Whitish genital lesions - RACGP

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Whitish genital lesions - RACGP
Clinical

Whitish genital lesions

                                                                                                             QUESTION 3
Tim Aung, Devita Surjana, Manisha Singh                        •   lichen simplex chronicus
                                                               •   lichen planus                             What is lichen sclerosus and what are the
                                                               •   post-inflammatory hypopigmentation        clinical features of LSV?
CASE                                                           •   morphoea
A woman aged 40 years presented for a                          •   extramammary Paget disease                QUESTION 4

cervical screening test (CST). She was a                       •   vulvar intraepithelial neoplasia (VIN).   What are the aetiology and epidemiology
mother of three and a recently arrived                         The pale-white and ivory-white                of LSV?
refugee. She had never had a Pap smear                         discolouration of the vulva and clitoris
or CST in the past because she had been                        with some anatomical distortion make          ANSWER 3

living in a remote area for decades. The                       LSV the most likely diagnosis in this case.   Lichen sclerosus is a chronic inflammatory
purpose of the test was fully explained                        If there is a prominent erythematosus         dermatosis commonly affecting the
to the patient. When asked before the                          with scales, then psoriasis and contact       anogenital region. The condition is
procedure about routine symptomatology                         dermatitis can also both be considered as     characterised by white sclerotic patches
including menstruation, discharge and                          differential diagnoses. Table 1 compares      that subsequently coalesce, becoming
itchiness, the patient had no complaints.                      and contrasts the features of LSV with        shiny porcelain-white or ivory-white
   During the procedure, in the presence                       other similar conditions.                     colour. When it affects the vulva, it is
of a chaperone female practice nurse,
whitish discolouration of the vulva,                           ANSWER 2

involving both labia majora and minora,                        A diagnosis of LSV can be made
and clitoris with distorted anatomy was                        clinically without a mandatory
noted (Figure 1). On further enquiry, the                      biopsy. However, a punch biopsy
patient stated she had experienced mild                        from the white sclerotic area is highly
pruritus for several years, and she thought                    recommended to confirm the diagnosis
it was due to friction from walking.                           and exclude alternative diagnoses
                                                               including squamous cell carcinoma
                                                               (SCC). The histopathology usually
QUESTION 1                                                     illustrates atrophic or hyperkeratotic
What is the differential diagnosis based                       epidermis with lichenoid infiltrate in
on this presentation, and what is the most                     the dermal–epidermal junction and
likely diagnosis?                                              the homogenisation of collagen in the
                                                               upper dermis.2–4
QUESTION 2

How will you definitively diagnose this
condition?                                                     CASE CONTINUED

                                                               The patient was informed about
ANSWER 1                                                       the unusual discolouration and the
Given whitish discolouration of the vulva                      possibility of a genital skin disorder.
in a woman aged 40 years, the differential                     She was scheduled for a biopsy to guide
                                                                                                             Figure 1. Lichen sclerosus of vulva with some
diagnosis for this particular case can                         management and rule out SCC. The
                                                                                                             anatomical distortion (A = Buried clitoris,
include:1,2                                                    biopsy confirmed lichen sclerosus with        B = Involvement of clitoris hood, C = Distorted
• lichen sclerosus of the vulva (LSV)                          no evidence of neoplasia (Figure 2).          labia minora, D = Extending to perineum)
• vitiligo

© The Royal Australian College of General Practitioners 2021                                                   Reprinted from AJGP Vol. 50, No. 1–2, Jan–Feb 2021   55
Whitish genital lesions - RACGP
Clinical                                                                                                                                        Whitish genital lesions

Table 1. Differential diagnoses of lichen sclerosus of the vulva9–13

                          Primary lesion                  Main            Genital        Extragenital   Possible
                          features                        symptom         involvement    involvement    dermoscopic features             Histology/microscopy

