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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 , 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 induced 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 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 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 . 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-

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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- , 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-

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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 trachomatis ses such as seborrheic dermatitis, psoria- sis and atopic dermatitis rarely present Fungal: with genital features alone. A history and Candidiasis, Tinea cruris a full skin exam should reveal some other Infestation: signs of these conditions. Scabies, Pediculosis pubis, Pinworms Finally, an increased tendency for depres- sive and obsessive-compulsive traits has Viral: been identified in patients with chronic Herpes simplex virus, Herpes zoster, Human papillomavirus, Molluscum contagiosum pruritic conditions (22, 23). Whether this B Non-infectious causes represents a cause or effect is unclear. It Contact dermatitis has been shown that depression corre- Irritant, allergic lates with the degree of symptomatic pruritus in patients with chronic pruritic dermatological disease. Table 2: Causes of chronic vulvar pruritis

A Malignancies Conclusion Extramammary Paget's disease, Vulvar intraepithelial neoplasia In conclusion, management of vulvar B Dermatoses pruritus is difficult. A detailed history is Seborrheic dermatitis, Psoriasis, Atopic dermatitis, Lichen sclerosus, Lichen planus important, thorough examination and C Atrophic vulvovaginitis biopsies of the vulva are essential to reach a diagnosis. Better knowledge of Diabetic patient, obese patient, inadequate hygiene, these diseases can be achieved by refer- psychogenic: Depression, Idiopathic pruritus () ring the patients to second opinion. ◗

Dr. med. Anne-Thérèse Vlastos prove spontaneously, but it is not known nating in the vestibule and variably ex- Département de Gynécologie et whether there is long-term risk of recur- tending up the vaginal canal. Obstétrique rence. Similar to lichen sclerosus, lichen planus Bd. de la Cluse 30 Pruritus is quite characteristic of lichen may be associated with an increased risk Hôpitaux universitaires de Genève sclerosous although it can be asympto- of malignancy (20). (HUG) matic in its early stages (figure 2). There Any times, no etiology is identified and E-Mail: [email protected] may be loss of the normal architecture examination reveals either normal skin, with atrophy of the labia minora, con- or lichenification, lichen simplex chroni- striction of the vaginal orifice (craurosis), cus or idiopathic/essential pruritus can References: synechiae development, ecchymoses, fis- be suspected. Lichenified skin is mildly 1. Bornstein, J., Pascal, B., Abramovici, H.: The common problem of vulvar pruritus. Obstetrical & sures, and telangiectases (19). A clear scaly, rather leathery and has increased Gynecological Survey 1993; 48 (2): 111–118. risk of association with vulvar puritus is skin markings. At times, the skin may, in 2. Hatch, K.D.: Vulvar and vaginal disorders. Cur- well documented (12, 13). fact, appear completely normal. Sym- rent Opinion in Obstetrics & Gynecology 1992; Two types of Lichen planus are known: ptoms of itching and burning predomi- 4(6): 904-906. 1. the «classic» type consisting of nate. A number of acute disorders may 3. Edwards, L.: The diagnosis and treatment of in- fectious . Dermatologic Therapy 2004; 17 sharply demarcated, flat-topped pla- evolve into lichen simplex chronicus in- (1): 102–110. ques on oral and genital membranes cluding recurrent vaginal infections and 4. Buchner, S., et al.: Swiss recommendations for and, long-standing eczema. Nighttimes itch is the management of genital herpes and herpes 2. The «erosive» type consisting of an a common feature and patients maybe simplex virus infection in the neonate. Swiss Med erosive, erythematous lesion origi- unaware of this behaviour (21). Lichen WKL. 2004; 134: 205–214.

