265 Indications for chemotherapy 74 \200 Plateauing or rising post evacuation HCG (taken days 1, 7, 14, 21) \200 HCG \211 20,000 IU/L 4 weeks post evacuation \200 \200\200 HCG 6 months post evacuation, even if levels dropping \200 Heavy vaginal bleeding, or gastroin testinal or intraperitoneal bleeding \200 Evidence of brain, liver, or gastrointestinal m etastases, or lung opacities >2cm (smaller lesions may regress spontaneously) Histology of choriocarcinoma y g o o c e a n y G l The vulva Pruritus vulvae Vaginal itch is distressing and embarrassing. Causes: There may b e a disorder causing general pruritus (p586) or skin disease (eg psoria- sis, lichen planus ). The cause may be local: infection and vaginal discharge (eg candida); infestation (eg sc abies, pubic lice, threadworms); or vulval dystro- phy (lichen sclerosis, leukoplakia, carc inoma). Symptoms may be psychogenic in origin. Psychosexual sequelae may ensue. Obesity and incontinence exacer- bate symptoms. Postmenopausal atrophy does not cause itch. 266 \200 The history may suggest the cause. Ask about autoimmune disorders and atopy. Examine genera l health and look for wider spread skin conditions. Ex- amine the vulva and genital tract, under magni \201 cation if possible, and take a cervical smear, if due. Consider taking va ginal and vulval swabs and tests for diabetes and thyroid disease. If vulval dermatitis che ck serum ferritin and dermatology patch tests. Biopsy if diagnosis in doubt, if there is no response to treatment, or vulval intraepithelial neoplasia or carcinoma are suspected. \200\207Scratching and self-medication may have changed the appearance. Treatment is often unsatisfactory.1 Treat the cause if possible. Avoid senstitiz- ers (patch testing may revea l sensitizing agents eg 26 \200\22380% in vulval dermati- tis studies). Reassurance can be very important. Vulval care advice (OPPOSITE), may help. A short course of topical steroids , eg betamethasone valerate cream 0.1% may help. Avoid any topical preparation which may se nsitize the skin, so give antipruritics orally if needed, eg promethazine 25mg/12 \200\2232 4h. Lichen sclerosis Thought to be an autoimmune disorder (40% develop other autoimmune dis orders), elastic tissue turns to collagen (usually after middle age \200\224or, occasionall y, before puberty). The \200\230bruised \200\231 red, purpuric signs may appear, to the un knowing, to suggest abuse \200\224particularly if there are bullae, erosions, and ulceratio ns. The vulva gradually becomes white, \202 at, and shiny. There may be an hourglass shap e around the vulva and anus. It is intensely itchy. It may be pre-malignant and long-term s urveillance is desirable if un- responsive to treatment. Treatment: Clobetasol proprionate cream daily for 28 days, then alternate days for 14 doses, then twice weekly for 8 doses, t hen as needed; 75 vulval ablation may be needed to relieve itch. The 4 \200\22310% who are steroid unresponsive may respond to topical tacrolimus (off license, use in specialist clin ic only, for <2 years). In children, 50% resolve by menarche. Leukoplakia (White vulval pat ches due to skin thickening and hypertrophy). It is itchy. It should be biopsied as it may be a pre-malignant lesion. Treat- ment: Topical corticosteroids (problems: mucosal thinning , absorption); pso- ralens with ultraviolet phototherapy; methotrexate; ciclosporin. Lichen planus Of unknown cause, this is more likely to present with pain than pruritus. In mouth & genital area it can be erosive, appearing with a well de- marcated glazed appearance arou nd the introitus. It can aff ect all ages. Lichen simplexThis presents with chronic intract able itching, especially at night, in those with sensitive skin or eczema. There is non-spe ci \201 c in \202 amma- tion of vulva, ( mons pubis and inner thighs). Stress, sensitiz ing chemicals an low body iron stores can exacerbate symptoms. Treatment is with vulval car e (OPPOSITE), using steroids to break the itch/scratch cycle, if needed. Antihista- mines o r antipruritics (above) can help. Vulvovaginitis Think of \201 xed drug reactions (NSAIDs , statins); stop for 2 weeks. Desquamative in \202 ammatory vaginitis, of unknown cause, c haracter- ized by shiny erythematous patches petechiae. It responds to 2 \200\2234 weeks of intravaginal clindamycin cream with hydrocortisone to the vulva. 