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ACOG

PRACTICE
BULLETIN
CLINICAL MANAGEMENT GUIDELINES FOR OBSTETRICIAN–GYNECOLOGISTS
NUMBER 93, MAY 2008

Diagnosis and
Management of Vulvar
Skin Disorders
This Practice Bulletin was devel- Symptoms of vulvovaginal disorders are common, often chronic, and can sig-
oped by the ACOG Committee on nificantly interfere with women’s sexual function and sense of well-being. In the
Practice Bulletins—Gynecology evaluation of women who report symptoms of vulvar disorders, the most com-
with the assistance of Lori A. mon diagnoses are dermatologic conditions and vulvodynia (both generalized
Boardman, MD, ScM, and Colleen and localized forms) (1). The purpose of this document is to review diagnostic
M. Kennedy, MD, MS. The infor-
approaches and provide a structured framework for the management of vulvar
mation is designed to aid practition-
disorders.
ers in making decisions about
appropriate obstetric and gyneco-
logic care. These guidelines should
not be construed as dictating an Background
exclusive course of treatment or
procedure. Variations in practice
Definition
may be warranted based on the Pruritus and pain are two of the most common presenting symptoms of vulvar
needs of the individual patient, disorders in women treated in clinics that provide specialized care for vulvar
resources, and limitations unique to conditions (1–3). Pruritus and vulvar pain may occur in the presence of obvi-
the institution or type of practice. ous dermatologic disease or in conditions with few visible skin changes. In
evaluating vulvar pruritus, it can be helpful to group women into those with
acute symptoms and those with chronic symptoms (see box, “Conditions
Commonly Associated With Vulvar Pruritus”). Vulvodynia, defined as burning,
stinging, rawness, or soreness, with or without pruritus, can be further charac-
terized by the site of the pain, whether it is generalized or localized, and
whether it is provoked, spontaneous, or both (4).
THE AMERICAN COLLEGE OF
OBSTETRICIANS AND Examination and Evaluation
GYNECOLOGISTS In cases of acute vulvar pruritus, common etiologies include vulvovaginal can-
WOMEN’S HEALTH CARE PHYSICIANS didiasis and contact dermatitis. Chronic vulvar pruritus should prompt a search

VOL. 111, NO. 5, MAY 2008 OBSTETRICS & GYNECOLOGY 1243


for underlying dermatoses, such as lichen sclerosus, lichen saline and potassium hydroxide preparations, in con-
simplex chronicus, or psoriasis; neoplasia, including vul- junction with vaginal pH determination, will help to
var intraepithelial neoplasia, squamous cell carcinoma, guide the use of subsequent diagnostic tools. These addi-
and Paget disease of the vulva; or vulvar manifestations of tional diagnostic approaches include vaginal yeast cul-
systemic diseases, such as Crohn disease. ture, a variety of point-of-care tests approved by the U.S.
Patients presenting with pain should first be evaluat- Food and Drug Administration, culture or polymerase
ed to rule out underlying organic causes, including chain reaction for confirmation of herpes simplex virus,
inflammatory conditions, neoplasias, infections, or neu- and specific serologic tests (5). For autoimmune disor-
rologic disorders. When organic causes are ruled out, the ders and suspected cases of neoplasia, biopsy is the pre-
diagnosis of vulvodynia can be made, and attention ferred approach.
should then be paid to more fully elucidating the nature
of the pain disorder. Vulvar Dermatoses
Chronic or recurrent forms of vulvovaginal disease Dermatitis (or eczema), a poorly demarcated, erythema-
can be difficult to diagnose and treat. When taking a tous, and usually itchy rash, has been reported to occur in
medical history, it is important to ask about the onset, 20–60% of patients with chronic vulvar symptoms (6).
duration, location, and nature of vulvar symptoms Contact dermatitis is one of the most frequently encoun-
(including the relationship of symptoms to the patient’s tered and often avoidable problems seen in clinics that
menstrual cycle), as well as any possible precipitating or provide specialized care for vulvar disorders. Many
known risk factors. In the evaluation of infectious caus- seemingly innocuous behaviors, such as bathing, the use
es of vulvovaginal symptoms, microscopy using both of sanitary or incontinence pads, or exposure to known
irritants or allergens in numerous topical medications
(see box, “Common Vulvar Irritants and Allergens”),
Conditions Commonly Associated have the potential to initiate contact dermatitis (7, 8).
