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Vulvar Pruritus Differential Diagnostic in Medical Practice SCHWERPUNKT Vulvar pruritus Differential diagnostic in medical practice Dr. med. Anne-Thérèse Vlastos Vulvar pruritus, defined as itching of the external female with very few skin findings. The list over the next pages is genitalia, is a common symptom in females of all ages (1) not exhaustive but attempts to cover the vulvar lesions that and may occur in the presence of dermatological disease or occur with a greater likelihood in the practitioner's office. KOH. Cultures for bacteria and fungi are dary syphilis) as a genital wart or over- athophysiologically, the nerves re- necessary to precisely identify Candida, diagnoses «micropapillomatosis» of the Psponsible for pruritus have been bacterial vaginosis, or trichomonas (3). vestibule as evidence of HPV infection identified as unmyelinated C-fibers and are not uncommun. histamine is defined as the main peri- Viral infection Virus of the poxvirus family causes small, pheral mediator of pruritus. Histamine- Viral infection such as Herpes simplex, benign skin tumors to grow on the vulva induced itch has been shown to activate Poxvirus or Human papillomavirus (HPV) known as Molluscum contagiosum, central motor areas which are linked to can also lead to vulvar pruritus although which are usually symptomless but may the act of scratching, thereby supposing it is not the main symptom. sometimes be itching. Confirmatory dia- the observed itch-scratch-itch behavior. A tingling or itching sensation precedes gnosis and treatment follow excisional Table 1 and 2 give a summary of these the development of painful blisters on biopsy or dermal curettage. pathologies. both sides of the vulva in acute herpes infection. The blisters then break and Allergic and contact dermatitis Acute vulvar pruritus form shallow ulcers that crust over and A number of topical agents may cause when the crusts fall off, the underlying allergic contact dermatitis also called ec- Acute vulvar pruritus is often of infec- skin looks normal. The process takes zema, in the genital area. This often pre- tious nature (2), however, allergic and ir- 7–10 days. sents as an acute eczematous dermatitis ritant contact dermatitis playing an im- During the ulcerative stage, the pain may with bright red erythema, weeping and portant role. Identification of these be so intense as to require narcotic anal- significant pruritus. causes may lead to a prompt resolution gesia. Urinating during this time can be Two major types of eczema, exogenous of pruritus with directed therapies. extremely painful due to the hot, salty and endogenous, occur (6): urine coming in contact with the open Exogenous eczema can be either «irri- Fungal infection sores on the vulva. The diagnosis is made tant» or «allergic». Eczematous lesions Yeast organisms, especially Candida albi- by the typical appearance and confirmed are symmetric and found on areas of the cans, are normally present in most va- with a herpes immunofluorescence, anti- vulva that may contact environmental ir- ginas, but in small numbers. A yeast in- gen detection by ELISA, PCR or culture. ritants or antigens. fection, then, is not merely the presence Recurrences are common. Treatment gui- Any chronic pruritic dermatosis of the of yeast, but the concentration of them delines have recently been published (4). vulva can lead to scratching and chronic in such large numbers as to cause the Most commonly found on the labia mi- rubbing which eventually leads to the typical symptoms of itching, burning and nora, forchette, perineum, and perianal changes of the skin known as lichen sim- typical cottage-cheese appearing dis- areas, exophytic wart is one manifesta- plex chronicus. Patch testing is of limited charge. Likewise, to cure yeast infections tion of «low oncogenic risk» HPV infec- value given the nearly unlimited number means that the concentration of yeast tion. Their classic appearance is helpful of potential irritants and allergens. How- has been restored to normal and sym- for the diagnosis, however, biopsy ever, in one study, 49% of women with ptoms have resolved. The diagnosis is of- should be performed to «rule out» Vul- chronic vulvar dermatoses were patch ten made by history alone, and enhan- var intraepithelial neoplasia (VIN) or test positive for a relevant allergen (7). ced by the classical appearance of squamous carcinoma in circumstances of Any topical agents (lanolin in creams and vaginal discharge. It can be confirmed by rapid growth, increased pigmentation, ointments) as well as rubber accelerators microscopic visualization of clusters of coexisting ulceration or fixation to un- associated with condom or diaphragm thread-like, branching Candida organ- derlying connective tissue (5). Misdia- use, propylene glycol (present in KY