|Year : 2007 | Volume
| Issue : 2 | Page : 87-90
A clinical study of vulval lichen sclerosus at a tertiary care hospital in South India
Nidhi Singh1, Devinder Mohan Thappa1, Telanseri J Jaisankar1, Syed Habeebullah2
1 Department of Dermatology and STD, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, India
2 Department of Obstetrics and Gynaecology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, India
Devinder Mohan Thappa
Department of Dermatology and STD, JIPMER, Pondicherry - 605 006
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Lichen sclerosus (LS) is a chronic inflammatory dermatosis that causes substantial discomfort and morbidity, most commonly in adult women. The objective of our study is to study the pattern of vulval LS and to correlate LS with various clinical parameters. The study included 26 female patients with vulval LS presenting over a period of 22 months, starting from September 2005 to June 2007. Demographic characteristics and clinical findings were recorded. The mean age of LS patients was 44 years (range 3-65 years). Lichen sclerosus was most commonly observed in postmenopausal women (18, 69.2%), followed by women in reproductive age group (5, 19.23%), and prepubertal girls (3, 11.5%). All patients presented with ivory white atrophic plaque. Surface of the plaque showed telangiectasia in one patient; both erosions and fissuring in two patients, erosions in four patients, fissuring in two patients, and wrinkling in all patients. Introitus was stenosed in five (19.2%) patients, out of which three (11.5%) patients also had perianal involvement leading to "figure of 8" appearance. Three patients had atrophy of labia minora and clitoris to an extent that labia minora appeared merged with labia majora and clitoris was buried. This study highlights the importance of diagnosing LS as it is associated with considerable morbidity.
Keywords: Lichen sclerosus, South India, vulva
|How to cite this article:|
Singh N, Thappa DM, Jaisankar TJ, Habeebullah S. A clinical study of vulval lichen sclerosus at a tertiary care hospital in South India. Indian J Sex Transm Dis 2007;28:87-90
|How to cite this URL:|
Singh N, Thappa DM, Jaisankar TJ, Habeebullah S. A clinical study of vulval lichen sclerosus at a tertiary care hospital in South India. Indian J Sex Transm Dis [serial online] 2007 [cited 2020 Feb 26];28:87-90. Available from: http://www.ijstd.org/text.asp?2007/28/2/87/39011
| Introduction|| |
Vulval lichen sclerosus (LS) is a chronic inflammatory dermatosis that causes substantial discomfort (intractable itching, soreness, constipation, and dyspareunia) and morbidity (introital narrowing, burying of clitoris, and atrophy labia minora), most commonly in adult women. In children, LS may be confused with sexual abuse.  In 1887, Hallopeau first described LS and used the term "lichen plan atrophique" for it. It was followed by the histological description by Darier who termed it "lichen plan sclιreux". Over the years numerous terms, such as kraurosis vulvae, hypoplastic dystrophy, weissflecken dermatose, and lichen sclιreux et atrophique, have been used to describe this condition. Now, it has been accepted that the condition is entirely distinct from lichen planus and the term LS should be used as advocated by International Society for the Study of Vulvovaginal Disease (ISSVD). 
Though literature is saturated with case reports of LS, no formal published study is present from India, although there are studies on LS from the other parts of the world. Hence, we undertook this study to find the pattern of vulval LS at a tertiary care center in South India and to correlate LS with various clinical parameters.
| Materials and Methods|| |
The study was a descriptive study, spanned over a period of 22 months, from September 2005 to June 2007. All female patients attending Skin out patient department (OPD) and Obstetrics and Gynecology OPD at our institute, were screened for vulval LS. Institute Ethics Committee clearance was obtained. All consenting female patients irrespective of their age and pregnancy status who presented with genital complaints were screened for vulval LS. Informed consent was taken. Detailed history including demographic data, chief complaints related to skin, presence of itching, skin lesions, onset, pregnancy status, menstrual status, associated medical or skin disorders, etc. was elicited and recorded on a proforma.
The external genitalia were examined and findings were noted. Detailed physical examination was made to see any associated lesions elsewhere in the body. Biopsy and histopathological examination of the specimen was done when required to confirm the diagnosis. Results were tabulated and analyzed using SPSS 13.0 software.
| Results|| |
Lichen sclerosus was recorded in 26 cases during the study period. The age of LS patients ranged from 3 to 65 years with a mean age of 44 years. Most cases belonged to 51-60 years age group (11 patients) followed by 6 patients in 41-50 age group [Table - 1]. As suggested by the age group distribution of cases, it was found that 69.2% (18 cases) women were postmenopausal, followed by 19.2% (five cases) in reproductive age group, and 11.5% (three cases) were girls who had not attained menarche. Most of the patients were from rural areas accounting for 80.8% cases.
