Vulvar dystrophies: A long-term Brisbane study of 155 cases

doi:10.4236/ojog.2012.23042

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Open Journal of Obstetrics and Gynecology, 2012, 2, 210-212 OJOG

http://dx.doi.org/10.4236/ojog.2012.23042 Published Online September 2012 (http://www.SciRP.org/journal/ojog/)

Vulvar dystrophies: A long-term Brisbane study of

155 cases*

Ian S. C. Jones1#, Alister Jones2

1Women’s and Newborn Services, Roya l B r i sba n e a n d W o men ’ s H o spi t a l , Brisbane and University of Queensland, Herston, Australia

2Department of Surgery, Gold Coast Hospital, Gold Coast, Australia

Email: #[email protected]

Received 2 May 2012; revised 8 June 2012; accepted 19 June 2012

ABSTRACT

Objective: To review the long-term outcomes for 155

women with a vulvar dystrophy (VD) who attended

the Royal Brisbane Hospital Vulvar Clinic between

1976 and 1988. Methods: VD data from Vulvar Dis-

eases Clinic were reviewed and analysed using the

computer software Statistical package for the Social

Sciences (SPSS) 11.0. Results: Of 155 patients 94 had

Lichen Sclerosus (LS), 41 Lichen Simplex Chronicus

(LSC) and 20 Mixed Dystrophy (MD). Three patients

developed squamous cell carcinomas of the vulva be-

tween 10 and 26 years after presentation with a VD.

To date only one of these three patients remains alive

following treatment. Conclusion: The need for long

term follow up is stressed and any of the three types

of VD may become malignant. Time from diagnosis to

malignant change is not predictive. VD treatments

seem to go through phases with the application of

potent steroid creams having stood the test of time.

Keywords: Vulvar Dystrophies; Features; Treatment;

Malignancy

1. INTRODUCTION

Lichen Sclerosus (LS) and Lichen Simplex Chronicus

(LSC) are two of the three most common non-neoplastic

epithelial disorders of the vulva (the third being lichen

planus) [1]. The International Society for the Study of

Vulvar Disease (ISSVD) proposed a new nomenclature

in 1976. This nomenclature was current when the study

cohort was managed and so is used in this article. Since

then there have been refinements to this nomenclature [2]

with LSC now termed Squamous Cell Hypertrophy and

MD termed Lichen Sclerosus with associated Squamous

Cell Hypertrophy. However, the histological criteria for

diagnosis are unchanged.

The macroscopic features of LS are white flat papules

which are scattered or confluent in plaques with atrophy

and pallor located on the labia minora, perineum and

clitoris. The histological features of LS include a thin,

flat epidermis and keratin layer, plus hyalinised dermis

bordered inferiorly by a band of chronic inflammatory

cells. Elastin fibres are reduced in areas of sclerosis. LSC

on the other hand appears as a generalised thickening of

the skin of the labia majora. Histologically the epidermis

and keratin layer are thickened and the rete pegs in-

creased in number and lengthened. The papillary dermis

is thickened and contains a variable chronic inflamma-

tory infiltrate. Mixed dystrophy (MD) combines features

of LS and LSC, both macroscopically and histologically

[3]. This paper reviews the features, treatment, and inci-

dence of malignant change for 155 women who pre-

sented with a vulvar dystrophy to the Vulvar Disease

Clinic at the Royal Brisbane Hospital between 1976 and

1988.

2. MATERIALS AND METHODS

One hundred and fifty five patients presenting to the

Vulvar Diseases Clinic between 1976 and 1988 were

diagnosed with VD. They were assessed and then seen

annually at either public or private gynaecology clinics.

Some patients chose to return to their referring private

gynaecologists, hence the need to check on follow up in

these cases. During 2010 the medical records from both

pu b lic and private gynaecology clinics were cro ss ch ec ke d

with the state wide Queensland Centre for Gynaecologi-

cal Cancer (QCGC) data base to determine if any VD

patient had developed a vulvar malignancy.

Data were stored and analysed using the computer

software Statistical Package for the Social Sciences

(SPSS) 11.0. Ethics approval for the study was obtained

from the Clinical Research Ethics Committee of the

Royal Brisbane and Women’s Hospital.

3. RESULTS

*Conflict of interest: There are no conflicts of interest.

#Corresponding author. Of 155 patients 94 had Lichen Sclerosus (LS), 41 Lichen

OPEN ACCESS

I. S. C. Jones, A. Jones / Open Journal of Obstetrics and Gynecology 2 (2012) 210-212 211

Simplex Chronicus (LSC) and 20 Mixed Dystrophy

(MD). All age groups were represented in the cohort with

the majority being aged 50 years or more. The age range

and mean age for the three conditions were for LS 11 -

88 years (mean 59), LSC 20 - 93 years (mean 59) and

MD 56 - 83 years (mean 65). Pruritus was the presentin g

symptom in 80% or more for each type of VD. The most

common anatomical location for LS was the labia minora,

for LSC the labia majora and for MD both sites. Using z

scores these findings were significant (p < 0.01). How-

ever it was not possible to equate diagnosis with ana-

tomical location in all cases.

During routine follow up three patients developed

squamous cell carcinomas of the vulva between 10 and

26 years after pr esentation with a VD ( Table 1). Of these

three patients one came from each of the three types of

VD. To date only one patient remains alive and she is

without evidence of recurrence of either her vulvar or

endometrial cancer. All three patients with vulvar cancer

underwent simple vulvectomy and groin node sampling.

In the early part of the study local steroid cream com-

bined with testosterone cream was the most frequently

used treatment for LS and steroid cream alone for LSC.

