Original Article  http://dx.doi.org/10.3126//njdvl.v17i1.23250

Clinico-epidemiological Profile of Women with Non- Venereal Vulval Diseases: A Hospital-Based Observational Study

Joshi S1, Shrestha S1, Joshi A2

1Department of Dermatology and Venereology, 2Department of Gynecology and Obstetrics, Nepal Medical College Teaching Hospital, Attarkhel, Gokarneshwor, Kathmandu, Nepal

Abstract Introduction: Vulval disorders can be of venereal and non-venereal etiology. Establishing non-venereal causation of vulval disorder helps in alleviating fears in patients with the condition. These patients are better dealt in a multidisciplinary clinic as patients with these disorders frequently visit dermatologists and gynecologists for the treatment. Objectives: To study the clinico-demographic profile of women with non-venereal vulval disorders and to determine their relative frequency. Materials and methods: This is an observational, descriptive study done at the Departments of Dermatology and Venereology and Gynecology and Obstetrics, Nepal Medical College Teaching Hospital. All consenting female patients with problems pertaining to female external genitalia were recruited for the study after excluding venereal diseases. Details of the patients were obtained and entered in a predesigned proforma. Results: Seventy-five females were recruited during a period of 20 months with a mean age of 34.79±17.90 years. Majority were married, uneducated and homemakers. Duration of disease ranged from three days to 35 years. Itching was the commonest presenting complaint (82.67%) followed by redness (32.00%), burning sensation (26.67%), white lesions (24.00%) and pain (24.00%). Commonest diagnosis was lichen sclerosus (17.33%), followed by candidiasis (14.67%). Patients presenting with vulval symptoms without lesions were diagnosed with non-specific vulval pruritus (9.33%) and (2.67%). Conclusion: Itching is the most common presenting complaint and contrary to the popular belief, inflammatory disorders especially lichen sclerosus, rather than infections were common diagnoses in females with non-venereal vulval disorders. Key words: Lichen Sclerosus et Atrophicus; Pruritus Vulvae; Vulvodynia

Introduction range from common problems like itching to diffi culty in maintaining a healthy sexual rela onship and isorders of female external genitalia are far and may result in a range of physical, psychological and Dwide and encompasses spectrum of diseases psychosoma c manifesta ons.2 ranging from infl ammatory disorders, autoimmune diseases, infec ons, benign and malignant neoplasms.1 Submitted: 28th October 2018 Diseases involving may be broadly classifi ed Accepted: 30th January 2018 into those of venereal and non-venereal e ology. Published: 31st March 2019 Non-venereal diseases of vulva may be mistaken for venereal diseases, thus escala ng mental distress How to cite this article and guilt in pa ents and may be a cause for marital Joshi S, Shrestha S, Joshi A. Clinico-epidemiological profile of women with non-venereal vulval diseases: a hospital- disharmony. Furthermore, associated symptoms based observational study. Nepal Journal of Dermatology, Address of Correspondence: Venereology and Leprology. 2019;17(1):32-8. doi: http://dx.doi. Dr. Smita Joshi, MD org/10.3126//njdvl.v17i1.23250 Lecturer Department of Dermatology and Venereology Nepal Medical College Teaching Hospital, Attarkhel Licensed under CC BY 4.0 International License which permits Gokarneshwor, Kathmandu, Nepal use, distribution and reproduction in any medium, provided E-mail: [email protected] the original work is properly cited.

