Benign vulval disorders
Leonardo Micheletti
Department of Gynaecology and Obstetrics
University of
On behalf of
Rolf Kirschner
Secretary General EBCOG
Oslo University Hospital, Oslo, Norway
Declaration: Conflict of interest
Professor Rolf Kirschner
has given presentations on a large number of
gynaecological topics, including contraception, by
invitation, from a number of pharmacological firms during
his career.
Professor Leonardo Micheletti
No disclosures or conflicts of interest
Benign vulval disorders
 Infections
 Skin diseases
 Tumors
 Painful conditions
 ... Sexual dysfunction – primary or
secundary
PRINCIPAL CONDITIONS AFFECTING THE VULVA
•Benign neoplasia
•Infections (20-30%)
• viral
• mycotic
• bacterial
•Dermatosis (10-60%)
● lichen sclerosus
● lichen planus
● lichen simplex chronicus
● psoriasys
● contact irritant dermatitis
● contact allergic dermatitis
● atopic dermatitis
● …………
•Malignant neoplasia (5-10%)
• VIN
• Paget disease
• invasive carcinoma
• melanoma
• sarcoma
● Vulvodynia
• Psychiatric
disorders
• Neurological disorders
• Psychosexual disorders
LICHEN SCLEROSUS
• Autoimmune disease affecting only the vulval skin,
not the vestibular and vaginal mucosa.
• Localized only on glabrous (devoid of hairs) vulval skin,
very rare in extra-genital skin.
• Symmetrical- 8 shape with whitish shiny skin.
• Loss of normal architecture, labia minora disappearance
with adhesions and fissures.
• Excoriations and ecchymoses.
TOO OFTEN MISDIAGNOSED
Interpreted as "vulval dystrophy" or "craurosis"
Mismanaged : topical estrogen/testosterone or surgery
TREATMENT of LICHEN SCLEROSUS
Topical Potent Steroids (clobetasol, mometason)
one daily application x 4 weeks, then on alternate days x 4 weeks ,
ad infinitum 2 x week.)
DO NOT FORGET
EMOLLIENTS
reduce itch and make skin feel more comfortable
OIL CLEANSIG
avoid soap and water
After 3 months
TOPICAL TREATMENT FAILURE
• systemic steroids
• topical tacrolimus or pimecrolimus
beware of side effects!
Surgery only for adhesions
Lichen sclerosusCO2-laser treatment for adhesions
Vulval Lichen Sclerosus is a Benign Dermatosis
but has the Potential for Malignant Transformation
SCC
dVIN
VLS
Neoplastic Progression Risk from VLS
in Longitudinal Studies
After the 1975 ISSVD Classification of Vulvar Dystrophies
n VLS
n Neoplasms
VIN
SCC
Incidence risk (%)
Total
Hart et al, 1975
92
1
1
1.1%
Rodke et al, 1988
42
3
3
7.1%
Carli et al, 1995
211
3
3
1.4%
Carlson et al, 1998
32
7
10
31.3%
Van der Avoort et al, 2010
84
1
1
1.2%
Total
461
15
18
3.9%
3
3
• High variability in results
• Highest incidence risk in the smallest study
J Lower Gen Tract Dis 2016; 20: 180-183
Objectives of the Study
To assess malignant transformation risk
in a large cohort of VLS women
undergoing a long term follow-up
Results
●
Neoplasia incidence risk: 3.5%
• Kaplan-Meier probability of neoplasia occurrence
Months of
follow-up
Probability of
neoplasia
occurrence
Standard
Error
24
1.2%
0.01
60
3%
0.01
120
7.1%
0.02
180
11.0%
0.03
240
21.6%
0.05
300
36.8%
0.09
KEY MESSAGES
• VLS is a non-neoplastic disorder, but with a
potential risk for malignant transformation
• The probability of malignant
increases with follow-up length
transformation
• The higher probability of malignant transformation
in the long period suggests a careful and lifelong
follow-up in all VLS patients
VULVAL LICHEN PLANUS
• Autoimmune disease affecting both the vulval
skin, the vestibular and vaginal mucosa.
• It starts in the mature age group, with erosions causing
burning pain, bleeding, and dyspareunia.
• If not early diagnosed can cause adhesions and stenosis of
the vagina.
• Gynaecologists and dermatologists must team up to keep
the vagina open.
VULVAL LICHEN PLANUS
• The mucosa of the oral cavity is also frequently
affected.
REMEMBER
when suspecting vulval lichen planus
Look at the Oral Cavity
TREATMENT of LICHEN PLANUS
same as for lichen sclerosus
MORE DIFFICULT
Vulval and Vaginal Topical Potent Steroids (clobetasol, mometason)
one daily application x 4 weeks, then on alternate days x 4 weeks ,
ad infinitum 2 x week.)
Topical Tacrolimus or Pimecrolimus
beware of side effects!
Systemic Steroids
Other Systemic Treatment : thalidomide, dapsone, azathioprine,
cyclosporin, methotrexate
DO NOT FORGET
EMOLLIENTS and OIL CLEANSING avoid soap and water
Psychological Support, Psychotropic Drugs
Surgery only for adhesions
Vulvodynia –
Vulvar pain of at least 3 months’ duration
without clear identifiable cause
which may have potentially associated factors
 Localized (e.g. vestibulodynia, clitorodynia)
 Generalized or Mixed (localized and generalized)
 Provoked (e.g. insertional, contact)
 Spontaneous or Mixed (provoked and spontaneous)
 Onset (primary or secondary)
 Temporal pattern (intermittent, persistent, constant,
immediate, delayed)
Provoked Vestibulodynia: inflammatory, neuropathic or dysfunctional
pain? A neurobiological perspective.