76 y g o o c e a n y G l 1 As chronic vulval itch and chronic vulval pain are often recalcitrant (often ignored, or inappropriately diagnosed as candida) \200\224and because of its eff ects on mood and sexua lity, a patients \200\231 group (with profes- sional input) has been set up in the UK (www. vul-pain.dircon.co.uk). 267 Vulval intraepithelial neoplasia Vulval malignancy has a pre-invasive phase, vulval intra-epithelial neoplasia (VIN, \201 g 1, note white areas with surround- ing in \202 ammation). It may be itchy. Cause: often HPV, p269 (esp. HPV16); there may not be visible warts but 5% acetic acid stains aff ected areas white. If VIN is found on bi- opsy, examine cervix, anal canal if within 1.5cm, \200 \22577 natal cleft skin and breasts (>10% have coexistent neoplasia elsewhere, most commonl y cervical). Treating VIN Wide local excision is gold standard treatment of choice. \200 \22578 Women should have access to reconstructive surgery afterwards, if needed. Histology reveals 12 \200\22317% unrec- ognized invasion in wide excision samples. There is higher re sponse rates in women undergoing excision than in those under- going medical treatment. Rec urrences are fewer if excision margins clear. Medical treatments have used 5% imiqui- mod c ream with regression of grade 2 \200\2233 disease in 77%. 79 Cidofovir use is being studied . Laser therapy is useful for areas where surgery is contrain- dicated (eg in glans and cli toral hood). Laser treatment failures are 40%. Therapeutic use of human papilloma virus va ccine, photodynamic therapy, interferon use, and cavitron ultrasonic surgical aspiration te chniques have been tried but none are currently recommended treatments. \200\22578 Recurre nce is common so follow-up regularly. y g o o c e a n y G l Fig 1. Vulval intra-epithelial neopla- sia (VIN). Vulval care for those with vulval disorders \200\22578 \200 Use soap substitute with wat er for washing (less drying than water alone). \200 Shower, bath (with emollient), or cle an vulva once daily only. \200 Wash vulva with hand (not sponge/ \202 annel); dab dry or blow dry with hair- dryer on cool setting held well away from the skin. Wear loose \201 tting silk or cotton white or light colored underwear (blue/ black dyes can be irritant). Sleep without underwear. Avoid tight jeans/cycling trousers but wear loose trousers, dresses or skirts. At home wear ing skirts without underwear may be more comfort- able. Avoid soap, bubble bath, shower gels, biological washing powders, fabric conditioners, vulv al creams or douches, antiseptics, regular sanitary towel or panty liner wear, baby wipe us e, colored toilet paper, nail varnish. \200 Regular emollient use (throughout day) can so othe and reduce \202 are ups. \200 For irritated skin, dabbings of aqueous cream kept i n the fridge can soothe. Vulval lumps and ulcers Causes of vulval lumps Local varicose veins; boils; sebaceous cysts ; keratoa- canthomata (rare); viral warts (condylomata acuminata); condylomata lata (syphil is); primary chancre; molluscum contagiosum; Bartholin \200\231s cyst or ab- scess; uterine prolapse or polyp; inguinal hernia; varicocele; carcinoma. Vulval warts Human papilloma vi rus (HPV) \200\224is usually spread by sexual contact. Incubation: weeks. Her partner may n ot have obvious penile warts. The vulva, perineum, anus, vagina, or cervix may be aff ected . Warts may be very \202 orid in the pregnant and immunosuppressed. HPV types 16, 18, and 33 can cause vulval and cervical intra-epithelial neoplasia, so she needs annual cervical smears and observation of the vulva. Warts may also cause anal car- cinoma (OHCM p633). Tre at both partners. Exclude other genital infections. Warts may be destroyed by diathermy, cr yocautery or laser. Vulval and anal warts (condylomata acuminata) may be treated