Benign vulval pathology Flashcards
What is the definition of vulvodynia?
(Classification B)
Vulvar pain of at least 3 months duration, without clear identifiable cause, which may have potential associated factors - Localized (e.g. vestibulodynia, clitorodynia) or Generalized or Mixed (Localized and Generalized)” (3).
What are possible causes of vulval pain
(Classification A)
Infection
Inflammatory
Neoplastic
Neurological
Trauma
Iatrogenic
Hormonal deficiencies
Central sensitisation
What forms the basis of treatment of vulvodynia
4 Ps
Patient education and reassurance
- explain condition, give information. Skin care
Pain modification
- lignocaine ointment, Gabapentin cream, amitriptyline cream, avoid opioids, oral amitriptyline, gabapentin, pregabalin
Physical therapy
- physiotherapist
- consideration of vaginal Diazepam, Botox
Psychological and psychosexual therapy
- involve partner
- minimise stress
- CBT, relaxation techniques
- treat mental illness if present
What is localised provoked vestibulodynia?
Younger women 20-30
Superficial dyspareunia / inserting tampon / cycling
Pain may persist after SI
Pain free at other times
Develop hypertonicity in levator ani, secondary vaginismus
What is spontaneous generalised vulvodynia?
Involves large part of vulva
Usually postmenopausal
Chronic vulval discomfort
Symptoms worsen during the day
Intercourse doesn’t worsen symptoms
Lichen sclerosus epidemiology:
What is the peak age(s) of prevalence?
What is the prevalence of LS?
What is the associated incidence of vulvar SCC?
Peak:
- Affects prepubertal and post-menopausal women most frequently (oestrogen responsive)
- Greatest prevalence in women >80yrs
- Up to 3% - True prevalence unknown.
2-5% associated with vulvar SCC
Aetiology of lichen sclerosis
- Genetics
- 15% will have first degree relative with lichen sclerosus
- Autoimmune
- Associated with other autoimmune conditions (thyroid disease, pernicious anaemia, alopecia)
- Antibodies to extracellular matrix proteins 1 (ECM-1) have been found in 60-80% patients
- Trauma/irritation
- It may follow or coexist with another skin conditions
- Uncircumsized males more likely to suffer with it, thought secondary to chronic inflammation from urine caught under the foreskin
- Hormonal
- Most common presentation post menopause after reduction in oestrogen
What are the histopathological changes associated with lichen sclerosus?
Lichenoid lymphocytic inflammation
Hyperkeratosis and thinning of epidermis
Vacuolar degeneration of basal layer
Condensation of dermal collagen
plasma cells, histiocytes, mast cells
Describe the examination findings associated with lichen sclerosus
Vulval lichen sclerosus
- Usually affects non-hair bearing, inner areas of the vulval skin
- Can be localised, or extend to involve the clitoral hood, labia minora, perianal area and surrounding perineum, occasionally can extend over labia majora to inguinal folds
- NEVER involves vaginal mucosa
- Typical figure eight distribution
- White, papery thin and crinkled areas of skin
- Skin fissuring
- Adhesions
- Fusion of labia and loss of normal vulval architecture
- Introitus can become narrowed
- Associated intense irritation
- Fissuring can cause pain or bleeding on urination and dyspareunia
Extragenital
- Affects 10% women with vulval disease
- Plaques may be found on inner thigh, axillae, abdomen, lower back, under breasts, neck
What are two differential diagnoses for lichen sclerosus and what distinguishing features help you tell them apart?
Lichen planus:
- NB Can occur together
- LP involves the vagina
- Classical LP has violaceous hue and wickhams striae
- Hypertrophic LP can form thick warty plaques
- Erosive LP has raw eroded mucosal surface with grey/white epithelial edges & Wickham’s striae
Lichen simplex chronicus:
- Hyperkeratotic with lichenification
- Well-demarcated, markedly thickened plaques with a leathery appearance, often unilateral, can be bilateral
- Usually diagnosis of exclusion.
Outline management of lichen sclerosus
First-line = ultra potent topical steroid
- Ultrapotent topical steroid (e.g. dermal - clobetasol propionate 0.05% ointment)
- application to ONLY affected area
- Course varies depending on severity of disease and symptom response
- Reasonable to start with daily application for 1-3 months, then reduce
- Most women will need to continue use once/twice weekly in the long term to keep symptoms in remission and prevent disease progression
- Schedule review after few weeks to ensure good response and no adverse reaction to steroids
Second line topical agents and adjuncts
- Topical oestrogen cream
- Calcineurin inhibitors (tacrolimus)
- Topical retinoid (e.g. tretinoin cream) - often poorly tolerated on vulval skin, causes burning
Oral agents (reserved for severe acute disease flare, not responsive to topical agents)
- Oral steroids
- Oral retinoids
- Methotrexate
- Ciclosporin
Vaginal dilators
- For the management of stenosed introitus in the sexually active
Surgery
- Very rarely indicated - only benefit is to release marked adhesions particularly if causing urethral or introitus stenosis
- Required if progressions to invasive SCC
Long term follow-up
- Once symptom management achieved will need annual screening for progression to SCC - can alternate between colposcopy clinic and primary care
What is the prognosis for LS?
- Chronic disease - often follows a waxing-waning course
- Skin changes may be reversible with treatment, but most adhesions and architectural change are permanent and irreversible
- 5% risk of progression to SCC - the risk is higher if disease progression is not controlled
- Associations with other autoimmune conditions
What is the pathogenesis of lichen planus?
T-cell mediated autoimmune response against basal keratinocytes
What is the histopathology of lichen planus?
- Apoptotic keratinocytes in epidermis
- Upper dermis lymphocytic infiltration
- Basal cell liquefaction
- Increased granular layer
- Irregular sawtoothed acanthosis
What symptoms are associated with lichen planus?
- Itch
- Soreness
- Dyspareunia
- Urinary sx
- Vaginal discharge
- Sometimes postcoital bleeding
What examination findings are associated with lichen planus?
Classical:
- Papules on keratinised anogenital skin with or without striae on the inner aspect of vulva; hyperpigmentation often follows resolution.
Hypertrophic:
- Rare, mimics malignancy
- Thickened warty plaques which may become ulcerated, infect and painful; no vaginal lesions.
Erosive: most common type on vulva
- Mucosal erosions with Wickham’s striae
- Vaginal lesions can lead to scarring and complete stenosis’ friable telangiectasia and patchy erythema
