal scarring

• Psychosexual counseling

Course

• Chronic without cure

• Progressive

• Symptomatic improvement with super-potent topical

corticosteroid in 54-68%1,2

• Combined local/site-directed and systemic treatment

1 Cooper SM, Wojnarowska F. Arch Dermatol 2006: 142: 289.2 Kirtschig G, Wakelin SH, Wojnarowska F. J Eur Acad Dermatol Venereol 2005: 19: 301.

Vulvar LP and SCC

• Retrospective series of 95 women

– Patient encounters occurred over 7 years

– 2 developed vulvar SCC (2.1%)1

• Prospective study of 114 F with VVLP

– Mean follow-up = 72 months

– 1 developed vulvar SCC (0.8%)2

1 Santegoets LA et al. J Low Genit Tract Dis 2010; 14: 323.2 Cooper SM, Wojnarowska F. Arch Dermatol. 2006; 142: 289.

Vulvar Contact DermatitisWhen To Suspect Contact Dermatitis

• ANYTIME and ALL THE TIME

• Pre-existing vulvar dermatosis

– Not responding to treatment as expected

– Acute worsening of condition

• Vulvar pruritus, irritation, dysuria

• Incontinence, diarrhea

• Elderly, obese, those with limited mobility

• “Obsessives”, many product users

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Common Irritants• Body fluids

– Abnormal vaginal

discharge

– Feces

– Semen

– Sweat

– Urine

• Excessive bathing

– Soaps, detergents

• Feminine hygiene

– Depilatories

– Douches (acids, alkalis)

– Hygiene wipes

– Panty liners, pads

• Medications

– Fluorouracil

– Imiquimod

– Podophyllin

– Propylene glycol

– Spermicides

Adapted from Margesson LJ. Dermatol Clin 2006; 24: 145-55.

Most Common Allergens

• Neomycin1,2,3

• Caine anesthetics

– Benzocaine (esters) > Dibucaine (amides)3,5

• Fragrance3,5

• Balsam of Peru (Myroxylon pereirae)1

• Nickel1,4,5,6

• Corticosteroids

1 Marren P et al. Br J Dermatol 1992; 126: 52. 4 Nardelli A et al. Dermatitis 2004; 15: 131.

2 Doherty VR et al. Br J Dermatol 1990; 123: 26. 5 Brenan JA et al. Australas J Dermatol 1996; 37: 40.

3 Lewis FM et al. Am J Contact Dermat 1997; 137. 6 Lucke TW et al. Contact Dermatitis 1998; 38: 111.

Foote CA et al. J Low Genit Tract Dis 2014; 18: E16.

Pemphigus Vulgaris

• Genital involvement is “rare”

• Retrospective study of 34 patients

– 33 Caucasian (6 Jewish)

– Mean age 49yo (range 18-80yo)

– 21 vulva, 3 vagina, 10 vulva + vagina

– 100% had cutaneous PV

– 97% had oral PV

Malik M and Ahmed AR. Obstet Gynecol 2005; 106: 105. Malik M and Ahmed AR. Obstet Gynecol 2005; 106:1005.

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Female Genital Tract Involvement

• 41 pts with PV, 31 had active disease

Anatomic Site Mucosal (n=16) Mucocutaneous (n=15)

Vulva 1 6

Vagina 0 0

Uterine cervix 1 1

Oral 15 12

Pharynx 1 0

Larynx 3 0

Esophagus 1 2

Conjunctiva 0 1

Barbosa ND et al. J Am Acad Dermatol 2012; 67: 409.

Cervical Pemphigus

Pearls

• Just because things are rare, doesn’t mean they

don’t happen.

• If you never look, you’ll never find.

• Educate our colleagues in other specialties.

– For your patients with immunobullous disease, the

vagina and cervix need to be evaluated

Mucous Membrane Pemphigoid

• Chronic, inflammatory, autoimmune subepithelial

blistering disease of mucous membranes; +/- skin

involvement

• Autoantibodies to 10 BMZ antigens

• Erythematous patches, bullae, erosions

• Scarring

Mucous Membrane Pemphigoid

• Diagnosis

• Clinical presentation

• DIF of perilesional mucosa/skin with linear deposits of one or a

combination of IgG, IgA or C3 at the BMZ

• Treatment

• High vs low risk

• Prednisone with either cyclophosphamide or azathioprine

• Dapsone, mycophenolate mofetil, IVIg, etanercept

• Rituximab

• Topical corticosteroids

Pearls

• You never know until you look.

• Multidisciplinary collaboration is essential.

• The vagina is a potential space.

• Surgical lysis isn’t the end of treatment.

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Pearls – Post-Lysis Management

• Combination topical therapy

– Potent corticosteroid + topical estrogen

– In addition to systemic therapy

• Vaginal dilators

– Lubricants: H2O or silicone

– Amielle vaginal dilators

– www.vaginismus.com

• Diligence and commitment

THANK YOU


[email protected]

Northwestern University

vulvar lp and more: all vulvar erosions are not created equal s052... · vulvar lp and more: all...

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