Medically speaking, the term denotes a squamous intraepithelial lesion of the vulva that shows dysplasia with varying degrees of atypia. The epithelial basement membrane is intact and the lesion is thus not invasive but has invasive potential.

The terminology of VIN evolved over several decades. In 1989 the Committee on Terminology, International Society for the Study of Vulvar Disease (ISSVD) replaced older terminology such as vulvar , Bowen's disease, and Kraurosis vulvae by a new classification system for "Epithelial Vulvar Disease":

- Nonneoplastic epithelial disorders of vulva and mucosa:

- Lichen sclerosus

- Squamous hyperplasia

- Other dermatoses

- Mixed neoplastic and nonneoplastic disorders

- Intraepithelial neoplasia

- Squamous vulvar intraepithelial neoplasia (VIN)

- VIN I, mildest form

- VIN II, intermediate

- VIN III, most severe form including carcinoma in situ of the vulva

- Non-squamous intraepithelial neoplasia

- Extramammary Paget's disease

- Tumors of melanocytes, noninvasive

- Invasive disease (vulvar carcinoma)

The ISSVD further revised this classification in 2004, replacing the three-grade system with a single-grade system in which only the high-grade disease is classified as VIN.

VIN is subdivided into: (Robbins Pathological Basis of Disease, 9th Ed)

Classic vulvular intraepithelial neoplasia: associated with developing into the warty and basaloid type carcinoma. This is associated with carcinogenic genotypes of HPV and/or HPV persistence factors such as cigarette smoking or immunocompromised states.

Differentiated vulvar intraepithelial neoplasia also known as VIN Simplex: is associated with vulvar dermatoses such as lichen sclerosus. It is associated with atypia of the squamous epithelium.

The patient may have no symptoms, or local symptomatology including itching, burning, and pain.

The diagnosis is always based on a careful inspection and a targeted biopsy of a visible vulvar lesion.

The type and distribution of lesions varies among the two different types of VIN. In the Usual type VIN, seen more frequently in young patients, lesions tend to be multifocal over an otherwise normal vulvar skin. In the differentiated type VIN, usually seen in postmenopausal women, lesions tend to be isolated and are located over a skin with a vulvar dermatosis such as Lichen slerosus.

Warty dyskeratoma must be differentiated from vulvar dysplasia, Bowenoid papulosis, squamous carcinoma, condyloma, and other viral-induced squamous lesions.

The most common location by far is the gingival margin and other areas of the masticatory oral mucosa, these occur more frequently in the fifth decade of life, and have good prognosis, the treatment of choice for oral VXs is surgical excision, and recurrence is rare.

The condition can affect other organs of body, such as the penis, vulva, and can occur in anal region, nose, the ear, lower extremity, scrotum.

Differential diagnosis includes seborrheic keratosis, verruca simplex, condyloma acuminatum, granular cell myoblastoma, vulvar intraepithelial neoplasia, bowenoid papulosis, erythroplasia of Queyrat, and verrucous carcinoma

Bowenoid papulosis is a cutaneous condition characterized by the presence of pigmented verrucous papules on the body of the penis. They are associated with human papillomavirus, the causative agent of genital warts. The lesions have a typical dysplastic histology and are generally considered benign, although a small percentage will develop malignant characteristics.

It is considered as a pre-malignant condition. Other terms used to describe the condition are: Erythroplasia of Queyrat, Squamous cell carcinoma in situ and Bowen’s disease. The term "Bowenoid papulosis" was coined in 1977 by Kopf and Bart and is named after dermatologist John Templeton Bowen.

The term “intraepithelial neoplasia” defines a premalignant intraepithelial change.

On the vulva it is termed VIN (vulvar or vulval intraepithelial neoplasia); on the penis, PIN (penile intraepithelial neoplasia); and on or around the anus, AIN (anal intraepithelial neoplasia). The terminology has been very confusing and it is now recommended that the terms Bowen’s disease, erythroplasia of Queyrat, and bowenoid papulosis should not be used for lesions in the anogenital area. However, dermatologists still recognize a distinct clinical variant, bowenoid papulosis, characterized by discrete papules in a younger age group and a tendency for spontaneous regression. Additionally, some authorities believe that erythroplasia of Queyrat and Bowen’s disease remain useful terms in men.

Many malignancies can develop in vulvar structures. The signs and symptoms can include:

- Itching, burn, or bleeding on the vulva that does not go away.

- Changes in the color of the skin of the vulva, so that it looks redder or whiter than is normal.

- Skin changes in the vulva, including what looks like a rash or warts.

- Sores, lumps, or ulcers on the vulva that do not go away.

- Pain in the pelvis, especially during urination or sex.