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The prevalence of females with vulval diseases from a Final diagnosis was based on clinical judgement and/ ter ary health center in eastern Nepal was reported to or inves ga ons made jointly by a dermatologist and a be 1.9%.3 This would be just the p of the iceberg as gynecologist. Pa ent par culars, history, examina on female are generally poorly represented in the hospital fi ndings, inves ga ons done, and fi nal diagnosis were sta s cs in a developing country like Nepal, where entered in a predesigned proforma. women generally tend to downplay their illness over other important ma ers of family and society. The Data obtained were entered in Microso Excel 2010 private nature of illness and the embarrassment one and descrip ve sta s cs were obtained. Quan ta ve feels while le ng the other person examine the part data were tabulated and interpreted in terms of in ques on makes the ma er worse. percentage, mean and standard devia on. Qualita ve data were represented in numbers and percentage. Diseases of female external genitalia, being a gray area, as to whether gynecologists or dermatologists are at Results be er posi on to treat the condi on, pose further problem in management of pa ents. Pa ents seek A total of 75 females with non-venereal vulval consulta on from mul tude of specialists, resul ng in disorders were included in the study during the study frustra on in the pa ent and the trea ng physician. period of 20 months. Mean age of study group was This study aims to determine the demographic profi le, 34.79±17.90 years (Range: four months to 75 years) rela ve frequency and characteris cs of non-venereal [Table 1]. Majority of pa ents belonged to 29 - 38 vulval diseases in Dermatology and Gynecology Clinic years age group [Figure 1]. Thirteen pa ents (17.80%) of a ter ary hospital. were in the pediatric age group (≤18 years). Majority of pa ents were married (74.67%) and presently residing Materials and Methods in Kathmandu valley (89.33%). Majority of females had not received any formal educa on (44.00%) and were The study is a cross-sec onal, descrip ve study fi nancially dependent (78.67%) [Table 1]. conducted at the Out-pa ent Department of Dermatology and Venereology, and Gynecology and Dura on of illness ranged from three days to 35 years Obstetrics, Nepal Medical College Teaching Hospital with maximum pa ents with disease dura on of ≤ 1 during a period of 20 months (October 2016 – May month (36.00%) [Table 1]. Itching was the commonest 2018). Ethical approval was obtained from Ins tu onal presen ng complaint being observed in 62 individuals Review Commi ee (IRC), Nepal Medical College. (82.67%) [Figure 2]. Concomitant medical illness was present in 16 females (21.33%). Other dermatological All female pa ents of any age with symptoms or lesions illness was present in nine individuals (12.00%) [Table pertaining to external genitalia were screened for 2]. Posi ve family history of similar illness was present presence of non-venereal vulval disorders. Informed in one pa ent each of psoriasis and dermatophytosis. consent was obtained from the pa ents and guardians of minors with newly diagnosed, non-venereal vulval Labia majora was the commonest site of involvement diseases for inclusion in the study. Cases with venereal (58.67%) [Table 1]. Fi y-eight pa ents had bilateral e ology were excluded from the study. Details of the involvement (77.33%). Extra-genital involvement was pa ent including age, place of residence, educa onal observed in two pa ents of candidiasis (inner thigh, status, marital status, occupa on, symptoms, dura on groin), two pa ents of nea cruris involving vulva of disease, menstrual history, obstetric history, (infra-mammary area, groin), and one pa ent each associated cutaneous and medical illness were of Behcet’s disease (oral aphthae), lichen simplex obtained. A comprehensive clinical examina on and chronicus (similar lesion in right shin), herpes zoster a thorough examina on of the external genitalia were (ipsilateral bu ock and inner thigh) and vulval eczema done in adequate light. Sites of involvement were noted. (inner thigh). The commonest diagnosis observed was Inves ga ons like Gram’s stain, Potassium hydroxide lichen sclerosus (17.33%). Other common diagnoses mount, bacterial culture and sensi vity, rou ne and in decreasing order of frequency were candidiasis microscopic examina on of urine and stool, rou ne (14.67%), vulval eczema (10.67%) and non-specifi c blood examina on, serological tests for venereal vulval pruritus (9.33%). Among those presen ng diseases (Human Immunodefi ciency Virus an body, without any skin lesion, seven had the diagnosis of Venereal Disease Research Laboratory test for syphilis, non-specifi c vulval pruritus (9.33%) and two were Herpes an body tre), biopsy and histopathological diagnosed with vulvodynia (2.67%) [Figure 3]. examina on etc. were advised to be done in respec ve labs as and when indicated to support the diagnosis.

