Pathology Thread

Non-neoplastic Epithelial Disorders

Objectives:

After completing this tutorial the student will be able to:

• list common non-neoplastic epithelial disorders of the vulva
• describe their clinical features
• describe/recognize histologic features
• state clinical behavior
• state treatment

1. Dermatoses

The vulva epithelium is subject to all the dermatoses that affect the body skin generally. These include psoriasis, lichen planus, contact dermatitis, lichen simplex chronicus.

2. Vulvar Dystrophy

This refers to a clinically related group of disorders of epithelial growth that usually present as white lesions (hence the old term leukoplakia) of the vulva.

Question: What other vulvar conditions can present as white lesions?

There are two basic clinicopathologic types:

1. Lichen sclerosis (lichen sclerosis et atrophicus)

This condition affects mainly women above 50 years but can affect any age group including children. The lesion appears as white, atrophic, "parchment paper-like" or crinkled paper patches of the vulvar skin, occasionally accompanied by marked contracture with narrowing of the introitus (hence the old term kraurosis vulvae).



Note the white epithelium with atrophic labia majora and narrowing of the vestibule.

Question: What symptoms may this patient complain of?

• Histology

There is both epidermal and dermal atrophy (atrophic vulvitis). The epithelium is thinned with blunting or loss of rete pegs and a band of homogeneous, acellular subepithelial zone due to edema and hyalinization of dermal collagen. A band of chronic inflammatory cell lies beneath this zone.



There is mild hyperkeratosis, loss of rete pegs and homogenization of the dermis.

• Clinical behavior

The disease is insidious, slow in developing and progressive. Most lesions have no premalignant potential, but if dysplasia is present, can be associated with a low risk for development of squamous cell carcinoma.

2. Squamous cell hyperplasia (hyperplastic dystrophy)

This presents as thick gray white plaques of the vulva and occurs in women between 30 and 60 years old. Areas lacking keratin layer appear red. The patches may be circumscribed or diffuse. Any part of the vulva, adjacent thighs, perineum or perianal skin may be involved. The lesions are pruritic and scratching adds trauma and chronic inflammation.

• Histology

There is epithelial thickening with acanthosis and hyperkeratosis. Parakeratosis may occur on the surface.



Mild hyperkeratosis is present along with epithelial thickening. No nuclear atypia is present.

• Clinical behavior

The lesion is not premalignant, but if dysplasia is present, can be associated with a low risk for development of squamous cell carcinoma.

Many conditions involving the vulva, benign, premalignant and malignant, present as white patches. It is very crucial for patient management to distinguish between these lesions. Biopsy is therefore very important and indicated for the diagnosis of all white lesions of the vulva.

3. Hidradenitis suppurativa

Hidradenitis suppurativa is a chronic, unrelenting skin condition that results from blockage and subsequent infection of the apocrine glands. The hair-bearing areas of the vulva are involved.

Question: Name other areas of the body that may be affected.

• Clinical presentation

Patients present with deep-seated, extremely painful and tender nodules in the vulva. The lesions progress and coalesce to form confluent abscesses, which rupture giving rise to multiple, chronically draining sinus tracts.

• Histology

In the early stages there is a perifolliculitis with a dermal acute and chronic inflammatory cell infiltrate. In the late stage there is destruction of skin appendages with sinus tract formation.

4. Bartholin’s cyst

This is due to obstruction of the duct draining the Bartholin gland leading to retention of secretion and resultant cystic dilatation of the duct.

• Clinical presentation

The condition presents as a palpable mass in the lower third of the vestibule, posterolateral to the introitus. It occurs at all ages and may cause local discomfort and dyspareunia.

Bartholin’s cyst. The lesion is located on the right lower third, posterolateral to the introitus.

• Histology

The cyst is lined by transitional, low cuboidal or flattened epithelium (due to increased intracystic pressure).

• Treatment

Marsupialization of the duct.

Question: What complication may involve the cyst?