Typically, a lesion presents in the form of a lump or ulcer on the labia majora and may be associated with itching, irritation, local bleeding or discharge, in addition to pain with urination or pain during sexual intercourse. The labia minora, clitoris, perineum and mons are less commonly involved. Due to modesty or embarrassment, patients may put off seeing a doctor.

Melanomas tend to display the typical asymmetry, uneven borders and dark discoloration as do melanomas in other parts of the body.

Adenocarcinoma can arise from the Bartholin gland and present with a painful lump.

Bowen's disease typically presents as a gradually enlarging, well-demarcated red colored plaque with an irregular border and surface crusting or scaling. Bowen's disease may occur at any age in adults, but is rare before the age of 30 years; most patients are aged over 60. Any site may be affected, although involvement of palms or soles is uncommon. Bowen's disease occurs predominantly in women (70–85% of cases). About 60–85% of patients have lesions on the lower leg, usually in previously or presently sun-exposed areas of skin.

This is a persistent, progressive, unelevated, red, scaly or crusted plaque which is due to an intraepidermal carcinoma and is potentially malignant. The lesions may occur anywhere on the skin surface, including on mucosal surfaces. Freezing, cauterization, or diathermy coagulation is often effective treatment. Pathomorphologic study of tissue sampling revealed: polymorphism of spiny epithelial cells has progressed into atypism; increased mitosis; giant and multinucleate cells; acanthosis; hyperkeratosis and parakeratosis; basal membrane and basal layer are retained.

Warty dyskeratoma, also known as an Isolated dyskeratosis follicularis, is a benign epidermal proliferation with distinctive histologic findings that may mimic invasive squamous cell carcinoma and commonly manifests as an umbilicated (Having a central mark or depression resembling a navel) lesion with a keratotic plug, WD have some histopathologic similarities to viral warts but it's not caused by HPV and the majority of these lesions display overall histopathologic features consistent with a follicular adnexal neoplasm. usually limited to the head, neck, scalp or face and vulva. Lesions are generally and sporadic and may be associated with a follicular unit. Oral involvement, particularly the hard palate, and genital involvement have been reported. it can also be thought of as one of the manifestations of focal acantholytic dyskeratosis, an epidermal reaction pattern that can be seen in several disorders, including Darier's disease and Grover's disease. But the main Difference between Darier disease and Warty dyskeratoma, is that Darier disease inherited dermatosis (autosomal dominant) consisting of multiple keratotic papules on the face, trunk, and extremities, while WD occurs as an isolated, noninherited, single keratotic nodule mainly confined to the head and neck as mentioned earlier.

A papillary hidradenoma, also hidradenoma papilliferum, is a sharply circumscribed nodule or benign tumor of the apocrine gland usually found on the labia majora or the interlabial folds. It is benign tissue (not cancerous) but is often confused clinically with carcinoma of the vulva because of its tendency to ulcerate.

Bowen's disease, also known as squamous cell carcinoma" in situ" is a neoplastic skin disease. It can be considered as an early stage or intraepidermal form of squamous cell carcinoma. It was named after John T. Bowen.

Erythroplasia of Queyrat is a particular type of Bowen's disease that can arise on the glans or prepuce in males, and, on the vulva in females, and may be induced by human papilloma virus. It is reported to occur in the corneoscleral limbus.

Basal cell carcinoma makes up about 1–2% of vulvar cancer. These tend to be slow-growing lesions on the labia majora but can occur anywhere on the vulva. Their behavior is similar to basal cell cancers in other locations. They often grow locally and have low risk for deep invasion or metastasis.

Treatment involves excision, but these lesions have a tendency to recur if not completely removed.

Eccrine carcinoma (also known as a syringoid carcinoma) is a rare cutaneous condition characterized by a plaque or nodule on the scalp, trunk, or extremities.

A poroma is a benign skin tumor derived from sweat glands. Although the original term "poroma "was initially used to describe a tumor derived from the ductal epithelium of eccrine sweat glands, the term is used in general reference to tumors derived from ductal portions of both eccrine and apocrine sweat glands. Specific sub-types are primarily defined by location of tumor, and include:

- eccrine poroma

- hidroacanthoma simplex (intraepithelial poroma)

- dermal duct tumor (intradermal poroma)

- syringoacanthoma

- syringofibroadenoma

- poroid hidradenoma ("disputed as different family")

Syringofibroadenoma (also known as "acrosyringeal nevus of Weedon and Lewis") is a cutaneous condition characterized by a hyperkeratotic nodule or plaque involving the extremities.

It is considered of eccrine origin.

