The correct answer is 5. Vulvar carcinoma is characterized by chronic vulvar itching resistant to multiple treatments, appearance of lumpiness or ulceration, dysuria and urinary urgency, and late pain and bleeding.

[HIDDEN] Vulvar carcinoma is characterized by chronic vulvar itching resistant to multiple treatments, appearance of lumpiness or ulceration, dysuria and urinary urgency, and late pain and bleeding.

Patient 76 years old, parity 3-0-3-1, with menopause at age 52. She reports that for at least 4-5 years she has had vulvar pruritus of variable intensity which has been treated sometimes with self-medication and other times on the advice of her general practitioner with topical preparations (creams and washes). The pruritus has evolved intermittently, but for the last 3-4 months she has also noticed a small lump on the left labium majus of the vulva, which has been leaking serohaematically on rubbing for a few days; she therefore consulted a gynecologist. On questioning, she reports occasional dysuria and her general condition is good. What is the most likely diagnosis in this patient?

Unusual locations of primary subepithelial squamous cell carcinomas of the vulva.

This study aimed to report 2 cases of squamous cell carcinoma (SCC) of the vulva, arising from unusual subepithelial locations. The first case was of an 85-year-old woman with a 65-year history of a mass in the left labium majus. The second case was of a 54-year-old woman who presented with a 1-week history of a painful left inguinal mass. She had previously sought medical attention for a 2-year history of a left-sided painless vestibular mass. In the first case, a simple excision showed a purely dermal SCC with no attachment to the epidermis. After the diagnosis of SCC, a wide, deep local excision of the left side of the vulva and left inguinofemoral lymph node dissection were performed. Pathological findings showed no residual tumor in the vulva, and the lymph nodes were clear. Based on the long history of a mass at the same site, the pathogenesis of the SCC was considered to be malignant degeneration of a previously benign epidermal cyst.In the second case, SCC was diagnosed on fine-needle aspirations of the vulvar and groin masses. The patient was treated with primary chemoradiation. Subsequently, wide, deep local excision of the left side of the vulva and left inguinofemoral lymph node dissection were performed. No residual tumor was found in the vulva, although atrophic Bartholin gland tissue was found at the site of the SCC. One 5-mm inguinofemoral lymph node metastasis was found in the groin node dissection. In the absence of any evidence of another pathogenesis, it was believed that the SCC had arisen from an unusually anterior located Bartholin gland. Subepithelial SCC should be considered in the differential diagnosis of unusually located vulvar masses.

A case of usual (basaloid)-type vulvar intraepithelial neoplasia that failed to respond to imiquimod cream: clinical implications.

The authors report a case of usual-type (basaloid-type) vulvar intraepithelial neoplasia (VIN) 3 that failed to respond to imiquimod cream. A 51-year-old Japanese woman visited her local gynecologist complaining of vulvar itching. Atypical cells were noted in cytology smears, but nine vulvar biopsy specimens showed benign proliferation of epithelial tissue. The patient was placed under careful observation for 8 months, when the vulvar smears once again showed atypical cells and biopsy specimens revealed VIN3. The patient was then referred to our hospital where she was given a diagnosis of VIN 3, basaloid type of usual type. The biopsy specimens were positive for p16 and the lesions were confirmed to be human papilloma virus (HPV)-related. We recommended simple vulvectomy but the patient requested conservative treatment with imiquimod cream. With her written informed consent, we prescribed imiquimod cream to be self-administered 3 times a week. Colposcopy and pap smear test were performed every 2 weeks. Four weeks after the start of treatment, a fingertip-sized papule was detected at the patient's vaginal introitus. By 6 weeks, the lesion had enlarged, and biopsy specimens revealed invasive squamous cell carcinoma. At 7 weeks, we performed simple vulvectomy. The surgical specimen showed stage pT1b keratinizing-type squamous cell carcinoma. HPV-16 DNA was detected in the specimen.

Concurrence of Primary Cutaneous Extra Mammary Paget's Disease and Squamous Cell Carcinoma in situ of Vulva: A Case Report.

