Open Journal of Obstetrics and Gynecology, 2012, 2, 210-212 OJOG Published Online September 2012 (
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
Received 2 May 2012; revised 8 June 2012; accepted 19 June 2012
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;
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
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.
*Conflict of interest: There are no conflicts of interest.
#Corresponding author. Of 155 patients 94 had Lichen Sclerosus (LS), 41 Lichen
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.
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
No endometrial ca
No vulva ca
Dead of disease Dead of disease
Copyright © 2012 SciRes. OPEN ACCESS
I. S. C. Jones, A. Jones / Open Journal of Obstetrics and Gynecology 2 (2012) 210-212
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
We thank the Queensland Centre for Gynaecological Cancer (QCGC)
staff for their assistance in providing data and checking their data on
our study patients.
[1] O’Connell, T.X., Nathan, L.S., Satmary, W.A. and Gold-
stein, A.T. (2008) Non-neoplastic epithelial disorders of
the vulva. American Family Physician, 77, 321-326.
[2] Maclean, A.B. (1991) Vulval dystrophy—The passing of
a term. Current Obstetrics & Gynaecology, 1, 97-102.
[3] Neill, S. and Lewis, F. (2009) Ridley’s the vulva. 3rd
Edition, Wi ley- Blackwe ll, Hoboken.
[4] Smith, Y.R. and Haefner, H.K. (2004) Vulvar lichen scle-
rosus: Pathophysiology and treatment. American Journal
of Clinical Dermatology, 5, 105-125.
[5] Scurry, J.P. and Vanin, K. (1997) Vulvar squamous cell
carcinoma and lichen sclerosus. The Australasian Journal
of Dermatology, 38, S20-S25.
[6] Ayhan, A., Guvendag Guven, E.S., Guven, S. , Sakinci, M.
and Kucukali, T. (2006) Medical treatment of vulvar
squamous cell hyperplasia. International Journal of Gy-
naecology & Obstetrics, 95, 278-283.
Copyright © 2012 SciRes. OPEN ACCESS