With Vulvar Pruritus Avoiding allergens can reduce the occurrence of both
contact dermatitis and lichen simplex chronicus. On
Acute
examination, clinical signs can range from mild erythe-
Infections ma, swelling, and scaling to marked erythema, fissures,
• Fungal, including candidiasis and tinea cruris skin thickening, erosions, and ulcers (8). The evaluation
• Trichomoniasis of dermatoses also should include assessment to rule out
candidiasis.
• Vulvovaginal candidiasis
Vulvar lichen simplex chronicus is a chronic eczema-
• Molluscum contagiosum tous disease characterized by scaling and lichenified
• Infestations, including scabies and pediculosis plaque with intense and unrelenting itching, which may
Contact dermatitis (allergic or irritant) result in sleep disruption. Although lichen simplex chron-
icus occurs primarily in mid- to late-adult life, it can occur
Chronic in children. From 65% to 75% of patients will report a
Dermatoses history of atopic disease, and lichen simplex chronicus
• Atopic and contact dermatitis can be seen as a localized variant of atopic dermatitis.
Lichen simplex chronicus represents an end-stage
• Lichen sclerosus, lichen planus, lichen simplex
response to a wide variety of possible initiating process-
chronicus
es, including environmental factors (eg, heat, excessive
• Psoriasis sweating, and irritation from clothing or topically applied
• Genital atrophy products) and dermatologic disease (eg, candidiasis,
Neoplasia lichen sclerosus) (9). In long-standing disease, the skin
appears thickened and leathery, and areas of hyper-
• Vulvar intraepithelial neoplasia, vulvar cancer
pigmentation and hypopigmentation may be present.
• Paget disease Erosions and ulcers also can develop, most commonly
Infection from chronic scratching. Vaginal yeast cultures are help-
• Human papillomavirus infection ful in identifying the presence of an underlying condition
on which lichen simplex chronicus is superimposed.
Vulvar manifestations of systemic disease
Lichen sclerosus, a chronic disorder of the skin, is
• Crohn disease most commonly seen on the vulva, with extragenital
lesions reported in up to 13% of women with vulvar dis-

1244 ACOG Practice Bulletin Vulvar Skin Disorders OBSTETRICS & GYNECOLOGY
mimic lichen sclerosus, a biopsy is necessary to confirm
Common Vulvar Irritants and Allergens the diagnosis, except in a prepubertal child (15, 16).
Lichen planus, an inflammatory disorder of the gen-
Adult or baby wipes
ital mucosa most likely related to cell-mediated immuni-
Antiseptics (eg, povidine iodine, hexachlorophene) ty, exhibits a wide range of morphologies. The most
Body fluids (eg, semen or saliva) common form and the most difficult to treat is the ero-
Colored or scented toilet paper sive form, which can lead to significant scarring and
pain. Most commonly recognized on the skin or oral
Condoms (lubricant or spermicide containing)
mucosa, this condition may affect the lower genital tract.
Contraceptive creams, jellies, foams, nonoxynol-9, Approximately 1% of the population has oral lichen
lubricants planus, and of women with oral disease (17), approxi-
Dyes mately 20–25% have genital vulvovaginal disease (18).
