Patients with urogenital complaints are frequently encountered by family practitioners, gynecologists, and dermatologists. This review article provides practical information regarding normal anatomy and embryology of the vulva, followed by a summary of procedural techniques. The new classifications of vulvar inflammatory and intraepithelial neoplasm proposed by the ISSVD are used as guidelines to review the wide spectrum of diseases involving this organ.
Currently, urogenital complaints are among the most common problems encountered by family practitioners, gynecologists, and dermatologists. When a patient suffering from a vulvar disorder presents to the health care system, there are 3 elements to be considered: (1) the patient’s sociocultural background that affects how she perceives the disease often leading to delay in seeking care or self-treatment, (2) the clinical presentation of the disease, and (3) the clinical setting of this evaluation, as there is not a single medical specialist trained to care for the spectrum of diseases occurring in this anatomic location.
In response to the intricacy of vulvar disorders, the International Society for the Study of Vulvovaginal Disease (ISSVD) was created to facilitate the exchange between clinicians and pathologists involved in the care of these patients. Often, the “diagnosis” on a biopsy result is not the name of a disease, but rather a description of the microscopic findings. For the average clinician, this is sometimes unhelpful. In an attempt to allow the clinician to benefit from a nondiagnostic biopsy, the ISSVD recently developed a histologic classification of inflammatory vulvar disorders that allows for the generation of a differential diagnosis based on microscopic findings ( Table 1 ). This can be extremely useful when correlated with a clinical differential diagnosis. The ISSVD has also proposed a clinically relevant histologic classification for vulvar intraepithelial neoplasia (VIN) ( Table 2 ).
Spongiotic pattern | Eczematous dermatitis (atopic, contact, and allergic) |
Acanthotic pattern | Lichen simplex chronicus (primary and secondary) |
Psoriasis | |
Reiter’s syndrome | |
Lichenoid pattern | Lichen sclerosus |
Lichen planus | |
Dermal sclerosis pattern | Lichen sclerosus |
Vesiculobullous pattern | Bullous pemphigoid |
Cicatricial pemphigoid | |
Pemphigoid gestationis | |
Pemphigus vulgaris | |
Pemphigo vegetans | |
Acantholytic pattern | Hailey-Hailey disease |
Darier’s disease | |
Acantholytic dermatosis of the vulvocrural area | |
Granulomatous pattern | Crohn’s disease |
Melkersson-Rosenthal syndrome | |
Vasculopathic pattern | Aphthous ulcers |
Behcet’s disease | |
Plasma cell vulvitis |