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20 18 16 14 12 10 8 6 Number of patients 4 2 0 ≤18 years 19-28 29-38 39-48 49-58 59-68 ≥69 years years years years years years

Figure 1: Age distribu on in pa ents with non-venereal vulval disorders.

Pricking 1 Thinning of skin 1 Fluid fi lled lesions over skin 1 Maceration 2 Fissuring 2 8 Raised lesions over skin 10 Discharge 13 Erosion/ ulceration 14 Swelling 14 Dysuria 15 Thickening of skin 15 Pain 18 White lesions 18 Burning 20 Redness 24 Itching 62

010203040506070 Number of patients

Figure 2: Presenta on in pa ents with non-venereal vulval disorders.

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Table 1: Socio-demographic profi le and clinical Table 2: Concomitant medical and dermatological parameters of study popula on. illness in study popula on. Mean age 34.79±17.90 years Number of pa ents Marital status (Percentage) Married 56 (74.67%) Medical illness Unmarried 19 (25.33%) Hypertension 4 (5.33%) Residence Diabetes mellitus 3 (4.00%) Kathmandu valley 67 (89.33%) Hypothyroidism 2 (2.67%) Outside Kathmandu valley 8 (10.67%) Acid pep c disease 2 (2.67%) Educa on Osteoarthri s 1 (1.33%) None 33 (44.00%) Depression 1 (1.33%) Primary 11 (14.67%) Migraine 1 (1.33%) Secondary 16 (21.33%) Higher Secondary 9 (12.00%) Asthma 1 (1.33%) Undergraduate 5 (6.67%) Tuberculosis 1 (1.33%) Chronic obstruc ve Postgraduate 1 (1.33%) 1 (1.33%) Occupa on pulmonary disease Homemaker 41 (54.67%) Enteric fever 1 (1.33%) Student 15 (20.00%) Dermatological illness Unemployed 3 (4.00%) Photoderma s 2 (2.67%) Employed 16 (21.33%) Hand/ foot eczema 2 (2.67%) Dura on of illness Atopic derma s 1 (1.33%) ≤ 1 month 27 (36.00%) Chronic ur caria 1 (1.33%) 1 month to ≤ 1 year 22 (29.33%) Palmoplantar psoriasis 1 (1.33%) > 1 year 26 (34.67%) Acne vulgaris 1 (1.33%) Menstrual history Herpes genitalis 1 (1.33%) Premenstrual 9 (12.00%) Menstrual – Regular 39 (52.00%) Menstrual – Irregular 9 (12.00%) disadvantage due to the fact that there is no clear cut Post-menopausal 18 (24.00%) dis nc on as to whether its place lies in dermatology Obstetric history or gynecology. The establishment of inter-disciplinary Nulliparous 27 (36.00%) vulval disease clinic with a team of dermatologists, Parous 48 (64.00%) gynecologists and pathologists working together has Pregnant 4 (5.33%) found its way especially in developed na ons which Sites of involvement has greatly increased the clinician’s understanding Labia majora 44 (58.67%) of vulval diseases and “the forgo en pelvic organ”, Labia minora 38 (50.67%) the a en on it deserves.7-10 Such a concept of vulval Perineum 12 (16.00%) disease clinic is s ll a far-fetched idea in a resource Anterior commissure 11 (14.67%) poor country like Nepal. Mons pubis 8 (10.67%) Ves bule 5 (6.67%) Interna onal Society for the Study of Vulvovaginal 5 (6.67%) Disease (ISSVD) classifi ed the vulval diseases as Fourche e 5 (6.67%) either vulval dermatoses (for condi ons associated with visible skin changes) or vulval dysesthesia (for Discussion condi ons that cause vulval pain and without visible skin changes).11 Vulval dysesthesia encompasses Diseases of the vulva are of paramount importance, vulvodynia, ves bulodynia, clitorodynia etc. and however are o en neglected and can be considered vulval dermatoses includes infl ammatory cutaneous an orphan disease.4,5 Although vulva is the most diseases (lichen sclerosus, lichen planus, psoriasis), approachable part of female pelvic organ, health autoimmune (vi ligo), mul system illness (Behcet’s prac oners seldom take me to stop and look for its syndrome, Crohn’s disease), exogenous (contact changes. Vulva thus is referred in the literature as “the eczema, drug erup ons, infec ons), benign and forgo en pelvic organ”.6 Vulval maladies o en are at malignant neoplasms.1,2