Emollients (eg, lanolin, jojoba oil, glycerin) The classic presentation of lichen planus on mucous
membranes, including the buccal mucosa, is that of
Laundry detergents, fabric softeners, and dryer sheets
white, reticulate, lacy, or fernlike striae (Wickham stri-
Rubber products (including latex) ae). On occasion, the skin may appear uniformly white,
Sanitary products, including tampons, pads and thus lichen planus can be confused with lichen scle-
Soaps, bubble bath and salts, shampoos, conditioners rosus. Pruritic, purple, shiny papules are typically asso-
ciated with lichen planus. However, if papules are found
Tea tree oil
on the genital skin, they appear dusky pink in color,
Topical anesthetics (eg, benzocaine, lidocaine, dibu- without an apparent scale, and less well demarcated.
caine) In erosive lichen planus, deep, painful, erythema-
Topical antibacterials (eg, neomycin, bacitracin, tous erosions appear in the posterior vestibule and often
polymyxin) extend to the labia minora, resulting in agglutination and
Topical antimycotics (eg, imidazoles, nystatin) resorption of the labial architecture. The vaginal epi-
Topical corticosteroids thelium can become erythematous, eroded, acutely
inflamed, and denuded of epithelium. Erosive patches, if
Topical medications, including trichloroacetic acid, 5-flu-
present, are extremely friable. Over time, these eroded
orouracil, podofilox or podophyllin
surfaces may adhere, resulting in synechiae and, eventu-
Vaginal hygiene products, including perfumes and ally, complete obliteration of the vaginal space (19, 20).
deodorants Symptoms commonly reported by patients with erosive
vulvar lichen planus include dyspareunia, burning, and
increased vaginal discharge (21).
ease (10). The mean age of onset is in the fifth to sixth Erosive lichen planus often is labeled as desquama-
decade, but it may occur at any age, including prepuberty tive inflammatory vaginitis when vaginal discharge
(11). The exact etiology of this condition is unclear, reveals predominance of inflammatory cells and imma-
although an autoimmune process or possible genetic link ture parabasal and basal epithelial cells (these will
is likely (12, 13). Patients presenting with lichen sclero- appear small and round, with relatively large nuclei).
sus most commonly report pruritus, followed by irrita- The vaginal pH is increased, usually in the range of 5–6.
tion, burning, dyspareunia, and tearing. The prevalence Whether desquamative inflammatory vaginitis is a type
of this condition remains unknown because lichen scle- of erosive lichen planus or a distinct type of vaginitis is
rosus may be asymptomatic. controversial (19, 20). Biopsy of the affected area, which
On examination, typical lesions of lichen sclerosus reveals a bandlike infiltrate of lymphocytes and colloid
are porcelain-white papules and plaques, often with areas bodies in the basal layers of the epidermis, may be rela-
of ecchymosis or purpura. The skin commonly appears tively nonspecific because of the complete loss of the
thinned, whitened, and crinkling (leading to the descrip- epithelium. However, a histologic specimen can help
tion “cigarette paper”). Although the vaginal epithelium rule out immunobullous diseases, such as cicatricial
is largely spared from lichen sclerosus, involvement of pemphigoid, bullous pemphigoid, and pemphigus vul-
the mucocutaneous junctions may lead to introital nar- garis, which may mimic lichen planus (22).
rowing. Perianal involvement can create the classic “fig-
ure of eight” or hourglass shape. Other findings include Vulvar Atrophy
fusion of the labia minora, phimosis of the clitoral hood, Estimates indicate that up to 50% of all postmenopausal
and fissures (14). Because other vulvar diseases can women will experience vulvovaginal irritation, soreness,

VOL. 111, NO. 5, MAY 2008 ACOG Practice Bulletin Vulvar Skin Disorders 1245
and dryness, lower urinary tract problems, and dyspare- If the patient is immunocompromised or the diagno-
unia (23, 24). The onset of symptoms can occur long sis is uncertain (eg, Paget disease can present as multiple
after other menopausal symptoms (eg, hot flushes) bright red, scaly, eczematoid plaques [35], whereas mela-
resolve. However, perimenopausal women who do not noma can range in color from brown to bluish-black [36]),
have visible signs of vulvovaginal atrophy also can expe- biopsy should be undertaken. Because multicentric dis-
rience symptoms of vulvar irritation and dryness (25). ease is commonly encountered, particularly in younger
Finally, despite the use of systemic hormone therapy,
women, a complete examination should include the
approximately 10–25% of users will continue to experi-
cervix, vagina, and perianal area. Up to 50% of women
ence symptoms of urogenital atrophy despite improve-
ment in other symptoms associated with estrogen with VIN will have antecedent or concomitant lower gen-
deficiency (26). ital tract neoplasia, usually cervical or vaginal intraepithe-
As women approach menopause, vulvar tissue lial neoplasia (5).
