Continuing medical education
Vulvar diseases: Conditions in adults and children

https://doi.org/10.1016/j.jaad.2019.10.077Get rights and content

The most problematic vulvovaginal conditions are familiar to dermatologists but may exhibit distinct clinical features or medication management because of the anatomic location. The second article in this continuing medical education series focuses on management pearls for treating vulvar diseases. We highlight key conditions, such as lichen sclerosus, erosive lichen planus, and vulvodynia. In addition, we review conditions that dermatologists may be less familiar with, such as plasma cell vulvitis, desquamative inflammatory vaginitis, vulvar aphthae, and low estrogen states. Nearly 1 in 6 women experience undiagnosed and untreated vulvovaginal discomfort at some point in their lives. Physicians who treat vulvar disorders will improve the quality of life of countless women.

Section snippets

Lichen sclerosus

Key points

  1. First-line therapy is a daily ultrapotent topical corticosteroid until the texture normalizes

  2. Maintenance therapy with a midpotency topical corticosteroid, such as daily triamcinolone ointment 0.1%, or an ultrapotent agent, such as clobetasol ointment 0.05%, 3 times weekly decreases the risk of squamous cell carcinoma

  3. Treatment should be ongoing rather than according to symptoms

Lichen sclerosus (LS) is a chronic inflammatory condition that preferentially affects the anogenital region. Autoimmune

Erosive lichen planus

Key points

  1. Treat erosive vulvovaginal lichen planus with ultrapotent daily topical corticosteroids

  2. Women with erosive vaginal lichen planus require aggressive management, including compounded vaginal corticosteroid suppositories and vaginal dilators to prevent stenosis

  3. Chronic therapy is required for optimal control and should be individualized

Erosive vulvovaginal lichen planus (EVVLP) is a common, painful, erosive condition found most often in postmenopausal women and is usually accompanied by oral

Plasma cell vulvitis

Key points

  1. Plasma cell vulvitis (Zoon vulvitis) is an uncommon but distinctive dermatosis of unknown cause that must be distinguished from EVVLP and differentiated vulvar intraepithelial neoplasia

  2. The management of plasma cell vulvitis consists primarily of chronic ultrapotent or intralesional corticosteroids and moderately improves but rarely resolves

Plasma cell vulvitis, also called Zoon vulvitis, is a distinctive, idiopathic dermatosis. Plasma cell mucositis can also affect the oral cavity and the glans

Desquamative inflammatory vaginitis

Key points

  1. Desquamative inflammatory vaginitis is a unique idiopathic inflammatory mucositis that occurs only in the vagina

  2. The diagnosis is made clinically after excluding infection, specific erosive skin disease, and estrogen deficiency

  3. Management involves intravaginal corticosteroids or clindamycin cream requiring recurrent or chronic dosing

Desquamative inflammatory vaginitis (DIV) is a unique vaginal mucositis that presents with purulent copious discharge as well as introital itching, irritation, and

Vulvar Crohn's disease

Key points

  1. Metastatic Crohn's disease of the vulva characteristically presents with vulvar edema or distinctive knife-cut ulcerations

  2. The course of metastatic cutaneous Crohn's disease may diverge from that of intestinal disease

  3. Fecal calprotectin level can be useful in making the diagnosis

Anogenital Crohn's disease requires familiarity with its distinctive ulcer morphology and a high index of suspicion when confronted with edema and draining nodules. The cutaneous presentation is protean: classic

Vulvar aphthae

Key points

  1. The diagnosis of aphthous ulcers (Lipschutz ulcers, non–sexually acquired genital ulcerations) should be considered in the appropriate clinical context after exclusion of infectious etiologies

  2. Herpes simplex virus infection in an immunosuppressed person can be indistinguishable from an aphthous ulcer

  3. Most patients with oral and genital aphthae do not meet diagnostic criteria for Behçet disease because this condition requires objective documentation of multisystem disease including the eye, gut,

Atrophic vagina

Key points

  1. Atrophic vagina, now renamed the genitourinary syndrome of menopause, results from estrogen deficiency and can manifest as vaginal dryness, itching, burning, dyspareunia, and urinary symptoms

  2. Symptomatic women are readily treated with intravaginal or systemic estrogen

Atrophic vagina is an older term used to describe a vaginal low-estrogen state in adult women and has been renamed genitourinary syndrome of menopause (GSM) when women are symptomatic.49 GSM occurs in postmenopausal women occurring

Vulvodynia

Key points

  1. Vulvodynia is a common underrecognized syndrome of vulvar pain of ≥3 months' duration without a definable cause

  2. Obtaining routine biopsy specimens of focal areas of pain or redness are not informative and should be avoided

  3. Vulvodynia represents a central neuropathic pain/processing disorder accompanied by pelvic floor muscle dysfunction and comorbid anxiety/depression to varying degrees

The prevalence of vulvodynia in the United States is estimated to be 8%.50,51 Its etiology is multifactorial,

References (59)

  • V.N. Sehgal et al.

