The Vulvar Dermatoses Part of the Differential Diagnosis for Sexual Dysfunction Sexuality Matters

The Vulvar Dermatoses Part of the Differential Diagnosis for Sexual Dysfunction Sexuality Matters

Sexuality Matters
The Vulvar Dermatoses
Part of the Differential Diagnosis
for Sexual Dysfunction
Jill M. Krapf, MD; Andrew Goldstein, MD
Vulvar complaints can be an uncomfortable discussion not only for
the patient but also for the health care provider. Because vulvar
dermatoses fall between two, often disparate, specialties—gynecology
and dermatology—clinicians may feel poorly trained to identify and treat
these disorders. Vulvar dermatoses must be considered as a part of the
differential diagnosis for any woman with sexual dysfunction or pain.
ulvar dermatoses can interfere with sexual function
because of discomfort, pain,
and embarrassment. Chronic
vulvar conditions impact
not only a woman’s sexual well-being
but also her overall quality of life.1 As
women become more comfortable with
vulvar health, they will seek the advice
of their physicians, especially about
their sexual health. Gynecologists must
be prepared to diagnose and treat vulvar
conditions, including chronic vulvar
skin disorders. This review will discuss
the diagnosis and treatment of common
vulvar dermatoses, with a focus on how
these conditions affect sexual wellbeing in women.
V
An Approach to the Vulva
Most women do not realize that vulvovaginal symptoms are common, and in
turn, may feel isolated due to their condition. In this solitude, women might
fear that their symptoms represent cancer or a sexually transmitted disease.2
Vulvovaginal disease can significantly
affect sexual well-being, including sexual function and intimacy. Frustration
and depression are common in most
chronic pain conditions and should
Follow The Female Patient on
and
be a consideration with chronic vulvar
disease as well.3
A comprehensive history and physical
examination are essential in the management of vulvar conditions. A full
Jill M. Krapf, MD, is Adjunct Instructor, The George
Washington University School of Medicine and Health
Sciences, Washington, DC. Andrew Goldstein, MD, is
Associate Clinical Professor, The George Washington
University School of Medicine and Health Sciences;
Director, Centers for Vulvovaginal Disorders, Washington,
DC, and New York, NY; President, The International
Society for the Study of Women’s Sexual Health.
The Female Patient | VOL 37 APRIL 2012 1
The Vulvar Dermatoses
in pigmentation, and scarring. A cotton
swab may be used to detect tenderness
or decreased sensation. Examination
with a colposcope can be very useful,
and we highly recommend its use.
A speculum examination is necessary
to detect vaginal findings such as ulceration, synechiae, loss of rugae, pallor,
and petechiae. Vaginal discharge should
be obtained for cultures, wet mount, and
pH assessment. Abnormalities warrant a
vulvar biopsy; many vulvar dermatologic
conditions cannot be diagnosed by mere
observation. A general examination, including skin, eyes, and mouth, should
be performed as some vulvar dermatoses
are associated with autoimmune conditions and extra-genital lesions. A systematic approach to physical examination is
essential when considering the differential diagnosis of vulvar presentations.
Irritant and Contact Dermatitis
FIGURE 1. Contact dermatitis occurs when an irritant or allergen
causes inflammation, presenting clinically in a range from mild
erythema and swelling to severe erythema, fissures, skin thickening,
erosion, and ulceration.
sexual history, including current sexual
practices and infections, is crucial. Eliciting use of topical or over-the-counter
medications is important. A history of
genital surgery, including labiaplasty,
which is becoming much more common,
should also be noted. It is also important
to ask about vulvar trauma. Lastly, exercise regimens and vulvovaginal care
habits should be discussed.
Examination of the vulva should be
systematic and thorough, performed in
dorsal lithotomy position with proper
lighting. A mirror may be helpful to
allow the patient to communicate the
location of concern. Observation of the
vulva includes identification of atrophy,
erythema, induration, fissures, lichenification, ulceration, erosions, changes
2
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Contact dermatitis is one of the most
common and often avoidable problems.