becomes increasingly sensitive to irritants (27), and in In addition to contact irritation from the array of
the absence of estrogen, the vaginal mucosa becomes treatments used to alleviate vulvar symptoms, many
pale, thin, and often dry. Vaginal secretions are reduced, patients with underlying chronic vulvar skin disorders,
the vaginal pH becomes more alkaline, and the vaginal including lichen sclerosus, lichen planus, and psoriasis,
flora is altered (28, 29). Activities that once were not are also at risk of developing steroid rebound dermatitis,
associated with discomfort may become so. The genital also called steroid rosacea. Withdrawing from use of
area becomes increasingly susceptible to trauma, chemi- a moderate- to high-potency topical steroid can result
cal irritants, and bacterial overgrowth. In severe atrophic in rebound vasodilation, accompanied by burning and
vaginitis, a purulent, noninfectious discharge may devel- irritation.
op, along with fissuring of the vestibule. The diagnosis
is based on an elevated vaginal pH (range of 6.0–7.5)
and the presence of parabasal or intermediate cells on Clinical Considerations and
microscopy. An amine test result will be negative in this Recommendations
setting (although bacterial vaginosis continues to be a
problem in this population as well) (30). When and how should a vulvar biopsy be
performed?
Other Considerations
The threshold for biopsy of the vulva should be low
In assessing vulvar pruritus and pain, the common caus- except in the pediatric population. Changes on the vulva
es of vulvovaginitis (candidiasis, bacterial vaginosis, and often are subtle and can be overlooked. Findings such as
trichomoniasis), particularly in their more complicated thickening, pebbling, hypopigmentation, or thinning of
forms, must be included in the differential diagnosis. For the epithelium indicate a possible dermatologic process,
example, non-albicans candidal infections typically and biopsy will aid in diagnosis and management.
present with burning, not itching, and minimal evidence Biopsy of hyperpigmented or exophytic lesions, lesions
of inflammation. Because most atypical species do not with changes in vascular patterns, or unresolving lesions
form hyphae or pseudohyphae, microscopy is not helpful is particularly important and should be performed in
and, therefore, culture is warranted (31, 32). Negative order to rule out carcinoma. Diagnostic delays in identi-
yeast cultures, in the setting of persistent vulvar burning fying vulvar cancer are exceedingly common and have
with no other identifiable organic cause, also will help been linked to failures or procrastination in the perform-
establish a diagnosis of vulvodynia (33, 34). ance of biopsies of abnormal-appearing vulvar skin (37).
Genital human papillomavirus (HPV), the most Anesthesia is recommended for vulvar biopsy; com-
common sexually transmitted viral infection, is associat- bination lidocaine and prilocaine cream or 4% liposomal
ed with a number of vulvar epithelial disorders, includ- lidocaine cream can be placed on the proposed biopsy
ing genital warts, vulvar intraepithelial neoplasia (VIN), site before inserting the anesthesia needle (38). A sys-
and some vulvar carcinomas. Distinguishing warts from tematic review of topical anesthetics for dermatologic
vulvar neoplasia on the basis of appearance alone is not procedures found both medications to be effective, but
always possible because VIN can present as red, white, liposomal lidocaine had a more rapid onset (30 minutes
dark, raised, or eroded lesions. In general, a biopsy versus 60 minutes with combination lidocaine and prilo-
should be performed on hyperpigmented, indurated, caine cream) and lower cost (39). To minimize discom-
fixed, or ulcerative lesions, or lesions that do not respond fort, a solution of 8.4% sodium bicarbonate can be added
to treatment or worsen during treatment. to the lidocaine (1:10 ratio). Onset of action occurs with-

1246 ACOG Practice Bulletin Vulvar Skin Disorders OBSTETRICS & GYNECOLOGY
in 2–5 minutes; if epinephrine is used with either of ineal pain (47–49). All forms of topical estrogen therapy
these anesthetics, the onset of action will be delayed, but increase the possibility of endometrial hyperplasia and
the duration of effect will be increased (40). overstimulation. However, it is currently unknown whether
Choice of biopsy instrument depends on the loca- women receiving long-term therapy with topical estro-
tion and nature of the lesions. For most inflammatory gen require prophylactic therapy with progesterone (24).
