Acute idiopathic vulvar ulcer: An acute, single, and painful ulcer of the medial aspect of the labia minora following within 24 hours of intercourse. The ulcer is of uncertain etiology.

Atopic dermatitis: Inflammation associated with pruritus and burning of unknown etiology but may be mediated through IgE and epidermal Langerhans cells.

Contact dermatitis: A cell-mediated inflammatory response secondary to a specific sensitizing agent (e.g., rubber, nickel, etc.). Alternatively, an irritant-type inflammatory reaction secondary to contact exposure to a specific chemical or physical agent.

Factitial vulvitis: Inflammation or ulceration of the vulva that is self-induced.

Fixed drug eruption (dermatitis medicamentosa): An urticarial, purpuric, bullous, maculopapular or other eruption of the vulva mediated by immunoglobulins (types I and II) or immunocomplexes (type III) or that is cell mediated (type IV) secondary to drug allergy.

Fox-Fordyce disease: A disorder of apocrine glands associated with plugging of apocrine sweat ducts with keratin, causing severe pruritus and a papular eruption of the involved area.

Fordyce spots: A normal finding reflecting the visualization of small white to pale yellow, slightly raised spots, representing normal sebaceous glands without hair follicles, beneath the epithelium. In the vulva these are most commonly seen in the posterior lateral vulva just peripheral to the Hart line.

Intertrigo: (See Eczema; also referred to as eczema intertrigo.) A symptomatic dermatitis involving opposed skin surfaces such as the groin area and gluteal folds that is characteristically superficial and more commonly observed in obese individuals. Erosion with secondary infection may occur.

Necrotizing fasciitis (synergistic bacterial infection): A life-threatening superficial and deep necrotizing inflammatory process of polymicrobial origin secondary to surgical trauma or injury.

Pyoderma gangrenosum: A localized progressive ulcerative inflammatory process involving the vulvar skin, of unknown etiology.

Vulvitis associated with vaginitis: Inflammation of the vulva due to a specific vaginitis or vaginosis.

Vulvitis granulomatosa: A granulomatous inflammatory process associated with edema, induration, and erythema of the labia majora and adjacent areas that may be associated with regional lymphadenopathy and related to chelitis granulomatosa and Crohn disease.

Ciliated cysts of the vulvar vestibule (Müllerian metaplasia and vestibular adenosis): Cysts and surface epithelial changes characterized by tubal-endometrial-type epithelium without adjacent endometrial stroma.

Gartner duct cyst: A benign cyst, much more common in the vagina, lined with low cuboidal epithelium related to cystic dilation of a Wolffian or mesonephric duct (see mesonephric-like cyst).

Müllerian metaplasia (acquired adenosis): Acquired Müllerian-type epithelium, as seen within the vagina or vulvar vestibule, in which Müllerian-type columnar epithelium is found where squamous mucosa was once present. These changes in the vestibular epithelium, with squamous epithelium replaced by columnar epithelium, have been described secondary to acute inflammation of Stevens-Johnson syndrome, as well as secondary to 5-fluorouracil and/or laser therapy of the vestibule.

Cyst of the canal of Nuck (mesothelial cysts; hydrocele, canal of Nuck): A fluid-filled simple, cystlike structure lined by peritoneum usually presenting as enlargement of the superior aspect of the labia majus or a mass in the inguinal canal.

Mesonephric-like cyst (Wolffian-like duct cyst): Simple cysts of the vulva lined by cuboidal or columnar epithelium that is not ciliated and has smooth muscle beneath the epithelium (see Gartner duct cyst).

Periurethral cysts: Cysts found immediately adjacent to the urethra that may have stratified squamous epithelial, transitional epithelial, or tall, pale mucus-secreting columnar epithelial lining.

Keratoacanthoma: A benign, but rapidly growing, self-limited epithelial neoplasm occurring on hair-bearing areas. The neoplasm has infiltrative margins and a keratinfilled crater. It regresses spontaneously if untreated.

Milium (singular); Milia (pl.): Small, superficial epidermal inclusion cysts that arise within skin bearing pilosebaceous
follicles. They contain keratinous material and are usually multiple and often clustered. Because of their clinical appearance they may be referred to as “whiteheads.”

Papillomatosis (vestibular papillomatosis, micropapillomatosis labialis): Although one or a few papillae may be seen normally in the vulvar vestibule near the hymen, papillomatosis is a clustering of papillae, usually associated with vulvar symptoms or local irritation

Trichilemmoma: A benign skin appendage tumor that contains amorphous keratin.

Trichoepithelioma: A benign tumor of hair follicle origin composed of small “horn cysts” containing keratin and surrounded by basaloid epithelial cells. Hair and hair-forming epithelial elements may be present.

Trichofolliculoma: A benign skin tumor associated with hair follicles that typically presents as a single small superficial dermal mass less than 1.2 cm in greatest dimension. The epithelium overlying the mass may be dimpled and communicate with the underlying cyst(s). Microscopically the tumor is lobular with basaloid-type epithelial cells with variable sebaceous differentiation lining the cyst. Within the cyst some fine hairs, mixed with keratinous material, are usually found.

Adenoma of minor vestibular glands: A benign, small (usually 1 to 2 mm) neoplasm composed of clustered mucin-secreting columnar epithelial-lined glands occurring within the vulvar vestibular; of minor vestibular gland origin.

Adenoma of Skene gland: A benign tumor, or nodular hyperplasia, arising within the vulvar vestibule of minor vestibular gland origin. Generally under 2 mm in greatest dimension, the tumor microscopically is multinodular and composed of clusters of low cuboidal mucin-secreting columnar epithelial-lined glands.

Breast-like tissue (“ectopic” breast tissue): Breast-like tissue within the vulva arising in specialized anogenital glands. Adenocarcinoma of the usual ductal breast type has been reported arising in the vulva, arising from this breast-like tissue, as have other benign breast-type tumors (see Duct adenocarcinoma).

Ectopic salivary gland tissue: The finding of ectopic salivary gland within the vulva.

Folliculosebaceous cystic hamartoma: A benign tumor of a pilosebaceous unit of the skin presenting as a nodule or papule. Microscopically the cysts are lined with squamous epithelium and surrounded with sebaceous lobules and fibrous tissue with features of a disorganized pilosebaceous unit.

Mixed tumor of the vulva (pleomorphic adenoma): A benign tumor composed of epithelial tubules with a fibrous stromal element that may contain osseous, myxoid, and chondromatous components. The complex stromal component is believed to be of myoepithelial origin.

Nodular hidradenoma (clear cell hidradenoma, clear cell myoblastoma, clear cell myoepithelioma, solid cystic hidradenoma, eccrine acrospiroma, eccrine sweat gland tumor, adenoma of clear cell type): A benign neoplasm of eccrine gland origin composed of glandular epithelial cells with distinctive clear cytoplasm.

Syringoma: A benign epithelial tumor arising from the ductal epithelium of the eccrine sweat gland characterized by small epithelial-lined duct like structures that are round to comma shaped and form cysts containing eosinophilic secretion. The surrounding stroma or dermis is fibrous.

Aggressive angiomyxoma: (see malignant soft tissue tumors).

Angiomyofibroblastoma:

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