Jelly) isms when the discharge is mixed with gnoses with condyloma latum (secon- fragrances in feminine hygiene prepara- 12 GYNÄKOLOGIE 6/2004 SCHWERPUNKT Vulvar pruritus Figure 1: Squamous cell carcinoma coexisting with lichen Figure 2: Lichen sclerosus sclerosus tions are common causes of exogenous pruritus. VIN, also previously referred to to therapy may warrant a biopsy. eczema (7). as Bowen's disease or squamous cell car- The histogenesis and pathogenesis of A large number of women behaviour cinoma in situ, as well as extramammary Paget’s disease of the vulva remain un- such as perceiving themselves unclean, Paget's disease may mimic dermatitis clear, though it is recognized as an intra- and initiating a vigorous or irritating with erythematous plaques and variable epithelial adenocarcinoma. About 5% of cleansing routine may exacerbate irritant scale. women with vulvar Paget’s disease have contact dermatitis. Feminine hygiene There are in fact two distinct types of regional or distant malignant disease products, fragrances, spray deodorants, VIN, one associated and the other not as- such as cervical adenocarcinoma, transi- and douches have many irritating ingre- sociated with HPV infection. The HPV-as- tional cell carcinoma of the urinary tract dients, such as alcohol, propylene glycol, sociated type of VIN or undifferentiated or breast carcinoma (14). or an acidic pH. VIN occurs predominantly in younger pa- Occasional cases of vulvar Paget’s dis- Endogenous eczema, also known as tients, tends to be a multicentric and ease have also been associated with vul- atopic dermatitis, may affect multiple multifocal disease and is of the undiffer- var squamous intraepithelial neoplasia sites including the vulva, and may coexist entiated Bowenoid or basaloid histologi- (VIN) 3 or invasive carcinoma (8). with asthma or allergic rhinitis. Vulval ec- cal type, while the non-HPV associated The prognosis, in terms of survival, for zema commonly presents with pruritus or differentiated VIN form generally patients with vulvar Paget’s disease with- usually exacerbating during sexual inter- found in older women, is commonly uni- out associated malignant neoplasm is course or menstruation, leading to em- focal, unicentric and usually of the dif- generally very good. However, in a pro- barrassment and interference with nor- ferentiated type (8, 9, 10). The potential portion of cases, ranging from 12% to mal life. This pathology is actually a for progression of undifferentiated VIN 3 47%, the intraepithelial lesion gives rise chronic cause of vulvar pruritus, nevert- to an invasive carcinoma has generally to an adenocarcinoma sometimes lead- heless the onset can be perceived as been thought to be low and usually esti- ing to death (15, 16). Patients with Pa- acute by both the patient and physician. mated to be no more than 3–4% (11). get’s disease associated with a regional However according to recent studies, the malignant neoplasm tend to have a poor Chronic vulvar pruritus risk of invasion is not consistently low prognosis (17). Treatment of intraepithe- and it is possible that it has been se- lial vulvar Paget’s disease is by surgical Chronic vulvar pruritus often has a long riously underestimated in the past, par- excision, but recurrence occurs in nearly history with gradual onset, and may in- ticularly in elderly women in whom pro- 40–50% of cases (18). clude a large variety of disorders like gression to an invasive lesion can occur primary inflammatory disorder, papulo- in at least 20% of VIN 3 cases (12, 13) Dermatoses squamous disorders as well as malignan- (figure 1). Lichen sclerosus is a common cause of cies, mechanical or psychogenic causes. Differentiated VIN is commonly associ- whitish lesions of the vulva. The cause of ated with lichen sclerosus and has a very this disorder is unknown, and it can oc- Malignancies strong association with invasive squa- cur at any age, although it is most com- Malignancies of the vulvar area are often mous cell carcinoma in VIN 3 cases (10). mon in the postmenopausal years. At slow growing and can cause low-grade Any apparent dermatosis not responding menarche, symptoms and signs can im- 13 GYNÄKOLOGIE 6/2004 SCHWERPUNKT Vulvar pruritus simplex chronicus may coexist with Table 1: Causes of acute vulvar pruritis chronic dermatoses such as lichen sclero- A Infectious causes sus or lichen planus. Essentially, it is an Bacterial: end-stage disorder that originates from a Staphylococcus aureus, group A Streptococcus, Mycoplasma homini, Haemophilus influen- wide number of irritative or infectious zae, Streptococcus pneumoniae; Neisseria meningitidis, Shigella, Yersinia, factors. Gardnerella vaginalis, Mobiluncus, Bacteroides Papulosquamous disorders or dermato- Trichomonas vaginalis, Neisseria gonorrheae, Chlamydia
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