diseases, ulcers, pigmented lesions, or suspected tumors, It should be noted that systemic estrogen therapy,
a punch biopsy is the preferred method because estab- although efficacious, raises concerns for many women
lishing the depth of such lesions is critical (41). Small and may still not be sufficient to relieve the symptoms of
lesions often can be completely excised, and lesions urogenital atrophy (50).
involving the submucosal or subcutaneous tissue should Whether or not estrogen use improves the function
be adequately sampled. When sampling ulcerative areas, of vulvar epithelium in vulvar atrophy (including clitoral
a biopsy of the edge of the ulcer is preferred; when sam- circulation and fibrosis) remains controversial. Small
pling hyperpigmented areas, a biopsy of the thickest studies have demonstrated improvement in circulation
region is recommended (42). and sexual function in women following localized or
One of the most common methods of biopsy systemic estrogen use (51, 52).
involves the use of a 3–5-mm Keyes punch. A shave or Topical estrogen therapy also has been reported for
snip biopsy works best for sampling more superficial the treatment of symptomatic labial adhesions (53, 54).
disorders, such as lichen sclerosus or lichen planus, or The use of estrogen for other vaginal disorders is less
for sampling bullous lesions. Although both shave and well documented, and includes treatment for radiation
snip samples will extend into the dermis, they should not injury (55) and treatment of vaginal mucosa burn injury
go through the dermis. A stitch or topical solution can be following misapplication of 100% acetic acid (56).
used to control bleeding (43). Indications for the use of testosterone cream in the
treatment of vulvar or vaginal disorders remain contro-
How should labial adhesions be treated? versial. Historically, testosterone cream was used in the
Labial adhesions are not uncommon in prepubertal girls treatment of lichen sclerosus. Subsequently, studies were
and typically resolve spontaneously by menarche. Labial undertaken comparing testosterone with topical steroids
adhesions should be observed unless they are sympto- in the treatment of vulvar lichen sclerosus and resulted in
matic (eg, urinary obstructive symptom). Topical estro- two findings, a lack of clear effect with testosterone and
gen cream is considered first-line medical treatment for the superiority of topical steroids (12, 57–59). Thus,
the separation of labial adhesions (44). Girls should be given the undesirable side effects of masculinization and
monitored for side effects of estrogen therapy, including virilization associated with the use of testosterone, and
breast budding and vaginal bleeding. To decrease the risk unproven therapeutic benefit, it should not be used in the
of recurrence and to prevent reagglutination of raw primary treatment of lichen sclerosus.
opposing skin surfaces, an emollient can be applied
nightly for at least 1 month. Manual separation in the How should vulvar atrophy be treated?
office setting should only be performed with adequate As noted previously, the data specifically addressing vul-
anesthesia, and surgical excision should be reserved for var atrophy are limited. Studies assessing the benefits of
acute urinary obstruction (45). One small trial showed a variety of therapies, including hormones, focus on
success with topical steroids as a potential alternative to patient symptoms such as vulvovaginal dryness, burning,
the use of estrogen (46). pruritus, and dyspareunia, with the distinction between
vulvar and vaginal symptoms not clearly made.
In what circumstances is estrogen or testos-
Management options for urogenital atrophy in adult
terone cream an appropriate treatment?
women include lifestyle modification strategies, the use of
The use of estrogen treatment for vaginal atrophy has vaginal moisturizers, and low-dose topical estradiol
been well documented and can be accomplished with a preparations. Maintaining regular vaginal intercourse pro-
variety of locally applied estrogen preparations (24). vides protection from urogenital atrophy by increasing
However, some differences have been demonstrated blood flow to the pelvic organs (60), an effect that also
between forms of local therapies. When compared in may occur through masturbation (61). The use of a non-
randomized controlled trials with either estrogen tablets hormonal vaginal moisturizer has been shown through
or with the estrogen ring, conjugated equine estrogen prospective randomized studies to exert a beneficial effect
cream was found to be significantly associated with similar to that of local hormone therapy (49, 62), although
adverse effects, including bleeding, breast pain, and per- the same cannot be said of vaginal lubricants (25).

