GYN 18

• Vulvar epithelial disorders


  Nonneoplastic epithelial disorders of vulvar skin and mucosa
• Vulvar intraepithelial neoplasia (VIN) lesions have been excluded from the classification
• ISSVD classification of vulvar dermatoses

  • Spongiotic pattern
  • Acanthotic pattern
  • Lichenoid pattern
  • Dermal homogenization/sclerosis pattern
  • Vesiculobullous pattern
  • Acantholytic pattern
  • Granulomatous pattern
  • Vasculopathic pattern
• Spongiotic pattern

  • Atopic dermatitis
  • Allergic contact dermatitis
  • Irritant contact dermatitis
• Acanthotic pattern


  • Psoriasis
  • Lichen simplex chronicus
  • Primary (idiopathic)
  • Secondary (superimposed on lichen sclerosus, lichen planus, etc.)
• Lichenoid pattern


  • Lichen sclerosus
  • Lichen planus
• Dermal homogenization/sclerosis pattern


  • Lichen sclerosus
• Vesiculobullous pattern

  • Pemphigoid, cicatricial type
  • Liner IgA disease
• Acantholytic pattern


  • Hailey-Hailey disease
  • Darier disease
  • Papular genitocrural acantholysis
• Granulomatous pattern


  • Crohn disease
  • Melkersson-Rosenthal syndrome
• Vasculopathic pattern


  • Aphthous ulcers
  • Behcet disease
  • Plasma cell vulvitis
• Factors implicated in etiology

  • Trauma (scratching)
  • Autoimmune
  • Allergy (atopic)
  • Irritation
  • Nutritional (deficiency of folic acid, vitamin B12, riboflavin, or achlorhydria, etc.)
  • Infection (fungus)
  • Metabolic or systemic (hepatic, hematological), etc.
• Autoimmune disorders like thyroid disease, pernicious anemia and diabetes are often associated
• About 40% women are either having or going to develop an autoimmune condition
• Common allergens


  • Cosmetics
  • Synthetic underwears
  • Fragrances
• Details to cover in history

  • Symptoms (itching, irritation, pain or vulvar discharge; associated symptoms; relationship with menstruation; association with cervical smear, cell abnormality or CIN)
  • Sexual history (dyspareunia, discharge, sexually transmitted infections, use of medications to cause allergy or irritation)
  • Others (smoking, alcohol intake)
• Skin biopsy may not be needed when a diagnosis can be made on clinical examination
• Biopsy is required if the woman fails to respond to treatment or there is clinical suspicion of VIN or cancer
• The sites for biopsy are from the margins of cracks and fissures and the sky blue areas left behind after applying 1% aqueous toluidine blue to the vulva and washing it off after 1 minute with 1% acetic acid
• Lichen sclerosus


  An autoimmune mediated dermotosis that affects the skin of the anogenital region
• Lichen sclerosus

  • Occurs in postmenopausal women
  • The epithelium is metabolically active
  • May occur even in childhood but resolves after menarche
• Pathophysiology of lichen sclerosus

  • Autoimmune disorders are associated in 20-30% cases
  • Levels of dihydrotestosterone (DHT) and androstenedione are low
• Distribution of lichen sclerosus lesion

  • Involves the entire vulva, encircles the vestibule, affects clitoris, labia minora, inner aspects of labia majora and the skin around the anus
  • Usually bilateral and symmetrical in a figure of eight distribution
  • May extend to the perineum and beyond the labiocrural folds to the thighs
  • May also affect other parts of the body (trunk and limbs—18%)
• Clinical features of lichen sclerosus

  • Pruritus is more than soreness
  • Dyspareunia (excluding childhood)
  • Sleeplessness
  • Dysuria
  • Thin and white skin
  • Inflammatory adhesions of the labia minora and fusion
  • Narrowing of vaginal introitus
  • Pruritus is related to active inflammation with erythema
  • Scratching results in subepithelial hemorrhages (ecchymosis)
• The diagnosis of lichen sclerosus should be confirmed by biopsy
• Histology of lichen sclerosus

  • Thin epithelium with epidermal atrophy
  • Hyperkeratosis, parakeratosis, acanthosis and elongation of rete ridges with evidence of collagen hyalinization
  • Fibroblasts are absent
  • Presence of inflammatory cells including lymphocytes and plasma cells
• The risk of malignancy in lichen sclerosus is less than 5%
• Treatment of lichen sclerosus

  1. Patient education to avoid chemical or mechanical irritants

  2. Ultrapotent topical corticosteroid preparation such as clobetasol propionate 0.05% (or 0.05% halobetasol propionate)

  3. Phototherapy following pretreatment with 5-aminolevulinic acid

  4. Lesions resistant to corticosteroids need treatment with tacrolimus and pimecrolimus

  5. Surgical excision for women nonresponsive to topical steroids or having hyperkeratotic lesion on biopsy

  6. Perineoplasty: Vaginal dilatation, surgical release of adhesions
• Lichen planus


  • Affects the skin, oral and genital mucosa
  • T cell autoimmunity directed against the basal keratinocytes is thought to be the cause
  • Lesion may be erosive, papulosquamous or hypertrophic
• Lichen planus


  • Erosive type is most common, involves the vulva, vagina and gingival margins
  • May be drug induced (methyldopa, β blockers, NSAIDs)
  • Presents with vulvovaginal pruritus, burning pain, dyspareunia and postcoital bleeding
  • Vaginal erosions can produce adhesions
• Management of lichen planus

  1. Multidisciplinary team involvement

  2. Ultrapotent topical corticosteroid (clobetasol 0.05%) is effective

  3. Vaginal lichen planus is treated with corticosteroid, vaginal suppositories containing 25 mg of hydrocortisone

  4. Surgical adhesiolysis is the last option
• The risk of malignancy in lichen planus is low
• Lichen simplex chronicus


  • Presents with severe intractable pruritus, especially at night
  • Nonspecified inflammation involves the labia majora, mons pubis and the inner thighs
  • Symptoms may be exacerbated by chemicals or low body iron stores
• Investigations and treatment of lichen simplex chronicus

  1. Investigations to look for thyroid dysfunction, diabetes, STIs, serum ferritin

  2. Mainstay of treatment is to avoid any irritants, antihistamines, ultrapotent topical steroids (clobetasol) to break the itch-scratch cycle

  3. Corticosteroid suppository may be used
• Extramammary Paget's disease of the vulva

  • A rare condition seen in postmenopausal women
  • Presents with pruritus and florid eczematous lesion with erythema and excoriation
  • Can be associated with underlying adenocarcinoma
• Management of extramammary Paget's disease

  1. Surgical excision to exclude adenocarcinoma of a skin appendage

  2. Photodynamic therapy and topical imiquimod have been used with some success

  3. Recurrence is common
• Vulvar intraepithelial neoplasia (VIN) presents as vulvar skin disorders
• Vulvar Crohn's disease


  • Crohn's disease affecting the intestine may involve the vulva in late stage of the disease in about 25% of cases
  • Vulvar lesions are commonly metastatic
  • Presents with typical granulomatous inflammation, swollen and edematous vulva with granulomas, abscesses, draining sinuses or ulceration
  • May have classic 'knife-cut' fissures in the interlabial sulci
• Treatment of vulvar Crohn's disease

  1. Multidisciplinary team involvement needed

  2. Potent topical steroids along with systemic steroids may be needed

  3. Other medications include prolonged courses of metronidazole and oral immunomodulators (corticosteroids)

  4. Surgery should be avoided as it may cause sinus and fistula formation and tissue breakdown
• Recurrence is common