The incidence is approximately 15% to
30%; however, with increased use of
over-the-counter vulvar products, the
incidence is rising.4 Exogenous agents
cause inflammation of the skin. Common irritants and allergens include
body fluids, menstrual pads, heat, soaps
and detergents, antibiotics, douches, fragrances, nickel (from piercings), rubber,
and spermicides.5 In addition, semen
can also act as an irritant or allergen.
Clinical examination may reveal a
range of fi ndings from mild erythema
and swelling to severe erythema, fissures, skin thickening, erosion and ulceration (Figure 1).6 A detailed history
and physical examination are keys to
diagnosis; however, physicians should
have a low threshold for biopsy to rule
out coexisting conditions. With continued exposure or chronic scratching, lichen simplex chronicus may develop.
Identification and removal of the causative agent is the main goal of treatment. It is essential to counsel patients
on proper vulvar hygiene. Inflammation
may be alleviated with topical steroids,
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Krapf and Goldstein
including triamcinolone 0.1% ointment
twice daily for moderate cases and clobetasol 0.05% ointment once daily for
severe cases. Ice packs and antihistamines, such as hydroxyzine, are helpful
for vulvar pruritus. Scratching during
sleep can be especially difficult to treat.
Low-dose tricyclic antidepressants, such
as amitriptyline, may be given at bedtime for this purpose. Patients should be
examined 1 month after initiating treatment, with steroids and antidepressants
tapered with resolution of symptoms.
Superimposed fungal and bacterial infections are common and should be
treated. Patients who do not respond to
treatment will need reevaluation and a
biopsy to exclude other conditions.
Lichen Simplex Chronicus
Lichen simplex chronicus of the vulva is a chronic eczematous condition
characterized by intense and unrelenting pruritus, leading to scratching and
lichenification (Figure 2). The disorder
represents an end-stage response to a
causative process. Excoriation and fissures can become infected with yeast or
bacteria. A biopsy is often necessary to
exclude lichen sclerosus, lichen planus,
or vulvar intraepithelial neoplasia.3
Lichen simplex chronicus affects quality of life, impacting both psychological
and sexual well-being. The condition
has been associated with psychological
problems, including demoralization, depression, anxiety, obsessive-compulsive
disorder, and sleep disturbances. Compared to matched controls, women with
lichen simplex chronicus demonstrated
significantly lower scores on the Female
Sexual Function Index, especially in the
domain scores of desire, arousal, lubrication, orgasm, and sexual satisfaction.1
Identifying and eliminating all irritant
and allergen exposure is the first step
in treatment of lichen simplex chronicus. It is also essential to break the itchscratch-itch cycle, which can be difficult
as women may scratch in their sleep.
Nighttime pruritus may be alleviated
with oral amitriptyline at bedtime and
FIGURE 2. Lichen simplex chronicus is a chronic eczematous
condition characterized by intense and unrelenting pruritus, leading to
scratching and lichenification.
application of ice. Inflammation may be
treated with topical application of high
potency corticosteroids. Concomitant
infections should be treated accordingly.
Lichen Sclerosus
Lichen sclerosus is a chronic inflammatory skin disorder that affects approximately 1 in 70 women, usually
presenting in premenarchal girls and
menopausal women.3 Vulvar irritation is
the most common presenting symptom,
although lichen sclerosus may also be
entirely asymptomatic and found incidentally on examination. With disease
progression, scratching and sclerotic
changes lead to erosions and fissures;
progressive scarring results in narrowing of the introitus, resorption of the labia minora, and phimosis of the clitoris
(Figure 3). Although the exact pathogenesis of lichen sclerosus is unclear,
the condition is generally accepted as
The Female Patient | VOL 37 APRIL 2012 3
The Vulvar Dermatoses
formed before treatment with topical
steroids is initiated.