VOL. 111, NO. 5, MAY 2008 ACOG Practice Bulletin Vulvar Skin Disorders 1247
What is the appropriate treatment for lichen In small, uncontrolled studies, other therapies
sclerosus? found to be of use in the treatment of complicated lichen
sclerosus (eg, unresponsive to topical steroids) include
The recommended treatment for lichen sclerosus is use retinoids (70, 71), potassium para-aminobenzoate (72),
of a high-potency topical steroid, the most studied of cryotherapy (73), and photodynamic therapy with topi-
which is clobetasol propionate (59, 63, 64). In one cal 5-aminolaevulinic acid (74). Topical cyclosporine
prospective cohort study of patients with lichen scle- failed to show benefit in the treatment of five cases of
rosus treated with ultrapotent topical steroids, 96% vulvar lichen sclerosus (75). Management with hormon-
experienced complete or partial relief of their vulvar al topical therapies, including estrogen, testosterone
symptoms, 23% demonstrated complete resolution of the (57, 61, 63, 76), and progesterone (63, 77), has incon-
vulvar skin to normal texture and color, and 68% showed sistently resulted in improvement in lichen sclerosus
partial resolution of the hyperkeratosis, purpura, fissur- and has been associated with significant side effects (eg,
ing, and erosions associated with this disorder (65). hyperandrogenism). The use of the topical macrolide
Although no randomized controlled trials provide evi- immunosuppressants (eg, pimecrolimus, tacrolimus)
dence of the most effective steroid regimen, a reasonable was proposed initially as a therapeutic option for lichen
approach is to begin with once-daily application of ultra- sclerosus, given that these medications do not cause der-
potent topical steroids for 4 weeks, tapering to alternate mal atrophy. Although promising in the treatment of
days for 4 weeks, followed by 4 weeks of twice weekly lichen sclerosus (78–80), these immunosuppressants are
application (16). Although some practitioners recom- considered by most experts to be second-line agents for
mend twice-daily application, pharmacodynamic studies treatment of patients unresponsive to or intolerant of
have clearly demonstrated that once-daily application of other treatment. Surgery, although not curative, is
an ultrapotent steroid is sufficient (66). Topical steroid reserved for the treatment of malignancy and postin-
therapy is not without complications, including the pos- flammatory sequelae (eg, release of labial adhesions or
sibility of contact sensitization, skin changes, and sec- introital stenosis) (81).
ondary infection. However, studies have shown that
long-term maintenance therapy with either a moderate or What is the appropriate treatment for lichen
ultrapotent topical steroid did not lead to steroid-induced planus?
atrophy, telangiectasia, striae, or secondary infection
(67). In general, a 30-g tube of an ultrapotent topical The prognosis for spontaneous remission of vulvovaginal
steroid should last approximately 3–6 months (16). lichen planus is poor. As with vulvovaginal atrophy and
There is conflicting evidence on the need for ongo- lichen sclerosus, lifestyle modifications are important in
ing maintenance therapy. Some experts recommend dis- maintaining function as well as providing comfort.
continuation of therapy following the initial regimen Treatment options are numerous and include topical and
described previously and reuse of therapy for flares or systemic corticosteroids, topical and oral cyclosporine,
recurrent symptoms (16). However, others recommend topical tacrolimus, hydroxychloroquine, oral retinoids,
an ongoing maintenance regimen of twice-weekly appli- methotrexate, azathioprine, and cyclophosphamide. Al-
cation of either an ultrapotent or moderate strength though symptomatic improvement is possible (21),
steroid (68). Monitoring at 3 months and 6 months fol- patients should be advised that complete control is not the
lowing initial therapy is recommended to assess the norm (20). Rather, vulvovaginal lichen planus is a frus-
patient’s response to therapy and to ensure proper appli- trating, chronic, and recurring disease that requires long-
cation of the medication. Annual examinations are term maintenance.