Lichen sclerosus          White patches with              Prominent       Very common    Rare           White structureless              Hyalinisation of
of the vulva (LSV)        sclerotic skin texture,         itch (+++)      (80%)          (15–20%)       areas with linear                upper dermis, with a
                          fissures, erosions                                                            vessels, ice slivers,            band of lymphocytes
                                                                                                        comedo-like openings             below; atrophic or
                                                                                                        (hair-bearing area)              hyperkeratosis epidermis

Vitiligo                  Well-defined                    No itch         Less*          More common*   Homogenous white                 Total absence of
                          depigmentation with                                                           structureless areas              functioning melanocytes,
                          no alteration of skin                                                         with absent or reduced           with the inflammatory
                          texture                                                                       pigment network                  lymphocyte on the edges
                                                                                                                                         of the lesions

Morphea                   Thick, hard skin                No itch         Rare           More common*   White-yellowish                  Atrophic epidermis with
                          (sclerotic/fibrosis)                                                          structureless areas,             increased collagen in
                                                                                                        linear vessels within            the dermis and loss of
                                                                                                        the lilac ring                   appendageal structures

Lichen planus             Hypertrophic erosions           Pain is         Less*          More common*   White crossing streaks           Hyperkeratosis and
                          of the vaginal introitus        greater                                       (Wickham striae)                 acanthosis; saw-toothing
                          (LSV does not affect            than itch                                                                      of rete pegs, band-like
                          vaginal mucosa)                                                                                                chronic inflammatory
                                                                                                                                         infiltrate obscuring
                                                                                                                                         the dermo-epidermal
                                                                                                                                         junction

Lichen simplex            Lichenification                 Itch (+++);     Less*          More common*   Scales, exaggerated              Marked hyperkeratosis
chronicus                 with scaling                    scratching is                                 skin markings                    associated with foci of
                                                          pleasurable                                                                    parakeratosis, prominent
                                                                                                                                         granular cell layer,
                                                                                                                                         papillary dermal fibrosis

Dermatitis                Erythematosus with              Itch (++)       Common,        Common,        Red dots in a patchy             Extensive spongiosis;
(atopic/contact)          or without scaling                              depending on   depending on   distribution and yellow          initially acute spongiotic
                          and lichenification                             triggers       triggers       scales                           dermatitis, evolving into
                                                                                                                                         subacute or chronic
                                                                                                                                         spongiotic dermatitis

Psoriasis                 Erythematosus with              Itch (+)        Less*          More common*   Scales and dotted                Regular acanthosis,
                          or without scaling                                                            vessels; under                   confluent parakeratosis,
                          and fissures                                                                  high-power imaging,              supra-papillary plate
                                                                                                        dilated, elongated and           thinning
                                                                                                        convoluted capillaries
                                                                                                        are visible

Cicatricial               Blisters (bullae) or            Pain            Rare           Mouth          NA                               Immunofluorescence
pemphigoid                erosions                                                                                                       analysis – linear
                                                                                                                                         deposition of
                                                                                                                                         immunoglobulin (Ig) G
                                                                                                                                         or IgA, and complement
                                                                                                                                         (C3) at the basement
                                                                                                                                         membrane zone

Vulvovaginal              Red, inflamed mucosa            Itch (+++)      Common         NA             NA                               Confirmation of
candidiasis               with white-curd                                                                                                candidiasis using vaginal
                          discharge                                                                                                      swab for microscopy,
                                                                                                                                         culture and sensitivities

Note: Itch is classified as mild (+), moderate (++) or severe (+++)
*When compared with LSV
NA, not applicable