L. Micheletti, G. Radici, P.J. Lynch
Journal of Obstetrics and Gynaecology. 2014; 34: 285-8
a) There is a lack of biochemical demonstration of active inflammation
b) There is no significant difference in neuropeptide staining between PV
patients and control
c) Anti inflammatory drugs are ineffective
pain occuring in PV
Not Inflammatory
The vestibular redness is a normal finding
Provoked Vestibulodynia: inflammatory, neuropathic or dysfunctional
pain? A neurobiological perspective.
L. Micheletti, G. Radici, P.J. Lynch
Journal of Obstetrics and Gynaecology. 2014; 34: 285-8
a) The absence of a specific neurologic disorder (herpes neuralgia,
spinal nerve compression, etc.)
b) The absence of low IENF (intraepidermal nerve fibres) density
(diagnostic criterion of small fibre neuropathy )
c) The recognition that QST (quantitative sensory testing) and
fMRI (functional Magnetic Resonance Imaging) abnormalities
are not specific for neuropathic pain
pain occuring in PV
Not Neuropathic
reduced IENF density
is associated with the risk of developing neuropathic pain
Provoked Vestibulodynia: inflammatory, neuropathic or dysfunctional
pain? A neurobiological perspective.
L. Micheletti, G. Radici, P.J. Lynch
Journal of Obstetrics and Gynaecology. 2014; 34: 285-8
if pain in Provoked Vestibulodynia is not inflammatory or neuropathic,
has to be dysfunctional
What is this thing called dysfunctional pain?
to answer this question
I will rapidly address the recent widely accepted
neurobiological classification of pain
Nociceptive
PAIN
Inflammatory
Neuropathic
Pathological
Dysfunctional
Woolf C.J., 2010
NOCICEPTIVE PAIN
Associated with the detection of NOXIOUS STIMULI:
intense mechanical, thermal or chemical stimuli that are
damaging or threaten damage to normal tissues
High-threshold pain
Early-warning physiological
protective system
WITHDRAWAL REFLEX
INFLAMMATORY PAIN
Pain associated with peripheral tissue damage and activation of
immune cells recruited into the vicinity of the nociceptors
Inflammation
Macrophage
Mast cell
Neutrophil
Low-threshold pain
Tissue damage
protective, adaptive
SENSITIZATION
Pain hypersensitivity discourages
physical contact and movement,
which, in turn promotes healing
of damaged tissue
ALLODYNIA: pain due to a stimulus that does not normally provoke pain
HYPERALGESIA: exaggerated and prolonged pain in response to a painful stimulus
SPONTANEOUS PAIN: in the absence of any stimulus
PATHOLOGIC PAIN
Peripheral nerve
damage
Neuropathic pain
Neural lesion
macro- or microscopically
identifiable structural damage
Central neural
damage
Low-threshold pain
SENSITIZATION
Dysfunctional pain
Abnormal neural function
No peripheral tissue inflammation
No neural lesion
Non-protective, maladaptive
Pathologic Pain represents a Disease State of the Nervous System
which arises either by way of structural damage (neuropathic pain) or
by abnormal function (dysfunctional pain).
DYSFUNCTIONAL PAIN
CENTRAL PAIN PREDISPOSING FACTORS
Female sex, genetics, early life trauma, family history of chronic pain and mood
disturbances, personal history of multifocal pain or other central mediated
symptoms, such as insomnia, fatigue, memory difficulties and psychological
problems, such as anxiety, depression and catastrophizing
DYSFUNCTION
of DESCENDING PAIN
INHIBITORY CONTROL SYSTEMS
Precipitating events
Peripheral tissue inflammation
Abnormal
central
sensitization
Suffering from vulvodynia increases by 2 times the probability of having an interstitial
cystitis, and vice versa
Suffering from fibromyalgia increases by 5 times the probability of having an interstitial
cystitis, and vice versa
Suffering from irritable bowel syndrome increases by 6 times the probability of having
an interstitial cystitis, and vice versa
CHRONIC COMORBID PAIN CONDITIONS:
NOT ONLY CASUAL EPIDEMIOLOGICAL ASSOCIATION
IC/PBS
VD
CENTRAL
DYSFUNCTIONAL
PAIN
IBS
FM
DIFFERENT CLINICAL EXPRESSION MODALITIES
of the SAME NATURE of PAIN
PREDISPOSITION TO CENTRAL PAIN
VULVODYNIA
Anxiety and depression increase to 4 times the risk of vulvodynia; in turn
vulvodynia doubles the risk of anxiety and depression
(Khandker M, 2011)
IC/BPS
At least half of the patients are suffering from depression, a quarter from
panic attack
(Clemens JQ, 2012)
IBS
At least half of the patients are suffering from depression, anxiety or
hypochondria
(Spiller R, 2007)
FM
Anxiety and depression are diagnostic standards for fibromyalgia
(2013 AltCr)
Vulvodynia/ Provoked Vestibulodynia
Management
Management of women suffering from Vulvodynia must be multidisciplinary and,
because dysfunctional pain syndromes result mainly from abnormal central
sensitization, the target for treatment must be the central nervous system and not only
the periphery.
Is frequently associated with other chronic comorbid pain conditions such as BPS,
IBS, FM, CFS, and TMD, individually and or in combination.
The presence of provoked vestibulodynia or any of these comorbid pain conditions
increases the likelihood that a woman will have one or more of the other chronic
pain conditions.
THANK YOU
FOR YOUR ATTENTION