Vulva, vagina & female urethra

Table 1: Diagnostic criteria of vulvar erosive lichen planus by the International Society of the Study of Vulvovaginal Disease (ISSVD) (adapted from Br J Dermatol 2013;169:337)

3 out of 9 criteria needed for diagnosis

Well demarcated erosions or glazed erythema of introitus

Wickham striae on surrounding skin

Pain or burning

Scarring or loss of architecture

Vaginal inflammation

Involvement of other mucosal surfaces

Well defined inflammatory band in superficial connective tissue involving dermoepidermal junction

Inflammatory band consists of predominantly lymphocytes

Evidence of basal cell layer degeneration (e.g., Civatte bodies, abnormal keratinocytes or basal apoptosis)

Table 2: Summary of minimum clinicopathologic criteria to diagnose vulvar erosive lichen planus, more specific than those proposed by ISSVD (adapted from J Low Genit Tract Dis 2020;24:317)

All 5 criteria must be present
Clinical presentation (signs)	Well demarcated glazed red macule or patch located on vestibule, labia minora or vagina*
Histopathological correlate	Nonkeratinized squamous epithelium, mucocutaneous junction or adjacent hairless skin
	Band-like lymphocytic infiltrate abuts the epithelial layer
	Basal layer with one of the following patterns Degenerative pattern: apoptotic bodies, vacuolar change or squamatization Specific features: Civatte bodies (dyskeratosis) within epidermis and colloid bodies in papillary dermis secondary to basal keratinocyte damage Regenerative patterns: high N:C ratio, mitoses, diminished epithelial maturation
	Sclerosis is absent

• *Supportive features
  • Architectural changes include midline fusion or labia minora, adhesions between affected opposed structures
  • Common comorbidities: lichen sclerosis (LS), other types of locations of lichen planus, systemic autoimmune diseases

Table 3: Clinicopathologic features of classic hypertrophic lichen planus (adapted from J Low Genit Tract Dis 2020;24:317)

	Classic lichen planus	Hypertrophic lichen planus
Clinical features	Red, gray-white or purple-brown plaque or papules on hairless or hair bearing skin	Circumferential red plaque over hairless and hair bearing skin of vulva or perianal area
Histopathological correlate
Stratum corneum*	Hyperkeratosis	Marked hyperkeratosis
Granular cell layer*	Hypergranulosis	Diffuse and marked hypergranulosis
Rete ridge morphology	Mild to moderate acanthosis with irregular rete ridges, often sawtoothed or spiky	Marked acanthosis with deep and irregular rete ridges
Basal layer	Apoptotic bodies, squamatization, vacuolar change Lymphocytosis	Apoptotic bodies, squamatization, vacuolar change may be confined to rete tips / basal layer above papillary processes Lymphocytosis
Dermis	Band-like applied lymphocytic infiltrate
		Papillary dermal fibrosis

• *May be altered by excoriation, especially in hypertrophic lichen planus

Contributed by M. Luann Racher, M.D.


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Contributed by Anna Sarah Erem, M.D. and Gulisa Turashvili, M.D., Ph.D.

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• p53: aberrant expression of basal cells in degenerative and regenerative areas

• J Low Genit Tract Dis 2019;23:185, J Low Genit Tract Dis 2021;25:57, J Low Genit Tract Dis 2021;25:71

A 63 year old woman previously diagnosed with lichen planus returns to the clinic with a persistent, well demarcated plaque and suboptimal treatment response. A biopsy shows lichenoid inflammation with minimal basal atypia. What result or clinical information would favor reactive process over neoplastic process?

1. Complete loss of p53
2. Diffuse, block-like expression of p16
3. Prior history of HPV independent squamous cell carcinoma
4. Wild type p53 expression

D. Wild type p53 expression. Wild type p53 expression pattern supports the diagnosis of lichen planus, although aberrant expression can be seen in the basal cells of regenerative erosive lichen planus. Answer A is incorrect because the complete loss of p53 constitutes aberrant expression and would be concerning for differentiated vulvar intraepithelial neoplasia. Answer B is incorrect because diffuse block-like expression of p16 is characteristic in HPV associated with a high grade squamous intraepithelial lesion. Answer C is incorrect because prior history of HPV independent squamous cell carcinoma increases a pretest probability in a 63 year old woman with a persistent vulvar plaque and suboptimal treatment response.

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Reference: Lichen planus

A 45 year old woman with a past medical history of hypothyroidism presents to the clinic with pain and burning discomfort in the vulvar area. On physical examination, a well delineated red plaque in the vaginal introitus is observed extending onto the labia majora. There is no scarring or obvious vaginal inflammation. A punch biopsy was obtained. PASF shows no fungal elements. Based on the microphotograph above and clinical information provided, what is the most likely diagnosis?

1. Fixed drug reaction
2. High grade squamous intraepithelial lesion (HSIL)
3. Syphilis
4. Vulvar hypertrophic lichen planus

D. Vulvar hypertrophic lichen planus. The patient presented with a circumferential red plaque over the hairless and hair bearing skin of the vulva. The microphotograph shows marked acanthosis with deep and irregular rete ridges, hypergranulosis and dense, band-like lymphocytic infiltrate in the superficial dermis, with apoptotic bodies squamatization and superficial perivascular inflammation. Taken together with the clinical presentation, the findings are consistent with vulvar hypertrophic lichen planus. Answer A is incorrect because although it is important to evaluate medication history, there are no conspicuous eosinophils in the epidermis and superficial dermis, which makes a fixed drug reaction less likely. Answer B is incorrect because high grade squamous intraepithelial lesion can be ruled out based on the absence of full thickness epithelial atypia and increased suprabasal mitotic activity. Answer C is incorrect because syphilis is typically associated with minimal dyskeratosis and neutrophilic infiltrate in the early stages and granulomatous inflammation later in the disease, usually with deep perivascular inflammation.

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Reference: Lichen planus