Whitish Genital Lesions

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, 1,2 include: 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

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 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 cancer – there is an increased risk of A B • vulvar SCC of approximately 5%.1,2 Figure 2. Histology and dermoscopy of lichen sclerosus of the vulva (LSV) a. Histology of LSV; b. Dermoscopy of LSV (A = Structureless white patches [sclerosus], B = Ice ANSWER 6 sliver, C = Scattered linear vessels mixed with the whitish structure) Goals of treatment are to: 1) alleviate symptoms of pruritus, fissuring and pain,

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. Devita Surjana MBBS, PhD, FACD, Dermatologist, J Aust 2003;178(8):391–95. doi: 10.5694/j.1326- 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. Manisha Singh MD, FRCPA, Dermatopathologist, Fam Physician 2011;40(7):490–96. 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. [email protected] 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 References 2013;14(1):27–47. doi: 10.1007/s40257-012-0006-4. 1. Marfatia Y, Surani A, Baxi R. Genital lichen 8. Lewis F. Dermotoses of the female genitalia. In: sclerosus et atrophicus in females: An update. Griffiths CEM, Baraker J, Bleiker T, Chalmers R, Indian J Sex Transm Dis AIDS 2019;40(1):6–12. Creamer D, editors. Rook’s textbook of dermatology. doi: 10.4103/ijstd.IJSTD_23_19. 9th edn. Oxford, UK: John Wiley & Sons, 2016. 2. Nair PA. Vulvar lichen sclerosus et atrophicus. 9. Kirtschig G. Lichen sclerosus-presentation, J Midlife Health 2017;8(2):55–62. doi: 10.4103/jmh. diagnosis and management. Dtsch Arztebl JMH_13_17. correspondence [email protected]

GLW0001_RACGP ad_210x125_art.indd 1 11/1/21 4:06 pm © The Royal Australian College of General Practitioners 2021 Reprinted from AJGP Vol. 50, No. 1–2, Jan–Feb 2021 59