First-line treatment is clobetasol propionate 0.05% ointment applied once daily
at night for 4 weeks, followed by alternate
nights for 4 weeks, then twice weekly for
4 weeks. Patients should follow up 2 to
3 months after initiating treatment, followed by 6 months, and then seen annually if disease is well controlled.7 Approximately 60% of patients will experience
complete remission of their symptoms
with this regimen.10 The topical calcineurin inhibitors tacrolimus and pimecrolimus have been studied, but given their
unclear long-term safety profiles, they are
not considered first-line treatment.11,12
Lichen Planus
FIGURE 3. Lichen sclerosus is a chronic inflammatory skin disorder
that has a waxy or “cigarette paper” appearance. With disease
progression, scratching and sclerotic changes may lead to erosions,
fissures, and narrowing of the introitus.
an autoimmune disorder. There is a 5%
associated risk of vulvar squamous cell
carcinoma, and it is unclear if treatment
decreases this risk.7
Chronic vulvar pain has been reported by 79% of women with lichen sclerosus.3 Of all quality of life domains, sexual function is most impacted. Lichen
sclerosus can cause sexual dysfunction, with introital dyspareunia and
decreased sexual activity. This disorder
has been shown to cause sexual distress
by affecting desire, arousal, lubrication,
orgasm, satisfaction, and pain.8 However, treatment of lichen sclerosus does
improve sexual dysfunction.9
Physical examination reveals ivory
white atrophic plaques with a “cigarette
paper” appearance. Although vulvar
lichen sclerosus can be a clinical diagnosis, skin changes may be difficult to
differentiate from vulvar intraepithelial
neoplasia, and a biopsy should be per-
4
The Female Patient | VOL 37 APRIL 2012
Lichen planus, an autoimmune inflammatory mucocutaneous disorder, affects
approximately 1% of women with a peak
incidence from age 30 to 60 years.12 Patients may present with pruritus, burning, dyspareunia, postcoital bleeding, or
vaginal discharge. This disease severely
affects sexual interaction. Nearly 8% of
women examined for evaluation of vulvar
pain were found to have lichen planus.
Ninety-five percent of women reported
sexual dysfunction, with dyspareunia in
60% and apareunia in 35% of women.13
Erosive lichen planus presents as
glassy, brightly erythematous erosions
accompanied by white striae (Wickham’s
striae). The disease may markedly alter
the vulvovaginal anatomy resulting in
loss of the labia minora, narrowing of the
introitus, and obliteration of the vagina
(Figure 4). Patients frequently report a copious yellow vaginal discharge. Lichen
planus can be misdiagnosed as lichen
sclerosus. Unlike lichen planus, lichen
sclerosus has a waxy or “cigarette paper”
appearance and rarely displays vaginal
involvement. It is important to remember
that lichen sclerosus and lichen planus
may coexist in the same patient.
Vulvar and vaginal lichen planus are
difficult to treat; lesions are relatively
resistant to available therapies. Firstline treatment is topical clabetasol pro-
Krapf and Goldstein
pionate 0.05%. Daily treatment should
be continued until lesions have resolved
and then slowly tapered, with a limit of
3 months. Soaking in warm water may
aid in penetration of the topical through
heavily keratinized lesions.14 Vaginal lichen planus may be treated with intravaginal hydrocortisone suppositories to
prevent obliteration of the vagina.
Calcineurin inhibitors have also been
used for vulvovaginal lichen planus
with good success. When topical medications fail, the next step is systemic
treatment with an oral corticosteroid, 40
to 60 mg per day for 2 to 4 weeks. Although not recommended for active lichen planus, in cases of severe scarring,
surgery to lyse vulvovaginal adhesions
may be necessary to restore a woman’s
sexual function.14
The Challenge
Vulvar pain and dyspareunia are common presenting complaints in the office
setting. Vulvar dermatoses must be considered as a part of the differential diagnosis for any woman with sexual dysfunction or pain. A detailed history and
physical examination, backed by a confident knowledge of the vulvar dermatoses, will aid in diagnosis and treatment.
The authors report no actual or potential conflicts of interest in relation to this
article.
References
1. Ermertcan AT, Gencoglan G, Temeltas G, Horasan GD,
Deveci A, Ozturk F. Sexual dysfunction in female patients
with neurodermatitis. J Androl. 2011;32(2):165-169.
2. Margesson LJ. Vulvar disease pearls. Dermatol Clin.
2006;24(2):145-155.
3. Burrows LJ, Shaw HA, Goldstein AT. The vulvar dermatoses. J Sex Med. 2008;5(2):276-283.
4. Beecker J. Therapeutic principles in vulvovaginal dermatology. Dermatol Clin. 2010;28(4):639-648.
5. Bhate K, Landeck L, Gonzalez E, Neumann K, Schalock
P. Genital contact dermatitis: a retrospective analysis.
Dermatitis. 2010;21(6):317-320.
