Definition:
Generally benign keratotic white lesions on the vulva are called as vulval leukoplakia or precancerous disease or white spot disease.
In these more are benign or pre-cancerous lesions. Only 4 to 6% can be cancerous.
Nowadays instead of using the general term leukoplakia, one standard nomenclature has developed based on the histopathological findings of the vulval lesions.
And these lesions are called non-neoplastic epithelial disorders in general. And these are subdivided into:
-Squamous cell hyperplasia.
-Lichen sclerosus.
-And other dermatoses.
Causes of leukoplakia:
Exact etiology is not clear. But the possible factors are:
-Systemic factors like:
Diabetes
Endocrine disorders
Malnutrition
Vitamin deficiency
Pituitary-ovarian dysfunction
-EB virus infection
-Candida albicans infection
-HPV infection seen in around 22% of the patients.
-Can be seen in patients with oral leukoplakia.
-P 53 gene mutation leading to cell proliferation.
-Local factors like:
Genital partial wetness
Heat stimulation
Rubbing
Age related atrophy
-Previous similar lesions
-The skin graft to vulva can also get the lesions.
Pathogenesis:
It is a mucosal or epidermal epithelial proliferative lesion.
In leukoplakia mucosal epithelium becomes keratinized ranging from granular layer thickness.
Histopathological features:
-Significant hyperkeratosis
-Granular layer thickening
-Mucosal or skin epithelial hyperplasia
-Acanthosis thickening
-Epithelial crest
-Infiltration of dermal lymphocytes and plasma cells
-Appearance of prickle cell layer
-Cells with irregular shape and mitotic figures
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Symptoms and signs:
-Patient will usually present with irregular milky white shiny patches or plaques with slightly elevated mucosal surface, well defined borders and state clearly.
These lesions can be seen on genital organs like vaginal mucosa, urethral mucosa, clitoris, labia majora, labia minora etc.
Itching is one more common feature.
Itching can lead scratching, rubbing which can in turn lead to flushing, edema, erosion, ulcers or liquenification.
With progression of time some lesions can become:
-Uplifted, state unclearly
-Surface becomes more keratinized, rough and hard to touch.
-Bleeding from the erosion or ulcers can be seen.
Diagnosis:
Histopathological examination and clinical features collectively help in diagnosis.
Prevention:
-Pertinacious diet containing vitamins, minerals etc. should include milk, eggs etc.
-Treating provoking factors like diabetes, endocrine diseases etc.
-Treating EB virus, candidial infections etc.
-Maintaining vulval local health like reducing the moisture, friction, heat production etc.
Treatment:
-With appropriate and early intervention around 96 to 97% cases can be prevented from becoming worse.
-Local treatment – to keep the vulva clean and dry.
-Using mild soaps and avoiding soap over scrub etc.
-For itching local corticosteroids can be used.
-For keratosis proliferative lesions topical 0.025% to 0.05% Vitamin-A acid ointment or 2.5% fluorouracil ointment can be used.
(Fluorouracil helps in blocking the conversion of uracil into thymidine. Thereby it inhibits DNA synthesis, which prevents tumor cell proliferation and differentiation. It can be used in precancerous skin lesions and also in malignant skin lesions.)
-Leukoplakia with mild dysplasia can be treated with this combination therapy and long term follow-up.
-In case of leukoplakia lesions with atypical cellular features with possibility of development of carcinoma in situ needs surgery.
Differential diagnosis of leukoplakia :
Keratosis:
-White skin cells due to hypopigmented keratosis with out heteromorphism.
-Appear as ill-defined white spots with no or mild infiltration.
-If the keratosis prolongs for long term it can turn into leukoplakia etc lesions.
Oral lichen planus in combination with genital lichen planus
-Can appear on genital skin or at mucocutaneous junction.
-Usually appears as polygonal flat purple or dark red papules with glossy surface.
-HPE: colloid bodies, basal liquefaction, degeneration, dermal infiltration of lymphocytes etc.
Atrophic lichen sclerosis:
-Often occurs on labia.
-It is atrophic vulvar dystrophy.
-Appear as blue and white papules, integrated pale hypo pigmented spots can be seen.
-Can involve anus and vagina to form a dumble – shaped lesions.
-Itching will be minimal usually.
Vulval vitiligo:
-Depigmented spots with clear boundaries can be seen.
-No keratosis or infiltration or itching.
-Same type of lesions can be on other parts of the body.
-HPE: complete lack of dopamine stained melanocytes in the basal layer.
-Easy to differentiate with leukoplakia.
Vulval neurodermatitis:
-Usually distributed on both sides of the outer labia.
-Conscious itching sensation will be present.
-Skin lesions can develop due to continuous scratching.
-HPE: changes of chronic dermatitis, thickening of skin layers can be seen. There will be no spindle shaped cells which will help in differentiating with leukoplakia.