suggested for patients whose lichen sclerosus is well Evidence supporting treatment options for vulvo-
controlled, and more frequent visits for those with poor- vaginal lichen planus have come largely from retro-
ly controlled disease (for whom intralesional steroid spective case reports and case series (82–85). The most
injections also may be beneficial) (69). Patients should frequently recommended treatment for vulvovaginal
be advised to return for visits if persistent ulcerations or lichen planus is the use of high-potency topical steroids,
new growths appear. Biopsy of such lesions, as well as with the evidence for this recommendation based on
of erosions, hyperkeratotic, or hyperpigmented areas, is small studies of oral rather than genital disease (84).
important to exclude intraepithelial neoplasia or invasive Given the potential for significant side effects with the
squamous cell cancer. Whether long-term topical treat- use of systemic immunosuppressants, methotrexate,
ment with a potent steroid cream can reduce the risk of cyclosporine, azathioprine, and hydroxychloroquine sul-
malignant evolution (68) remains unclear pending fate should be used only after local treatments have
results from larger follow-up studies. failed. Furthermore, study results of systemic therapies

1248 ACOG Practice Bulletin Vulvar Skin Disorders OBSTETRICS & GYNECOLOGY
have been inconsistent, likely related to selection of associated disease is present, it is typically local (adeno-
those patients with more severe disease (85). carcinoma in the skin adnexa or Bartholin gland) but also
As with lichen sclerosus, surgery is reserved for the may be distant (most commonly, of the breast, but also
treatment of postinflammatory sequelae, including vagi- of the genital, urinary, or intestinal tract). In contrast,
nal coaptation and release of labial adhesions. Finally, perianal extramammary Paget disease is associated with
although squamous cell carcinomas are rarely encoun- underlying colorectal adenocarcinoma in up to 80% of
tered, women with lichen planus should be routinely cases (90). Thus, when Paget disease is confirmed by
monitored (85, 86). biopsy, evaluation including breast, genitourinary tract,
and gastrointestinal tract should be undertaken.
What other conditions should be investigated
in women presenting with vulvar disease (eg,
lichen planus, lichen sclerosus, or Paget Summary of
disease)?
Recommendations
Diseases with vulvar and perianal involvement include
not only dermatologic processes (eg, lichen sclerosus The following recommendation is based on limit-
and psoriasis) but also systemic diseases such as Crohn ed or inconsistent scientific evidence (Level B):
disease. Approximately one third of women with Crohn The recommended treatment for lichen sclerosus is
disease present with gynecologic complications, includ- an ultrapotent topical corticosteroid, such as clo-
ing enteric fistulas to both the upper and lower genital betasol propionate.
tract, granulomatous salpingitis and oophoritis, and vul-
var inflammation, edema, granulomas, abscesses, and
The following recommendations are based prima-
ulcerations (87).
Numerous studies have now demonstrated a strong
rily on consensus and expert opinion (Level C):
association, particularly in women, between lichen scle- Biopsy of hyperpigmented or exophytic lesions,
rosus and a variety of autoimmune-related disorders, lesions with changes in vascular patterns, or unre-
including alopecia, vitiligo, thyrotoxicosis, hypothy- solving lesions is particularly important and should
roidism, and pernicious anemia (12). In a recent case
be performed to rule out carcinoma.
series of women with vulvovaginal lichen planus, 55%
had a personal or family history of an autoimmune dis- For patients with biopsy-confirmed Paget disease,
order (88). Despite the strong association between lichen further evaluation of the breast, genitourinary tract,
sclerosus and autoimmune-related disorders, there are no and gastrointestinal tract should be undertaken.