Hidradenoma (also known as acrospiroma, from "akral ""peripheral"" + spiroma ""epithelial tumor of sweat gland") refers to a benign adnexal tumor of the apical sweat gland. Another name for Hidradenoma is Cystadenoma and Hydrocystadenomas. These are 1–3 cm translucent blue cystic nodules.It usually presents as a single, small skin-colored lesion, and is considered distinct from the closely related poroma. Hidradenomas are often sub-classified based on subtle histologic differences, for example:

- clear-cell hidradenoma or acrospiroma

- nodular hidradenoma or acrospiroma

- solid-cystic hidradenoma

Discussion of sweat gland tumors can be difficult and confusing due to the complex classification and redundant terminology used to describe the same tumors. For example, acrospiroma and hidradenoma are synonymous, and sometimes the term "acrospiroma" is used to generally describe benign sweat gland tumors. In addition, a single lesion may contain a mixture of cell-types. There has also been a change in understanding about how tumors that were previously believed to strictly derive from specific sweat gland types may, in fact, derive from both eccrine "or" apocrine glands.

Hidradenomas are by definition benign, with malignant transformation very rare. When tumors show malignant characteristics, they are known as hidradenocarcinoma. Surgical excision is usually curative and local recurrences are rare, although malignant tumors may metastasize.

An eccrine poroma specifically refers to a tumor derived specifically from the most apical portion of eccrine sweat gland ("acrosyringium"). They are most commonly found on acral distribution (on palms & soles), and most commonly in adults. They are described as <1–2 cm pink/red shiny, exophytic lesions that can be pigmented and are clinically very similar to pyogenic granulomas. Histologically, the cells as PAS-positive and clearly surrounded by normal keratinocytes. These tumors are surgically excised usually without complication or recurrence, although malignant porocarcinomas can recur and metastasize to local lymph nodes.

Hidradenocarcinoma (also known as malignant hidradenoma, malignant acrospiroma, clear cell eccrine carcinoma, or primary mucoepidermoid cutaneous carcinoma) is a malignant adnexal tumor of the sweat gland. It is the malignant variant of the benign hidradenoma. It may develop de novo or in association with an existent hidradenoma.

This type of tumor typically develops in older individuals (after age 40).

Symptoms are not specific; most patients report itching, burning, and soreness. A small subset of patients may be asymptomatic. Presence of vulvar pain, bleeding, and tumor formation are reported to be more common in patients affected by invasive disease.Signs and symptoms are skin lesions, often mistaken as eczema, that may be itchy or painful.

Dermal cylindromas are:

- dermal lesions consisting of nests of cells that are surrounded by hyaline (i.e. glassy, eosinophilic, acellular) material and have:

- hyperchromatic nuclei that may palisade (columnar nuclei arranged around the periphery of the cells nests - with their short axis tangential to the nest periphery), and

- cells with lighter staining ovoid nuclei at their centre.

They lack of a significant number of lymphocytes; this differentiates them from spiradenomas.

Vulvar Paget disease presents as a variety of clinical lesions that may occur over a protracted course. Initially it is velvety, soft, and red or bright pink with scattered white islands of hyperkeratosis. (a strawberry and cream appearance) The lesions become erythematous, plaque like, and desquamating especially when located in dry areas. Rarely the appearance is ulcerated. The borders appear irregular, slightly elevated, and sharply demarcated. The visible borders of vulvar Paget disease are often misleading as Paget cells may spread along the basal layers of normal appearing skin with multicentric foci. Involvement may be extensive including the perianal region, genitocrural, and inguinal folds. Clinical examination should determine the presence of periurethral and perianal lesions. In these cases an involvement of the skin by a noncutaneous internal neoplasm may occur.

Bartholin gland can be differentiated by histology to determine whether the malignancy is due to squamous cell carcinoma, adenoid cystic carcinoma, or adenocarcinomas.

Spiradenoma, also spiroma or eccrine spiradenoma, is a cutaneous condition that is typically characterized, clinically, as a solitary, deep-seated dermal nodule of approximately one centimeter, occurring on the ventral surface of the body. Spiradenoma lesions are benign sudoriferous tumors, and have also been described as cystic epitheliomas of the sweat glands.

The histological origin is controversial.

Though Bartholin gland carcinoma is rare, along with other unusual Bartholin gland growths, it many not be the typical practice for clinicians to consider lesions malignant. Early diagnosis can help to prevent the cancer from the glands to surrounding. Though malignancies of the Bartholin gland are rare clinicians biopsy Bartholin gland lesions in older women or when the growth reoccurs or does not respond to original treatment.

In dermatologic pathology, a dermal cylindroma, also dermal eccrine cylindroma or Cutaneous Cylindroma) and (less specifically) cylindroma, is a benign adnexal tumor, which occurs on the scalp and forehead.when multiple Cylindroma-Show Hat like configration.

Multiple cylindromas may grow together in a "hat-like" configuration, sometimes referred to as a turban tumor. Cylindromas are uncommon dysplasias of skin appendages.