The most common presenting complaint was itching in 92.3% patients. Fifteen (57.7%) patients complained of the color change in genitalia to white and 13 of these had associated itching. Dyspareunia was complained by two (7.7%) patients. One 9-year-old patient had constipation and burning sensation during micturition. Duration of complaints ranged from 2 weeks to 40 years, with a median value of 2.5 years [Table - 2]. Labia majora along with labia minora and clitoris was the most commonly involved site accounting for 57.7% (15) cases [Table - 3].
All patients presented with ivory white atrophic plaque [Figure - 1]. Surface of the plaque showed telangiectasia in one patient; both erosions and fissuring in two patients, erosions in four patients, fissuring in two patients, and wrinkling in all patients [Figure - 2]. Introitus was stenosed in five (19.2%) patients, out of which three (11.5%) patients also had perianal involvement leading to "figure of 8" appearance. Three patients had atrophy of labia minora and clitoris to an extent that labia minora appeared merged with labia majora and clitoris was buried. Associated conditions with LS are given in [Table - 4]. None of the cases had any family history of LS.
| Discussion|| |
Lichen sclerosus is the chronic inflammatory dermatoses associated with substantial discomfort and morbidity.  Anogenital LS is characterized by porcelain white atrophic plaques, which may become confluent extending around vulval and perianal skin in a figure of eight configuration.  Atrophic plaque may have a cellophane paper like texture, wrinkled, and fragile surface associated with telangiectasia, purpura, erosions, fissuring, or ulceration. , Clinical findings in cases of LS in this study were found to be in concordance with the literature review. Atrophy can lead to loss of labia minora, burying of the clitoris, obstruction of urinary outflow or other architectural changes.  Architectural changes like atrophy of labia minora and clitoris were observed in our study. The lesions occur on the inner aspects of labia majora, labia minora, and clitoris.  Perianal lesions occur in 30% of cases.  This is consistent with our study where we found 30.8% patients having perianal lesions. Genital mucosal involvement does not occur; vagina and cervix are always spared. However, some mucosal involvement at the edge of mucocutaneous junctions may lead to introital narrowing.  Introital narrowing was present in 19.2% patients of LS in our study. Involvement of labial, perineal, and perianal areas along with introital narrowing is referred to as "Keyhole" or "hourglass" or "figure of 8". , " Figure of 8" appearance was present in three of our patients.
Prevalence of LS is estimated to range from 1 in 300 to 1 in 1000 of all patients referred to dermatology departments.  The prevalence of childhood vulval LS in a UK local health authority was determined to be 1 in 900.  The onset of LS has been reported at all ages although it is not common in <2 years of age.  Lichen sclerosus has two peak ages of presentation - prepubertal girls and postmenopausal women (mean age of onset is fifth or sixth decades).  The majority of our cases were postmenopausal and very few cases were prepubertal as recorded in the literature. In our study, the mean age of patients was 44 years (range 3-65 years); the median duration of symptoms of LS was 2.5 years (range 2 weeks-40 years); and the mean age at onset of symptoms of LS was 39.15 years (range 3-59.8 years). A study of 350 cases of women with LS by Thomas et al. ,  showed that the mean age of patients was 56 years (range 4-91 years); the mean duration of symptoms of LS was 10.5 years (range 1-65 years); and the mean age at onset of symptoms of LS was 45.5 years (range 1-90 years). 
The exact etiology is unknown.  Autoimmune, genetic, infective, hormonal, and local factors have been implicated. A strong association with autoimmune disorders (21.5-34% patients have been reported to have autoimmune disease and up to 74% patients found to have autoantibodies) has been reported. , The common disorders associated with LS are alopecia areata, vitiligo, thyroid disease, and pernicious anemia.  Familial cases have also been reported and a significant association with HLA class II antigen DQ7 has been demonstrated.  Infective etiology like spirochaete Borrelia has been implicated in pathogenesis of LS.  However; studies have shown conflicting results regarding infectious etiology. , Local factors like friction, trauma, or rubbing may cause Koebner phenomenon triggering LS.  Higher incidences of LS in postmenopausal women suggest a hormonal influence, but a protective effect from estrogen has not been shown. , Androgen sensitive fibroblasts in vulval skin are responsible for sclerosis. At menarche, there is increased metabolism of testosterone in genital skin which may be responsible for the spontaneous improvement in childhood vulval LS.  Oxidative stress may also be responsible for sclerosis, autoimmunity, and carcinogenesis in case of LS. 