    Nonspecific genital ulcers

    Clin Dermatol

    (2014)
  • S. Dixit et al.

    Management of nonsexually acquired genital ulceration using oral and topical corticosteroids followed by doxycycline prophylaxis

    J Am Acad Dermatol

    (2013)
  • J.A. Letsinger et al.

    Complex aphthosis: a large case series with evaluation algorithm and therapeutic ladder from topicals to thalidomide

    J Am Acad Dermatol

    (2005)
  • B.D. Reed et al.

    Prevalence and demographic characteristics of vulvodynia in a population-based sample

    Am J Obstet Gynecol

    (2012)
  • B.L. Harlow et al.

    Prevalence of symptoms consistent with a diagnosis of vulvodynia: population-based estimates from 2 geographic regions

    Am J Obstet Gynecol

    (2014)
  • S.A. Prendergast

    Pelvic floor physical therapy for vulvodynia: a clinician's guide

    Obstet Gynecol Clin North Am

    (2017)
  • E. Gentilcore-Saulnier et al.

    Pelvic floor muscle assessment outcomes in women with and without provoked vestibulodynia and the impact of a physical therapy program

    J Sex Med

    (2010)
  • C. Chisari et al.

    The experience of pain severity and pain interference in vulvodynia patients: The role of cognitive-behavioural factors, psychological distress and fatigue

    J Psychosom Res

    (2017)
  • D. Funaro et al.

    A double-blind, randomized prospective study evaluating topical clobetasol propionate 0.05% versus topical tacrolimus 0.1% in patients with vulvar lichen sclerosus

    J Am Acad Dermatol

    (2014)
  • J. Bornstein et al.

    2015 ISSVD, ISSWSH, and IPPS consensus terminology and classification of persistent vulvar pain and vulvodynia

    J Sex Med

    (2016)
  • C.A. Higgins et al.

    A population-based case-control study of aetiological factors associated with vulval lichen sclerosus

    J Obstet Gynaecol

    (2012)
  • P. Halonen et al.

    Lichen sclerosus and risk of cancer

    Int J Cancer

    (2017)
  • A. Lee et al.

    Long-term management of adult vulvar lichen sclerosus: a prospective cohort study of 507 women

    JAMA Dermatol

    (2015)
  • F. Behnia-Willison et al.

    Use of platelet-rich plasma for vulvovaginal autoimmune conditions like lichen sclerosus

    Plast Reconstr Surg Glob Open

    (2016)
  • H. Cheng et al.

    Diagnostic criteria in 72 women with erosive vulvovaginal lichen planus

    Australas J Dermatol

    (2015)
  • S.M. Cooper et al.

    Influence of treatment of erosive lichen planus of the vulva on its prognosis

    Arch Dermatol

    (2006)
  • J. Bradford et al.

    Management of vulvovaginal lichen planus

    J Low Genit Tract Dis

    (2013)
  • R.C. Simpson et al.

    Real-life experience of managing vulval erosive lichen planus: a case-based review and U.K. multicentre case note audit

    Br J Dermatol

    (2012)
  • A. Virgili et al.

    Comparative study on topical immunomodulatory and anti-inflammatory treatments for plasma cell vulvitis: long-term efficacy and safety

    J Eur Acad Dermatol Venereol

    (2015)
  • Cited by (25)

    • Geriatric Genital Dermatology

      2024, Journal of the American Medical Directors Association
    • A20 Haploinsufficiency: A Systematic Review of 177 Cases

      2024, Journal of Investigative Dermatology
    • Inflammatory Vulvar Dermatoses (Part I)

      2022, Urology
      Citation Excerpt :

      Chemical cauterization with silver nitrate sticks can be considered. Cases may require intralesional triamcinolone or systemic steroids and recurrent disease may require colchicine or dapsone or tumor necrosis factor antagonists.1,9 Of note, Behcet disease is an uncommon condition which consists of genital and oral aphthae in the setting of multisystem inflammation including other cutaneous manifestations, ocular involvement, gastrointestinal, neurologic, or vascular involvement.

    View all citing articles on Scopus

    Drs Mauskar and Marathe are cofirst authors.

    Funding sources: None.

    Conflicts of interest: None disclosed.

    Date of release: June 2020.

    Expiration date: June 2023.

    View full text