56   Reprinted from AJGP Vol. 50, No. 1–2, Jan–Feb 2021                                                        © The Royal Australian College of General Practitioners 2021
Whitish genital lesions - RACGP
Whitish genital lesions                                                                                                                                      Clinical

called vulvar lichen sclerosus or LSV. It                      neck, thigh, buttock and breast, is seen in   sclerosus is underreported because of a
was previously known as lichen sclerosus                       15–20% of cases.2 Figure 3 shows various      number of factors: lack of awareness of the
et atrophicus, kraurosis vulvae, leukoplakic                   morphologies of genital lichen sclerosus.     patient and practitioner; embarrassment
vulvitis and lichen albus. When it affects                        Dermoscopically, patchy white              and reluctance to disclose symptoms; and
the penis, the term balanitis xerotica                         structureless areas, ice slivers,             presentation at different practitioners
obliterans has been used historically.1,5                      comedo-like openings (hair bearing area       of general practice, sexual health,
   Occasionally LSV can be asymptomatic                        only), purpuric globules and dotted or        gynaecology, urology and dermatology.1,3,6
and discovered incidentally during CST.3,6                     sparse thin linear vessels can be seen        As a result, delays in diagnosis and
The possible clinical manifestations of LSV                    (Figure 2).3                                  undertreatment are not uncommon.
include:                                                          Lichen sclerosus can be associated with    Although earlier literature reported that
• pruritus (often intractable), pain and                       autoimmune-related diseases such as           lichen sclerosus and LSV generally affect
   bleeding from fissuring and erosion                         thyroid disease, vitiligo, alopecia areata    individuals of Caucasian descent, the
• dyspareunia and other sexual                                 and pernicious anaemia.2,7                    condition can be seen in patients of any
   dysfunction                                                                                               ethnicity.1
• constipation and painful defecation if                       ANSWER 4

   perianal skin is involved                                   The exact aetiology of lichen sclerosus
• atrophy and distortion of anatomical                         remains speculative. Several theories         CASE CONTINUED

   structures including burying of the                         such as autoimmune (approximately             The patient underwent a blood test for
   clitoris, fusion or loss of labia minora,                   20% association), genetics (12% positive      autoantibody screening, and the results
   stenosis of the introitus, and distortion                   family history), hormonal factors and         showed no associated autoimmune
   of urethral orifice resulting in urinary                    chronic trauma/irritation have been           disorders; such investigation is not
   problems.                                                   proposed.1,7,8 LSV commonly affects           routinely required for every case. She had
Morphologically, LSV results in an atrophic                    individuals from the fifth decade onwards     no other skin disorders. She was referred
or hyperkeratotic surface of the vulva with                    but can be seen at any age including          to a dermatology clinic at public hospital.
white sclerotic papules, patches or plaques,                   prepuberty. Approximately 30–50%              While waiting to be seen by a specialist,
which may extend to the perineum and                           of affected women develop symptoms            the patient was treated with potent topical
perianal area. Areas of purpura, fissures                      prior to menopause.6,9 The prevalence         corticosteroid (TCS; mometasone furoate
and erosion can sometimes be seen.                             of LSV is estimated to be one in 30 older     0.1% ointment) with general advice as
Extragenital lichen sclerosus, which                           women and one in 900 prepubertal girls.1      per current guidelines. She had some
predominantly affects the shoulder,                            Most scholars suggest that genital lichen     improvement in symptoms and skin
                                                                                                             texture within six weeks.

                                                                                                             QUESTION 5

                                                                                                             What are the complications of LSV?

                                                                                                             QUESTION 6

                                                                                                             What are the management options for LSV?

                                                                                                             ANSWER 5

                                                                                                             The complications of LSV are:
                                                                                                             • anatomical distortion/alteration – as
                                                                                                               described in the clinical manifestations,
                                                                                                               resulting in sexual dysfunction plus
                                                                                                               urinary and bowel opening problems
                                                                                                             • psychological – LSV significantly affects
                                                                                                               the individual’s sexual function and
                                                                                                               quality of life, resulting in psychological
                                                                                                               distress and low self-esteem
 A                                                             B
                                                                                                             • cancer – there is an increased risk of
                                                                                                               vulvar SCC of approximately 5%.1,2
Figure 2. Histology and dermoscopy of lichen sclerosus of the vulva (LSV)
                                                                                                             ANSWER 6
a. Histology of LSV; b. Dermoscopy of LSV (A = Structureless white patches [sclerosus], B = Ice
sliver, C = Scattered linear vessels mixed with the whitish structure)                                       Goals of treatment are to: 1) alleviate
                                                                                                             symptoms of pruritus, fissuring and pain,