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Lichen sclerosus (13) Candidiasis (11) Vulval eczema (8) Non-specifi c vulval pruritus (7) Genital aphthosis (5) Lichen planus (4) (4) Vitiligo (4) Tinea cruris (3) Bartholin’s cyst (2) Vulval edema (2) Vulvodynia (2) Behcet’s disease (1) Herpes zoster (1) Hypertrophic scar (1) Infantile hemangioma (1) Inverse psoriasis (1) Lymphangiectasia (1) Mucinous cyst (1) Steatocystoma multiplex (1) Vulval abscess (1) Vulval melanosis (1)

Figure 3: Final diagnosis among pa ents with non-venereal vulval disorders.

Mean age of 34 years in our study is in concordance similar to the study done by Pathak et al (11.40%). It with previous studies from India and Turkey evalua ng should be noted that Pathak et al reported signifi cant vulval dermatoses,7,12 but is slightly higher than the associa on of vulval diseases with irregular menses study done in eastern Nepal.3 It should be noted that in their study and raised the possibility of eff ect of we excluded pa ents with venereal diseases in the hormonal imbalance leading to epithelial fragility and present study as compared to inclusion of venereal and predisposi on to irrita on.3 non-venereal vulval diseases in the study by Pathak et al and Gokdemir et al.3,7 Nearly 3/4th of pa ents were Labia majora was the commonest site of involvement married which is similar to the results in study by in present study which is in concordance with Pathak et al and Singh et al.3,12 This is in contrast to the previous studies.12,14 We encountered 22 diff erent study by Sartori et al wherein majority (58.7%) were vulval disorders in our cohort of pa ents. Among single women.13 It should be noted that only 1/5th of them lichen sclerosus was the commonest condi on, pa ents had received higher secondary educa on or being diagnosed in 17.33% of individuals. Fischer and above and 1/5th had reported that they were engaged Rogers evaluated 130 prepubertal girls with vulval in some form of occupa on. This gives a rough portrayal complaints and reported commonest diagnosis to be of pa ent’s knowledge, economic dependence, their atopic or irritant derma s (33%). Other common prac ces and health seeking behavior, which could disorders included lichen sclerosus (18%), psoriasis be poten al barriers in the op mal management of (17%), hemangioma and nevi (12%) and streptococcal females with vulval diseases. Furthermore, 90% of vulvovagini s (10%).15 In the present study, out of 13 the pa ents in present study were currently residing girls of ≤18 years, lichen sclerosus (23.07%) and genital in Kathmandu valley. The poorer socio-economic and aphthosis (23.07%) were the commonest diagnoses. educa onal status of females in far-fl ung rural areas of Nepal renders management of readily iden fi able and In the study by Gokdemir et al, 33.54% had specifi c easily treatable vulval condi ons more inaccessible to dermatological condi ons, commonest being vi ligo, them. psoriasis, contact derma s and lichen simplex chronicus, 32.25% had vulval infec ons and 26.45% Itching was the predominant symptom in present vulval pruritus.7 In another study by Hansen et al, study (82.67%) which is in agreement to the study similar fi nding was noted with 47.5% of women with from eastern Nepal (82.8%) and two other studies vulvovaginal complaint having diagnosed with specifi c from India (60% and 61.7%).3,12,14. Other common dermatological condi on.16 This emphasized the symptoms were redness (32.00%), burning sensa on role of dermatologist in the management of vulval (26.67%), white lesions (24.00%) and pain (24.00%). disorders which many consider to be gynecologist’s Co-morbi es were reported in 21.33% in the present forte. Gokdemir et al further underscored the role study as compared to 41.30% in the study by Sartori of mul disciplinary approach in the management et al.13 Majority of pa ents were menstrual which is of vulval disorders as pa ents with same symptoms in agreement to the study from eastern Nepal but in may be diagnosed diff erently by dermatologists and contrast to the study from India wherein majority of gynecologists due to the diff erent training obtained by pa ents were post-menopausal.3,12 Irregular menses them.7 were observed in 12.00% of study popula on which is

