The differential diagnosis can be difficult, which often leads to diagnostic delays. The hallmark characteristics of VVS are the character of the pain (raw, burning pain or sharp, knife-like pain) (6), its localization (confined to the vulvar vestibule), and its elicitation (in response to touch or pressure). Thus, VVS differs from dysesthetic vulvodynia, which involves chronic, often nonlocalized vulvar pain that occurs with or without stimulation. These two vulvar pain syndromes are distinguished from contact dermatitis, the vulvar dermatoses, and acute vulvovaginal infection by minimal clinical findings (i.e., visual, microscopic, and/or histopathologic), other than the symptom of pain with an unexplained cause.
Other organic causes of vulvar pain must be ruled out before establishing the diagnosis of VVS. Itch is not a symptom; hence, the absence of vulvar itch is a distinguishing characteristic from acute vulvovaginal candidiasis, allergic contact dermatitis, lichen simplex chronicus, lichen sclerosus, and lichen planus. The absence of skin or mucosal lesions, or of visual signs of inflammation other than mild vestibular erythema also distinguish VVS (and dysesthetic vulvodynia) from contact dermatitis, lichen simplex chronicus, lichen sclerosus, erosive lichen planus, and genital herpes simplex.
Microscopic findings help to exclude infectious vulvitis. Patch testing with standard allergens is not recommended unless allergic contact dermatitis (delayed contact hypersensitivity) is suspected in the differential diagnosis; no relevant reactions either to standard allergens or to a series pertinent to perianal or vulvar disorders were found in VVS patients (18). However, a subset of women with VVS exhibited immediate-type (or Type I) hypersensitivity to seminal fluid, as assessed by their plasma antibody titers to pooled semen samples. A majority of these patients reported that their symptoms began with an episode of sexual intercourse and that they experienced symptoms only during and after intercourse. Hence, allergy to a component of seminal fluid may be an unrecognized contributing or exacerbating factor in some cases of VVS.
Traditionally, histopathology has been of little value except to exclude other conditions. The inflammatory nature of this syndrome is the subject of debate; a nonspecific inflammatory infiltrate is observed in the tissue surrounding the vestibular glands, but this is also seen in normal tissue (19). Recently, computerized image analysis of immunostained biopsy samples has demonstrated that the number of degranulated mast cells localized to the minor vestibular glands and the overall heightened innervation of the tissue are distinguishing factors in patients with severe VVS (20).
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