6. American College of Obstetricians and Gynecologists. ACOG Practice Bulletin No. 93: diagnosis and
management of vulvar skin disorders. Obstet Gynecol.
2008;111(5):1243-1253.
7. Murphy R. Lichen sclerosus. Dermatol Clin.
2010;28(4):707-715.
FIGURE 4. Lichen planus is an autoimmune inflammatory
mucocutaneous disorder targeting oral and vulvovaginal mucosa.
Erosive lichen planus presents as glassy, brightly erythematous
erosions accompanied by white striae (Wickham’s striae). Loss of the
labia minora, narrowing of the introitus, and obliteration of the vagina
occur with disease progression.
8. Van de Nieuwenhof HP, Meeuwis KA, Nieboer TE, Vergeer
MC, Massuger LF, De Hullu JA. The effect of vulvar lichen
sclerosus on quality of life and sexual functioning. J
Psychosom Obstet Gynaecol. 2010;31(4):279-284.
9. Burrows LJ, Creasey A, Goldstein AT. The treatment of
vulvar lichen sclerosus and female sexual dysfunction
[published online ahead of print October 18, 2010]. J
Sex Med. 2011;8(1):219-222. doi: 10.1111/j.17436109.2010.02077.x.
10. Neill SM, Lewis FM, Tatnall FM, Cox NH. British Association
of Dermatologists’ guidelines for the management of lichen
sclerosus 2010. Br J Dermatol. 2010;163(4):672-682.
11. Hengge UR, Krause W, Hofmann H, et al. Multicentre,
phase II trial on the safety and efficacy of topical tacrolimus ointment for the treatment of lichen sclerosus. Br J
Dermatol. 2006;155(5):1021-1028.
12. Goldstein AT, Creasey A, Pfau R, Phillips D, Burrows LJ.
A double-blind, randomized controlled trial of clobetasol versus pimecrolimus in patients with vulvar lichen
sclerosus. J Am Acad Dermatol. 2011;4(6):e99-104.
13. Cooper SM, Haefner HK, Abrahams-Gessel S, Margesson
LJ. Vulvovaginal lichen planus treatment: a survey of current practices. Arch Dermatol. 2008;144(11):1520-1521.
14. Goldstein AT, Metz A. Vulvar lichen planus. Clin Obstet
Gynecol. 2005;48(4):818-823.
The Female Patient | VOL 37 APRIL 2012 5
The Vulvar Dermatoses
Coding for The Vulvar Dermatoses:
Part of the Differential Diagnosis for Sexual Dysfunction
Philip N. Eskew Jr, MD
This important article discusses a clinical condition that requires a great deal of time discussing the impact on a patient’s
life. If seen during an annual examination, you should request that the patient return for a visit where you can not only
obtain a comprehensive history but also perform indicated biopsies. You may need to wait on the biopsy results before you
submit your claim. Several of the ICD-9 codes mentioned in this article are:
692
693
625.0
625.7
625.9
697.0
698.1
Contact dermatitis and other eczema
(692.0 – 692.9)
Dermatitis due to substances taken internally
(693.0 – 693.9)
Dyspareunia
Vulvodynia
Unspecified symptom associated with female
genital organs (chronic vulvar pain)
Lichen planus (lichen planopilaris, ruber planus)
Pruritus of genital organs
698.3
701.0
Lichenification and lichen simplex chronicus
(Hyde’s disease, neurodermatitis [circumscripta]
[local], prurigo nodularis)
Circumscribed scleroderma (lichen sclerosus
et atrophicus)
As mentioned in the article, the use of the colposcope
can be very helpful. The CPT codes for its use in examining
the vulva are:
56820 Colposcopy of the vulva
56821
with biopsy(s)
Philip N. Eskew Jr, MD, is past member, Current Procedural Terminology (CPT) Editorial Panel; past member, CPT Advisory Committee; past
chair, ACOG Coding and Nomenclature Committee; and instructor, CPT coding and documentation courses and seminars.
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