© The Royal Australian College of General Practitioners 2021                                                   Reprinted from AJGP Vol. 50, No. 1–2, Jan–Feb 2021   57
Whitish genital lesions - RACGP
Clinical                                                                                                                                         Whitish genital lesions

2) improve sexual function and quality                    and inexpensive to use, and it is effective         irritants may include hygiene products,
of life and 3) reduce scarring (structural                in 90% of patients.10                               creams, lubricants, contraceptives and
distortion) and risk of cancer.8 The                         It is advisable to review the patient in         procedures (eg improper hair removal).
detailed management for LSV is outlined                   4–6 weeks and three months from the                 Tight underwear and any activities
as follows.                                               start of TCS therapy. A 6–12-monthly                that can rub onto the sensitive mucosa
   Ultra-potent or potent TCSs are the                    follow-up is recommended during                     (eg riding a bicycle or horse) should also
first line of treatment. They provide                     maintenance treatment. Treatment failure            be avoided.10,11
both symptomatic relief and clinical                      at any stage indicates the need to consider      • Advise patients to become familiar with
improvement, reducing complications of                    an incorrect diagnosis, noncompliance               the appearance of their genital area,
scarring and malignant change.3,4,9 TCSs                  issue, development of VIN or SCC, or                as lifelong monitoring is required to
(one fingertip unit = 0.5 g) are applied                  superimposed factors such as allergic               detect, diagnose and treat new lesions
twice daily until symptoms (itchiness,                    reaction to specific medications, infection         or scarring.
soreness) are relieved (1–2 weeks),                       (candida, virus [herpes simplex virus],          Consider multidisciplinary care involving
then reduced to daily application until                   bacteria [Staphylococcus spp., Gardnerella       a local vulvar clinic (if available) or
the return of normal texture of the                       spp.]), and irritation from excess sweat         dermatologist or gynaecologist with an
skin (usually 1–2 months), and later on                   or urinary and faecal incontinence.              interest in lichen sclerosus. For example,
alternative days, totalling approximately                 There is no role for topical testosterone        surgery for correction of anatomical
three months from the start of treatment.                 and oestrogen.                                   distortion or treatment of early carcinoma
Afterward, maintenance treatment using                       General measures include the following:       may be needed.
lower potency (mid-strength) TCSs such                    • Counsel patients about the nature of
as betamethasone valerate (0.02%),                           disease, course, treatment and need for
triamcinolone acetonide (0.02%) or                           follow-up. Some individuals may need          Discussion and conclusion
methylprednisolone aceponate (0.1%)                          reassurance that lichen sclerosus is not a    General practitioners (GPs) have an
twice weekly is generally recommended.                       sexually transmissible infection.             opportunity to detect genital skin lesions
Frequency of TCS application can                          • Advise patients to avoid scratching and        while performing a CST. It is prudent
be individualised depending on                               irritants to the genital area by using soap   for GPs to be familiar with characteristic
hyperkeratosis. Ointment-based TCSs are                      substitutes for cleaning and applying         features of genital skin disorders to be
commonly preferred to cream in genital                       a protective barrier (eg soft paraffin or     effectively able to carry out further actions.
areas because of better absorption as well                   emollient) to minimise the contact with       Early detection and treatment with timely
as barrier function.6,9 TCS therapy is safe                  sweat, urine and faeces. Other vulvar         referral for genital skin disorders such as
                                                                                                           LSV will reduce the impact on the patient’s
                                                                                                           morbidity both physically and mentally.
                                                                                                           Management of vulvar skin disorders
                                                                                                           spans dermatology, gynaecology and
                                                                                                           sexual health, and referral to a pertinent
                                                                                                           specialist is recommended depending
                                                                                                           on the severity and complication of the
                                                                                                           disease. The prognosis of LSV is usually
                                                                                                           favourable if diagnosed and treated in the
                                                                                                           early nonscarring stages.