weekly in surgeries and GU clinics with 15% podophyllin paint, washed off after 30min (CI: pregnancy) . Only treat a few warts at once, to avoid toxicity. Self-application with 0.15% podophyllo toxin cream (Warticon 5g tubes \200\224enough for 4 treatment cours- es \200\224is suppli ed with a mirror): use every 12h for 3 days, repeated up to 4 times at weekly intervals if the area covered is <4cm2. Relapse is common. HPV im- munization and cervical cancer: See p 272. NB: HPV types 6 and 11 may cause laryngeal or respiratory papillomas in the off spring of aff ected mothers (risk 1 : 50 \200\2231 : 1500; 50% present at <5yrs old). Any warty l esion in a post-menopau- sal woman should be biopsied to exclude vulval cancer. \200\22580 Exclude other genital infections. Treat both partners. Urethral caruncle This is a small r ed swelling at the urethral ori \201 ce. It is caused by meatal prolapse. It may be tender and give pain on micturition. Treatment: Excision or diathermy. Bartholin \200\231s cyst a nd abscess The Bartholin \200\231s glands and ducts lie under the labia minora. They secret e thin lubricating mucus during sexual excitation. If the duct blocks a painless cyst forms ; if this becomes infected the resulting abscess is extremely painful (she cannot sit down) and a hugely swollen, hot red labium is seen. Treatment: The abscess should be incised, an d permanent drainage ensured by marsupialization, ie inner cyst wall is folded back and sti tched to the skin, or by balloon catheter insertion. \200 81 Tests: Exclude gono- coccus. Vulvitis Vulval in \202 ammation may be due to infections, eg candida (p284), her- pes sim plex; chemicals (bubble-baths, detergents). It is often associated with, or may be due to, vaginal discharge. Causes of vulval ulcers: Always consider syphilis. Herpes simplex is com mon in the young. Others: carcinoma; chancroid; lymphogranuloma venereum; granuloma inguina le; TB; Beh et \200\231s syndrome; aphthous ulcers; Crohn \200\231s disease. Herpes simple x Herpes type II, sexually acquired, classically causes genital infection, but type I trans ferred from cold sores can be the cause. The vulva is ulcerated and exquisitely painful. Ur inary retention may occur. Treatment: Strong analgesia, lidocaine gel 2%, salt baths (and m icturating in the bath) help. Exclude coexistent infections. Aciclovir topically and orally shortens symptoms and infectivity. Oral dose: 200mg 5 times daily or 400mg/8h for 5 days ( longer if new lesions appear during treatment or if healing is incom- plete). If immunocomp romized/HIV+ve: 400mg 5 times daily for 7 \200\22310 days dur- ing 1st episode or 400mg/8h for 5 \200\22310 days during recurrent infection. 268 y g o o c e a n y G l Reassure that subsequent attacks are shorter and less painful. Prescribe ac- iclovir cream for use when symptoms start. For herpes in pregnancy, see p36. Carcinoma of the vulva \200\207Refer unexplained vulval lumps urgently. 90% are squamous. Others are melanoma, basal cell carcinomas or carcino- ma of Bartholin \200\231s glands. Th ey are rare and usually occur in the elderly (age 70 \200\22380). 269 Presentation may be as a lump; as an indurated ulcer which may not be noticed unless it cau ses pain and bleeding hence often presenting late (50% already have inguinal lymph node inv olvement). There may be a pre-invasive phase as VIN (for explanation and treatment see p267 ). Treatment 80 If tumor <2cm width and <1mm deep node excision is not needed. If >1mm deep do \200\230triple incision surgery \200\231 =wide (>15mm margin) local excision + ipsilat eral groin node biopsy (and, if aff ected, sample contralateral side too). More advanced di sease may need radical vulvectomy (wide excision of the vulva + removal of inguinal glands) . Skin grafts may be needed. Radio- therapy may be used pre-op to shrink tumors if sphincte rs may be aff ected. Chemoradiation is used if unsuitable for surgery, to shrink large tumo rs pre- operatively and for relapses. 5-yr survival is >80% for lesions <2cm with no node i nvolvement; otherwise <50%. y g o o c e a n y G l