Continuing medical educationVulvar diseases: Conditions in adults and children
Section snippets
Lichen sclerosus
Key points First-line therapy is a daily ultrapotent topical corticosteroid until the texture normalizes 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 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 Treat erosive vulvovaginal lichen planus with ultrapotent daily topical corticosteroids Women with erosive vaginal lichen planus require aggressive management, including compounded vaginal corticosteroid suppositories and vaginal dilators to prevent stenosis 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 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 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 Desquamative inflammatory vaginitis is a unique idiopathic inflammatory mucositis that occurs only in the vagina The diagnosis is made clinically after excluding infection, specific erosive skin disease, and estrogen deficiency 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 Metastatic Crohn's disease of the vulva characteristically presents with vulvar edema or distinctive knife-cut ulcerations The course of metastatic cutaneous Crohn's disease may diverge from that of intestinal disease 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 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 Herpes simplex virus infection in an immunosuppressed person can be indistinguishable from an aphthous ulcer 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 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 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 Vulvodynia is a common underrecognized syndrome of vulvar pain of ≥3 months' duration without a definable cause Obtaining routine biopsy specimens of focal areas of pain or redness are not informative and should be avoided 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)
- et al.
An autoimmune phenotype in vulvar lichen sclerosus and lichen planus: a Th1 response and high levels of microRNA-155
J Invest Dermatol
(2012) - et al.
Intradermal injection of autologous platelet-rich plasma for the treatment of vulvar lichen sclerosus
J Am Acad Dermatol
(2017) - et al.
Fractional carbon dioxide laser in recalcitrant vulval lichen sclerosus
Australas J Dermatol
(2016) - et al.
A randomized double-blind placebo-controlled trial of autologous platelet-rich plasma intradermal injections for the treatment of vulvar lichen sclerosus
J Am Acad Dermatol
(2019) - et al.
Vulvar cancers in women with vulvar lichen planus: a clinicopathological study
J Am Acad Dermatol
(2014) - et al.
Vulvar ulcers in young females: a manifestation of aphthosis
J Pediatr Adolesc Gynecol
(2006) - et al.
Lipschütz ulcers: should we rethink this? An analysis of 33 cases
Eur J Obstet Gynecol Reprod Biol
(2016) - et al.
Reactive nonsexually related acute genital ulcers: review of cases evaluated at Mayo Clinic
J Am Acad Dermatol
(2010) - et al.
Necrotizing genital ulcerations in a premenarcheal female with mononucleosis
Obstet Gynecol
(1998) - et al.
Ulcus vulvae acutum, a rare diagnosis to keep in mind
Eur J Obstet Gynecol Reprod Biol
(2004)
Nonspecific genital ulcers
Clin Dermatol
Management of nonsexually acquired genital ulceration using oral and topical corticosteroids followed by doxycycline prophylaxis
J Am Acad Dermatol
Complex aphthosis: a large case series with evaluation algorithm and therapeutic ladder from topicals to thalidomide
J Am Acad Dermatol
Prevalence and demographic characteristics of vulvodynia in a population-based sample
Am J Obstet Gynecol
Prevalence of symptoms consistent with a diagnosis of vulvodynia: population-based estimates from 2 geographic regions
Am J Obstet Gynecol
Pelvic floor physical therapy for vulvodynia: a clinician's guide
Obstet Gynecol Clin North Am
Pelvic floor muscle assessment outcomes in women with and without provoked vestibulodynia and the impact of a physical therapy program
J Sex Med
The experience of pain severity and pain interference in vulvodynia patients: The role of cognitive-behavioural factors, psychological distress and fatigue
J Psychosom Res
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
2015 ISSVD, ISSWSH, and IPPS consensus terminology and classification of persistent vulvar pain and vulvodynia
J Sex Med
A population-based case-control study of aetiological factors associated with vulval lichen sclerosus
J Obstet Gynaecol
Lichen sclerosus and risk of cancer
Int J Cancer
Long-term management of adult vulvar lichen sclerosus: a prospective cohort study of 507 women
JAMA Dermatol
Use of platelet-rich plasma for vulvovaginal autoimmune conditions like lichen sclerosus
Plast Reconstr Surg Glob Open
Diagnostic criteria in 72 women with erosive vulvovaginal lichen planus
Australas J Dermatol
Influence of treatment of erosive lichen planus of the vulva on its prognosis
Arch Dermatol
Management of vulvovaginal lichen planus
J Low Genit Tract Dis
Real-life experience of managing vulval erosive lichen planus: a case-based review and U.K. multicentre case note audit
Br J Dermatol
Comparative study on topical immunomodulatory and anti-inflammatory treatments for plasma cell vulvitis: long-term efficacy and safety
J Eur Acad Dermatol Venereol
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2022, UrologyCitation 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.
Drs Mauskar and Marathe are cofirst authors.
Funding sources: None.
Conflicts of interest: None disclosed.
Date of release: June 2020.
Expiration date: June 2023.