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5. Moscicki, A.B., et al.: Risks for incident human vulva: 24 years' experience in southwest Florida. Cancer Institute experience with extramammary papillomavirus infection and low-grade squa- Southern Medical Journal 1988; 81 (5): 589–593. Paget's disease. British Journal of Dermatology mous intraepithelial lesion development in young 12. Crum, C.P.: Carcinoma of the vulva: epidemio- 2000; 142 (1): 59–65. females. JAMA 2001; 285 (23): 2995–3002. logy and pathogenesis. International Journal of 19. Carlson, J.A., et al.: Vulvar lichen sclerosus 6. Salim, A., Wojnarowska, F.: Skin diseases af- Radiation Oncology, Biology, Physics 1992; 22 (3): and squamous cell carcinoma: a cohort, case fecting the vulva. Current Obstetrics & Gyne- 525-527. control, and investigational study with historical cology 2002; 12: 81–89. 13. Thuis, Y.N., et al.: Contemporary experience of perspective; implications for chronic inflamma- 7. Lewis, F.M., Shah, M., Gawkrodger, D.J.: the management of vulvar intraepithelial neo- tion and sclerosis in the development of neo- Contact sensitivity in pruritus vulvae: patch test plasia. Int J Gynecol Cancer 2000; 10: 223–227. plasia. Human Pathology 1998; 29 (9): 932–48. results and clinical outcome. American Journal 14. Fishman, D.A.: Extramammary Paget's dis- 20. Jones, R.W., et al.: Vulvar lichen planus: pro- of Contact Dermatitis 1997; 8(3): 137–140. ease of the vulva. Gynecologic Oncology 1995; 56 gression of pseudoepitheliomatous hyperplasia 8. Hart, W.R.: Vulvar intraepithelial neoplasia: (2): 266–270. to invasive vulvar carcinomas. BJOG: an Interna- historical aspects and current status. Internatio- 15. Fanning, J., et al.: Paget's disease of the tional Journal of Obstetrics & Gynaecology 2001; nal Journal of Gynecological Pathology 2001; vulva: prevalence of associated vulvar adenocar- 108 (6): 665–666. 20(1): 16–30. cinoma, invasive Paget's disease, and recur- 21. Virgili, A., Bacilieri, S., Corazza, M.: Managing 9. Haefner, H.K., et al.: Vulvar intraepithelial neo- rence after surgical excision. American Journal vulvar lichen simplex chronicus. Journal of Re- plasia: age, morphological phenotype, papilloma- of Obstetrics & Gynecology 1999; 180: 24–27. productive Medicine 2001; 46 (4): 343–46. virus DNA, and coexisting invasive carcinoma. 16. Crawford, D., et al.: Prognostic factors in Pa- 22. Sheenhan-Dare, R.A., et al.: Anxiety and de- Human Pathology 1995; 26(2): 147–154. get's disease of the vulva: a study of 21 cases. In- pression in patients with chronic urticaria and 10. Yang, B., Hart, W.R.: Vulvar intraepithelial neo- ternational Journal of Gynecological Pathology generalized pruritus. Br J Dermatol 1990 (123): plasia of the simplex (differentiated) type: a clini- 1999; 18 (4): 351–359. 769–774. copathologic study including analysis of HPV and 17. Parker, L.P.: Paget's disease of the vulva: pa- 23. Hatch, M.L., Paradis C., and Friedman S.: Ob- p53 expression. American Journal of Surgical Pa- thology, pattern of involvement, and prognosis. sessive-compulsive disorder in patients with thology 2000; 24 (3): 429–41. Gynecologic Oncology 2000; 77 (1): 183–89. chronic pruritic conditions. Case studies and dis- 11. Fiorica, J.V., et al.: Carcinoma in situ of the 18. Zollo, J.D., Zeitouni N.C., The Roswell Park cussion. J Am Acad Dermatol 1992; 26: 549–551.

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Solidarität gegen Brustkrebs

Die Diagnose Brustkrebs ist ein Schock zerner Solidaritätswanderung» einen lungen, die nicht nur die Heilungschancen für jährlich etwa 4500 betroffene geselligen Abend mit Schifffahrt auf dem erhöhen, sondern auch weniger belastend Frauen in der Schweiz. Die Angst vor Vierwaldstättersee, Kurzspielfilm und als ältere Methoden sind. Dazu gehören dem Verlust der Brust, vor Haarausfall Benefizkonzert. Der Reinerlös wird dem brusterhaltende Tumoroperationen und und weiteren Nebenwirkungen der psycho-onkologischen Dienst der Krebs- nebenwirkungsärmere Chemotherapien. Chemotherapie wird ständiger Beglei- liga Zentralschweiz zugute kommen. Fast Chemotherapien mit modernsten Brust- ter. Dabei fürchten die Frauen um die 170 Personen nahmen teil und unterstütz- krebsmedikamenten, etwa dem eingekap- Beziehung, die Familie, ihr Überleben en den guten Zweck. Nicht zuletzt dank selten Doxorubicin Caelyx, verursachen und fühlen sich mit ihren Ängsten des Hauptsponsors Essex Chemie können heute bei maximaler Wirksamkeit deutlich meist alleingelassen. Der Arzt kann der Krebsliga Zentralschweiz 12 000 Fran- weniger Nebenwirkungen als herkömmli- psychologisch oft zu wenig helfen, die ken zur Verfügung gestellt werden. che Behandlungen. Studien belegen, dass Familie, die Freunde und der Partner Wie gross die psychische Belastung von Haarausfall, Übelkeit und Erbrechen we- sind überfordert. Hier setzt der neue Brustkrebspatientinnen ist, zeigt der Kurz- sentlich seltener auftreten. Dennoch brau- psycho-onkologische Dienst der Krebs- spielfilm «Busenfreundinnen» von Gabri- chen Brustkrebspatientinnen dringend die liga Zentralschweiz an: Ab Januar 2005 ele Schärer. Einfühlsam und eindringlich Hilfe, die ihnen der neue psycho-onkolo- wird die Psycho-Onkologin Carmen thematisiert der Film die Sprachlosigkeit gische Dienst in Luzern geben kann. ◗ Schürer betroffenen Frauen mit Rat und Ängste von Brustkrebspatientinnen und Tat zu Seite stehen. und mündet in betroffenem Schweigen. Die elektronische Version dieser Pressemittei- Dabei gibt es, wie Professor Dr. Rudolf A. lung finden Sie unter: www.schlegelpharma.ch/brustkrebs/ Um dem neuen Projekt eine kleine Joss, Chefarzt Onkologie am Kantonsspi- (Benutzer: presse, Passwort: presse) Starthilfe zu geben, organisierte die «Lu- tal Luzern, betont, medizinische Entwick-

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