Later potent steroid creams became the sole treatment

regime for all VDs. Treatment was considered to be suc-

cessful if the patient was symptom free and the skin ap-

pearance returned to normal. However in 20% of LS

cases skin appearance improved but did not return to

normal despite regular use of treatment.

4. DISCUSSION

The prevalence of female LS is unknown, with estimates

ranging from 1:30 to 1:59 women in general gynaeco-

logical practice. The highest incidence of LS in women

was found post menopause. The differential diagnosis of

LS includes lichen planus, with which it may overlap,

and vitiligo. Th e most important risk for these patien ts is

the possibility (between 2% - 5%) of progressing to

squamous cell carcinoma [4]. Squamous cell carcinoma

of the vulva in younger women is frequently associated

with Human Papilloma Virus (HPV) infection and Vulvar

Intraepithelial Neoplasia (VIN). This is not usually the

case in the older woman who h as VIN but no HPV infec-

tion [5].

The aetiology of LS is unknown but causation th eories

abound starting with achlorhydria, the transplant study

which suggested local vulval factors facilitated disease

expression, the association with auto-immune disease

like autoimmune thyroid disorders, vitiligo and alopecia

areata, HLA 30/31 and HLA-B4 association, 5 alpha

reductase deficiency, acid fast bacterial infection and

increased collagen inhibitor enzyme. More recently there

is evidence that immunological changes are present in all

layers of skin affected by lichen sclerosus. In addition the

increased fragility and scarring in skin affected by LS

has been explained by the reorganisation of the extracel-

lular matrix due to changes in tenascin, fibrinogen (de-

crease) and fibronectin (increased) which are important

compone nt s as soc i at ed wi t h wound repair.

Lichen simplex chronicus (LSC), describes a non-

neoplastic morphological alteration of skin related to

chronic irritation with a characteristic histological ap-

pearance. LSC is nowadays considered to be a localised

form of neurodermatitis with underlying psychological

disturbance being present in most cases. The prevalence

of LSC is unknown but is estimated to be 1:75 to 1:100

women presenting to a general gynaecological practice

(Jones, unpublished work). LSC is a diagnosis of exclu-

sion from other conditions that can cause hyperplastic

epithelial changes (e.g. psoriasis, lichen planus, eczema,

seborrheic dermatosis, HPV infection and candidiasis).

There are other vulval conditions that can present with

Table 1. Cases that subsequently developed vulvar squamous cell carcinoma.

Condition Lichen sclerosus

n = 1 Lichen simplex chronicus

n = 1 Mixed dystrophy

n = 1

Age when ca found 1994 aged 78 1995 aged 64 1994 aged 55

Duration of V D 26+ years 10+ years 20 years

Location Labia minora Clitoris Labia minora

Parity 0 8 3

Menopause aged 35 Aged 50 Aged 48

Preceding VIN/atypia No Yes, 6 years Yes, 4 years

Current status

Alive

Dementia

No endometrial ca

No vulva ca

Dead of disease Dead of disease

I. S. C. Jones, A. Jones / Open Journal of Obstetrics and Gynecology 2 (2012) 210-212

212

pruritus vulvae for example candidiasis, lichen planus

and HPV infection. Most VDs occur in post menopausal

women but can occur in the child and during reproduc-

tive years. Even pregnant women with their high levels

of female hormones can present with VDs suggesting

that theories to explain the basis of these conditions can

not be solely due to a lack of female hormones.

The study found an association between autoimmune

thyroid disease and LS of 10%, which is in line with

other reports. There are reports of LS having a hereditary

basis with mothers and sisters of sufferers being reported

however this was not recognised in the current study. The

associations between VDs and diabetes, other skin dis-

orders (psoriasis, vitiligo and lichen planus), drug aller-

gies and non genital cancers did not reach statistical sig-

nificance. Depression requiring treatment was present in

14% of LS patients, 29% of LSC and 20% of MD. No

significant difference was found between these three

groups using z scores (LS v LSC z = 1.822; LS v MD z =

0.338; LSC v MD z = 0.44). The benefits of anti depres-

sants include their ability to modify pain.

Of available treatments, most were based on the use of

potent glucocorticoid steroid creams, although once sym-

ptoms settled the weekly or bi-weekly use of 1% hydro-

cortisone proved effective. The concern with long-term

use of steroid creams is the atrophying effect this has on

vulval skin, especially that affected by LS. Another pro-

blem with the use of steroid creams is the development

of fungal infection, which is frequently worse in the

presence of obesity and diabetes. Steroid creams can also

be used in conjunction with vaginal oestrogen cream

when vaginal atrophy causes coital difficulties. Steroid

cream was a popular and effective treatment for LSC,

findings which are in agreement with a series of 976 pa-

tients reported by Ayhan et al. [6].

Malignant change in VD patients occurs in between

3% - 5%. In the current study vulval malignancy was

found in three patients. None of the three types of VD

were spared from developing malignancy; hence all forms

of VD require long term follow up. All three cases of

malignancy were post menopausal and those with LS had

had their condition for over 20 years (Table 1). Time

since menopause for these three patients were 43 years

for LS, 14 years for LSC and 7 years for MD, however

the duration of VD was not useful in differentiating

malignancy risk but the presence of vulval intra epithe-

lial neoplasia (VIN) or cellular atypia was, supporting

the need for further biopsies if the skin appearance is

suspicious.

5. ACKNOWLEDGEMENTS

We thank the Queensland Centre for Gynaecological Cancer (QCGC)

staff for their assistance in providing data and checking their data on

our study patients.

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