recommendations regarding evaluation for coexisting
autoimmune-related disorders in women with lichen
sclerosus beyond a brief examination for alopecia areata Proposed Performance
and vitiligo and consideration of thyroid function tests
(89). In women, there is also an association of vulvar
Measure
lichen sclerosus with squamous cell carcinoma, with up The percentage of women with lichen sclerosus who are
to a 5% incidence of malignancy (12). Therefore, close offered high-potency topical corticosteroids as first-line
follow-up is recommended with biopsy of any concern- treatment
ing lesions, although the recommended follow-up inter-
val has not been determined (eg, 6 months, 12 months, or
other). Additionally, although the concern of malignancy References
is greater in poorly controlled lichen sclerosus, there is
1. Hansen A, Carr K, Jensen JT. Characteristics and initial
no clear evidence that optimal control of lichen sclerosus diagnoses in women presenting to a referral center for vul-
symptoms reduces the risk of malignancy. vovaginal disorders in 1996-2000. J Reprod Med 2002;
Paget disease of the vulva is a rare form of intra- 47:854–60. (Level III)
epithelial neoplasia characterized by adenocarcinoma- 2. Bornstein J, Pascal B, Abramovici H. The common prob-
tous cells and accounts for approximately 2% of vulvar lem of vulvar pruritus. Obstet Gynecol Surv 1993;48:
neoplasms. Although most cases of extramammary vul- 111–8. (Level III)
var Paget disease are primary rather than associated with 3. Kehoe S, Luesley D. Pathology and management of vul-
underlying adenocarcinoma, approximately 25% of val pain and pruritus. Curr Opin Obstet Gynecol 1995;
cases are associated with neoplastic disease (90). When 7:16–9. (Level III)

VOL. 111, NO. 5, MAY 2008 ACOG Practice Bulletin Vulvar Skin Disorders 1249
4. Moyal-Barracco M, Lynch PJ. 2003 ISSVD terminology 113 patients seen in a vulvar specialty clinic. J Reprod
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J Reprod Med 2004;49:772–7. (Level III) 22. Ramer MA, Altchek A, Deligdisch L, Phelps R,
5. Workowski KA, Berman SM. Sexually transmitted dis- Montazem A, Buonocore PM. Lichen planus and the vul-
eases treatment guidelines, 2006. Centers for Disease vovaginal-gingival syndrome. J Periodontol 2003;74:
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1252 ACOG Practice Bulletin Vulvar Skin Disorders OBSTETRICS & GYNECOLOGY
Copyright © May 2008 by the American College of Obstetri-
The MEDLINE database, the Cochrane Library, and cians and Gynecologists. All rights reserved. No part of this
ACOG’s own internal resources and documents were used publication may be reproduced, stored in a retrieval system,
to conduct a literature search to locate relevant articles pub- posted on the Internet, or transmitted, in any form or by any
lished between January 1985 and August 2007. The search means, electronic, mechanical, photocopying, recording, or
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organizations or institutions such as the National Institutes 409 12th Street, SW, PO Box 96920, Washington, DC 20090-6920
of Health and the American College of Obstetricians and Diagnosis and management of vulvar skin disorders. ACOG Practice
Gynecologists were reviewed, and additional studies were Bulletin No. 93. American College of Obstetricians and Gynecologists.
located by reviewing bibliographies of identified articles. Obstet Gynecol 2008;111:1243–53.
When reliable research was not available, expert opinions
from obstetrician–gynecologists were used.
Studies were reviewed and evaluated for quality according
to the method outlined by the U.S. Preventive Services
Task Force:
I Evidence obtained from at least one properly
designed randomized controlled trial.
II-1 Evidence obtained from well-designed controlled
trials without randomization.
II-2 Evidence obtained from well-designed cohort or
case–control analytic studies, preferably from more
than one center or research group.
II-3 Evidence obtained from multiple time series with or
without the intervention. Dramatic results in uncon-
trolled experiments also could be regarded as this
type of evidence.
III Opinions of respected authorities, based on clinical
experience, descriptive studies, or reports of expert
committees.
Based on the highest level of evidence found in the data,
recommendations are provided and graded according to the
following categories:
Level A—Recommendations are based on good and con-
sistent scientific evidence.
Level B—Recommendations are based on limited or incon-
sistent scientific evidence.
Level C—Recommendations are based primarily on con-
sensus and expert opinion.

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