Benign vulvar disorders are a significant issue for patients.

In the last few years, interest in vulvar disease has greatly increased. However, the relevant material has been scattered throughout the literature of various specialties, including dermatology, genitourinary medicine, gynecology, and pathology. The spectrum of involved specialties reflects the complexity of vulvar diseases and the necessity of a multidisciplinary approach to the study of the vulva.[1] In response to the various approaches of the specialists faced with treating vulvar disease, the World Health Organization, the International Society for the Study of Vulvar Disease, the International Federation of Gynecology and Obstetrics, and the International Society of Gynecological Pathologists have made an effort to standardize the nomenclature.

Some general anatomic, embryologic, and histologic findings of the vulva merit review. The vulva is the part of the female genital tract located between the genitocrural folds laterally, the mons pubis anteriorly, and the anus posteriorly. Embryologically, it is the result of the junction of the cloacal endoderm, urogenital ectoderm, and paramesonephric mesodermal layers. This hollow structure contains the labia majora, labia minora, clitoris, vestibule, urinary meatus, vaginal orifice, hymen, Bartholin glands, and Skene ducts. Different epithelia, from keratinized squamous epithelium to squamous mucosa, cover the vulva. The labia minora are rich with sebaceous glands but have few sweat glands and no hair follicles. The epithelium of the vestibule is neither pigmented nor keratinized and contains eccrine glands.

Benign vulvar disorders are a significant issue for patients. These disorders include vulvar atrophy, benign tumors, hamartomas and cysts, infectious disorders, and nonneoplastic epithelial disorders.[2] Infectious disorders include diseases caused by known transmissible agents, such as viruses, bacteria, fungi, and protozoa. They may first be seen by physicians of various specialties, including dermatologists and gynecologists, and often require a multidisciplinary approach.

Developmental abnormalities of vulva are generally rare. Vulvar atrophy may be related to advanced age or other disorders, but these abnormalities often represent an almost physiological finding in the elderly.

Benign tumors of the vulva are relatively uncommon and may show nonspecific clinical features. Therefore, a biopsy is often needed to exclude a malignant neoplasm and to indicate proper treatment. Vascular neoplasms may also occur in the vulva and are similar to such lesions found elsewhere.

Nonneoplastic epithelial disorders include several inflammatory, ulcerative, and blistering disorders, as well as pigmentary changes involving the vulvar region.

Inflammatory diseases

• Lichen sclerosus
• Squamous cell hyperplasia (with and without atypia)
• Lichen simplex chronicus (localized neurodermatitis)
• Primary irritant dermatitis
• Intertrigo
• Allergic contact dermatitis
• Fixed drug eruption
• Atopic dermatitis
• Seborrheic dermatitis
• Psoriasis
• Reiter disease
• Lichen planus
• Lupus erythematosus
• Darier disease
• Aphthosis and Behçet disease
• Pyoderma gangrenosum
• Crohn disease
• Hidradenitis suppurativa
• Fox-Fordyce disease
• Plasma cell vulvitis
• Vulvar vestibulitis

Blistering diseases

• Familial benign chronic pemphigus (Hailey-Hailey disease)
• Bullous pemphigoid
• Cicatricial pemphigoid
• Pemphigus vulgaris
• Erythema multiforme
• Epidermolysis bullosa

Pigmentary changes

• Acanthosis nigricans
• Lentigo, lentiginosis, and benign vulvar melanosis
• Melanocytic nevus
• Postinflammatory hyperpigmentation
• Postinflammatory hypopigmentation
• Vitiligo

Benign tumors, hamartomas, and cysts

• Mucous cysts
• Bartholin and Skene duct cysts
• Epidermal inclusion cyst
• Seborrheic keratosis
• Acrochordon (fibroepithelial polyp)
• Fibroma, fibromyoma, and dermatofibroma
• Lipoma
• Hidradenoma
• Syringoma
• Hemangioma
• Lymphangioma
• Angiokeratoma
• Pyogenic granuloma
• Endometriosis
• Heterotopic sebaceous glands and sebaceous gland hyperplasia
• Papillomatosis (papillary vulvar hirsutism)

Congenital malformations

• Ambiguous external genitalia
• Congenital labial hypertrophy
• Labial adhesions

Atrophy of the vulva