                                                                                                           Key points
                                                                                                           •   Genital skin disorders are generally
                                                                                                               underreported because of various
                                                                                                               factors.
                                                                                                           •   GPs have an opportunity to detect
                                                                                                               genital skin lesions while performing a
                                                                                                               CST or investigating a patient’s direct
                                                                                                               concerns.
 A                                                        B
                                                                                                           •   Early detection and treatment
Figure 3. Various morphology of lichen sclerosus of the vulva (LSV)                                            for genital skin disorders such as
a. LSV with typical shiny porcelain-white vulva (image courtesy of DermNet NZ); b. LSV with                    LSV will reduce the impact on the
erosion and fissures (image courtesy of DermNet NZ)                                                            patient’s morbidity physically and
                                                                                                               psychologically.

58   Reprinted from AJGP Vol. 50, No. 1–2, Jan–Feb 2021                                                         © The Royal Australian College of General Practitioners 2021
Whitish genital lesions                                                                                                                                                 Clinical

             Authors                                                        3. Lee A, Fischer G. Diagnosis and treatment                Int 2016;113(19):337–43. doi: 10.3238/
             Tim Aung FRACGP, FRNZCGP, ProfDip (Skin                           of vulvar lichen sclerosus: An update                    arztebl.2016.0337.
             Cancer Surg), ProfDip (Gen Derm), Primary Care                    for dermatologists. Am J Clin Dermatol                10. Welsh BM, Berzins KN, Cook KA, Fairley CK.
             Practitioner, Qld                                                 2018;19(5):695–706. doi: 10.1007/s40257-018-              Management of common vulval conditions. Med
                                                                               0364-7.                                                   J Aust 2003;178(8):391–95. doi: 10.5694/j.1326-
             Devita Surjana MBBS, PhD, FACD, Dermatologist,
             Cutis Medical, Northern Dermatology and Sunnybank              4. Cyrus N, Jacobe HT. Morphea and lichen                    5377.2003.tb05257.x
             Dermatology, Qld                                                  sclerosus. In: Kang S, Amagai M, Bruckner AH,         11. Drummond C. Common vulval dermatoses. Aust
                                                                               et al, editors. Fitzpatrick’s Dermatology. 9th edn.       Fam Physician 2011;40(7):490–96.
             Manisha Singh MD, FRCPA, Dermatopathologist,
                                                                               New York, NY: McGraw-Hill Education, 2019; p.
             Infinity Pathology, Qld                                                                                                 12. Errichetti E, Stinco G. Dermoscopy in general
                                                                               1116–122.
             Competing interests: None.                                                                                                  dermatology: A practical overview. Dermatol Ther
                                                                            5. Oakley A. Lichen sclerosus. Hamilton, NZ:                 (Heidelb) 2016;6(4):471–507. doi: 10.1007/s13555-
             Funding: None.                                                    DermNet NZ, 2016. Available at https://                   016-0141-6.
             Provenance and peer review: Not commissioned,                     dermnetnz.org/topics/lichen-sclerosus [Accessed
                                                                                                                                     13. Lallas A. Dermoscopy in general dermatology/
             externally peer reviewed.                                         17 June 2020].
                                                                                                                                         inflammoscopy. Graz: Dermoscopedia, 2019.
             Correspondence to:                                             6. Fisher G. Vulval lichen sclerosus – Diagnosis and         Available at https://dermoscopedia.org/w/index.
             timmynz2006@gmail.com                                             treatment. Med Today 2019;20(1): 21–29.                   php?title=Inflammoscopy&oldid=16575 [Accessed
                                                                            7.   Fistarol SK, Itin PH. Diagnosis and treatment of        17 June 2020].
                                                                                 lichen sclerosus: An update. Am J Clin Dermatol
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                JMH_13_17.                                                                                                                      correspondence ajgp@racgp.org.au

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             © The Royal Australian College of General Practitioners 2021                                                              Reprinted from AJGP Vol. 50, No. 1–2, Jan–Feb 2021   59
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