Lichen sclerosus most commonly affects anogenital region (85-98%). Extragenital LS can be seen in 15-20% cases.  Women complain of intractable pruritus (worse at night), , irritation, soreness, dyspareunia, dysuria  and urinary, and/or faecal incontinence. , Nine percent of cases may be asymptomatic.  Prepubertal girls usually complain of itching and soreness as in adults, but they can also have dysuria, constipation,  pain on defecation, soiling, fissuring, and bleeding. ,, Our observation was in concordance with the literature. In prepubertal girls signs of LS may be confused with sexual abuse.  Extragenital lesions may occur in 10% of cases. Common extragenital sites are trunk, sites of pressure, upper back, wrists, buttocks, and thighs.  There is 4-5% risk of squamous cell carcinoma in vulval LS. ,
In conclusion, this study highlights that LS is not uncommon in India and generally present as an itchy vulvar dermatosis which a gynaecologist may mistake for candidal vulvovaginitis.
| References|| |
|1.||Powell JJ, Wojnarowska F. Lichen sclerosus. Lancet 1999;353:1777-83. [PUBMED] [FULLTEXT]|
|2.||Funaro D. Lichen sclerosus: A review and practical approach. Dermatol Ther 2004;17:28-37. [PUBMED] [FULLTEXT]|
|3.||Bunker CB, Neill SM. The genital, perianal and umbilical regions. In : Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's textbook of dermatology. 7 th ed. Blackwell Science: Oxford; 2004. p. 68.1-68.104. |
|4.||Khaitan BK. Nonvenreal diseases of genitalia. In : Sharma VK, editor. Sexually Transmitted Diseases and AIDS. 1 st ed. Viva books Pvt Ltd: New Delhi; 2003. p. 413-21. |
|5.||Neill SM, Tatnall FM, Cox NH; British Association of Dermatologists. Guidelines for the management of lichen sclerosus. Br J Dermatol 2002;147:640-9. [PUBMED] [FULLTEXT]|
|6.||Meffert JJ, Davis BM, Grimwood RE. Lichen sclerosus. J Am Acad Dermatol 1995;32:393-416. [PUBMED] [FULLTEXT]|
|7.||Wallace HJ. Lichen sclerosus et atrophicus. Trans St Johns Hosp Dermatol Soc 1971;57:9-30. [PUBMED] |
|8.||Powell J, Wojnarowska F. Childhood vulvar lichen sclerosus: An increasingly common problem. J Am Acad Dermatol 2001;44:803-6. [PUBMED] [FULLTEXT]|
|9.||Meyrick Thomas RH, Ridley CM, McGibbon DH, Black MM. Lichen Sclerosus et atrophicus and autoimmunity: A study of 350 women. Br J Dermatol 1988;118:41-6. [PUBMED] |
|10.||Tasker GL, Wojnarowska F. Lichen sclerosus. Clin Exp Dermatol 2003;28:128-33. [PUBMED] [FULLTEXT]|
|11.||Powell J, Wojnarowska F, Winsey S, Marren P, Welsh K. Lichen sclerosus premenarche: Autoimmunity and immunogenetics. Br J Dermatol 2000;142:481-4. [PUBMED] [FULLTEXT]|
|12.||Breier E, Khankah G, Stanek G, Kunz G, Aberer E, Schmidt B, et al . Isolation and polymerase chain reaction typing of Borrelia afzelii from a skin lesion in a seronegative patient with generalized ulcerating bullous lichen sclerosus et atrophicus. Br J Dermatol 2001;144:387-92. |
|13.||Todd P, Halpern S, Kirby J, Pembroke A. Lichen sclerosus and the Koebner phenomenon. Clin Exp Dermatol 1994;19:262-3. [PUBMED] |
|14.||Sander CS, Ali I, Dean D, Thiele JJ, Wojnarowska F. Oxidative stress is implicated in the pathogenesis of lichen sclerosus. Br J Dermatol 2004;151:627-35. [PUBMED] [FULLTEXT]|
|15.||Owen CM, Yell JA. Genital lichen sclerosus associated with Incontinence. J Obstet Gynaecol 2002;22:209-10. [PUBMED] [FULLTEXT]|
|16.||Maronn ML, Esterly NB. Constipation as a feature of anogenital lichen sclerosus in children. Pediatrics 2005;115:e230-2. [PUBMED] [FULLTEXT]|
|17.||Fischer GO. Lichen sclerosus in childhood. Australas J Dermatol 1995;36:166-7. [PUBMED] |
|18.||McLelland J. Lichen sclerosus in children. J Obstet Gynaecol 2004;24:733-5. [PUBMED] [FULLTEXT]|
|19.||Al-Khenaizan S, Almuneef M, Kentab O. Lichen sclerosus mistaken for child sexual abuse. Int J Dermatol 2005;44:317-20. [PUBMED] [FULLTEXT]|
[Figure - 1], [Figure - 2]
[Table - 1], [Table - 2], [Table - 3], [Table - 4]