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The most common non-venereal dermatosis of female and less psychological stress with mild symptoms were external genitalia was reported to be lichen sclerosus enumerated as possible factors in less number of (21.7%) by Singh et al from South India in 2008.12 This cases compared to western literature.3 Whether that fi nding is similar to the result in present study. Other is the case, or pa ents with vulvodynia are not seeking common dermatoses in the study by Singh et al. were care and suff ering in silence, or dermatologists in our vi ligo (15.8%), lichen simplex chronicus (13.3%), vulval setup are under-diagnosing the condi on needs to be candidiasis (9.2%), benign tumors and cysts (6.6%) and explored in future studies. lymphedema (5.8%).12 Of note, commonest diagnosis in interdisciplinary vulval clinics was also reported The study of vulval diseases has gained momentum to be lichen sclerosus in two diff erent studies.8,9 This in recent mes especially in western world.19 The is in contrast to another Indian study done in 2016 necessity and benefi t of interdisciplinary vulval clinic which reported nea cruris to be the commonest has been emphasized in various studies and is the diagnosis in sexually ac ve women.14 Similarly, the obvious way forward in op mal management of these study from eastern Nepal observed vulval dermatoses pa ents.7-10 The present study established the burden in 62.85% among which vulvovaginal candidiasis of non-venereal vulval diseases in Nepalese women in was the commonest condi on (11.4%).3 In the most Dermatology and Gynecology clinics of ter ary health recent study from Brazil, the commonest diagnosis center and forms a basis for planning and alloca on was condyloma acuminata (37.3%) followed by of resources in this neglected area. Mul center and lichen sclerosus (16.0%).13Among other factors, these community based studies including larger sample size, varia ons in the commonest vulval diseases could be studies regarding less reported condi ons like non- due to diff erent inclusion and exclusion criteria in the specifi c vulval pruritus and vulvodynia and studies present and the aforemen oned studies. concerning impact of vulval diseases in quality of life could be poten al areas to explore in future. Non-specifi c pruritus vulvae though commonly encountered in clinical prac ce has been poorly This study was a hospital based study, hence is not reported in literature. We observed non-specifi c completely representa ve of the situa on in the vulval pruritus in 9.33% of individuals, ranking fourth community. We did not study the risk factors of vulval amongst the common diagnoses. Pathak et al reported diseases and the study does not establish associa on 36.2% of pa ents to have pruritus vulvae, majority between cause and outcome. Limited sample size occurring in children and young women.3 Paek et al might have lead to exclusion of rare diseases in our similarly noted non-specifi c vulval pruritus in 75% of study popula on. pre-pubertal children evaluated for pruritus vulvae which was alleviated or cured by be er hygiene and Conclusion avoidance of irritants.17 In contrast, vulvodynia is extensively researched and frequently diagnosed This study outlined the clinico-epidemiologic profi le condi on with prevalence of 10-15% in hospital based of women presen ng with non-venereal vulval studies.9,18 However, study from eastern Nepal reported diseases. Pa ents mostly presented with itching and in vulvodynia in only 0.9% of pa ents with vulval diseases contrary to common belief that infec ons, par cularly and only 2.67% of pa ents in the present study were candidiasis is the commonest cause of consulta on, diagnosed with vulvodynia. Simpler standard of living infl ammatory condi on, specifi cally lichen sclerosus was the leading diagnosis in a ter ary referral center.

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