Extramammary Paget's disease and vulvar intraepithelial neoplasia are common lesions of vulve, but synchronous occurrence is rare in a same location. Herein we describe a concurrence of primary cutaneous extra mammary Paget's disease and squamous cell carcinoma in situ of vulva in an Iranian women. A 59 year old woman, initially presented to Kosar teaching hospital gynecology clinic April 2017 with a single, well defined, scaly, white ulcerated mass, 2 cm in diameter on right minor labia, but other examinations were normal. She had no urinary or gastrointestinal symptoms. Incisional biopsies from the mass represented concurrence extramammary Paget's disease and VIN3. She underwent radical vulvectomy .No evidence of disease recurrence was noted after 16 months follow up. Participant consent was obtained before patient was enrolled in this study. There are lot of diseases that involve genitalia and lead to vulvar lesions. But in this rare case, we diagnosed concurrence of extramammary Paget's disease and vulvar intraepithelial neoplasia in a same location. Additionally, the clinical presentation as a vulvar mass was found unusual. Therefore, we report the case to sensitize gynecologists and pathologists for uncommon pathologies and their manifestations in vulva.

Vulvodynia

It is estimated that among sufferers, only half will seek medical help, among whom many will see several doctors before a correct diagnosis is made. Only 2% of the people that seek help do obtain a diagnostic. Many gynecologists are not familiar with this family of conditions, but awareness has spread with time. Sufferers are also often hesitant to seek treatment for chronic vulvar pain, especially since many women begin experiencing symptoms around the same time they become sexually active. Moreover, the absence of any visible symptoms means that before being successfully diagnosed many patients have been told that the pain is "in their head" (a medical stance more associated with the 1970s than with modern medicine). Differential diagnosis Infections: candidiasis, herpes, HPV Inflammation: lichen planus Neoplasm: Paget's disease, vulvar carcinoma Neurologic disorder: neuralgia secondary to herpes virus, spinal nerve injury

Ulcus vulvae acutum in a 13-year-old girl after influenza A infection.

A 13-year-old otherwise healthy premenarchal girl presented with acute onset of painful vulvar ulcerations. One day before developing vulvar ulcerations, she experienced flu-like symptoms, including a low-grade fever, cough, sore throat, and myalgia. Results of a throat swab were positive for influenza A infection (polymerase chain reaction [PCR] assay), and the patient was treated with oseltamivir. The patient's constitutional symptoms improved slightly, but within 2 days after her initial presentation, she returned to her primary care provider and described 24 hours of dysuria and vulvar swelling. She had a history of herpes labialis (cold sores) and rare episodes of minor oral aphthae (canker sores) that occurred less than twice a year. The patient denied a history of sexual activity, sexual abuse, or physical trauma. Physical examination showed ulceration and swelling of the labia minora, and the patient received an empiric dose of acyclovir (200 mg 4 times daily) for presumed autoinoculated herpes simplex virus (HSV) infection. An ulcer swab was performed, and urinalysis revealed no evidence of infection. Two days later, the patient presented to the emergency department with increasing vulvar pain and vaginal discharge. The previous ulcer swab findings were negative for HSV (PCR assay), and consequently, acyclovir was discontinued after 1 day of therapy. She received topical viscous lidocaine and an empiric dose of oral fluconazole. The lidocaine provided temporary symptomatic relief. Results of DNA amplification studies were negative for Chlamydia trachomatis and Neisseria gonorrhoeae. A potassium hydroxide preparation was negative for fungi, and an ulcer swab for bacterial culture revealed usual flora. Of note, the PCR assay for Epstein-Barr virus was not performed on ulcer cells. The patient was referred to the department of dermatology, and results of a physical examination showed copious white mucoid discharge and a 2-cm ulceration of the left labia minora (Figure, panel A). Two smaller pinpoint ulcerations and swelling of the left labia minora were also noted. The lesions were clinically indistinguishable from the genital aphthous ulcers of patients with complex aphthosis (recurrent, severe aphthous ulcers on oral or genital mucosa). A diagnosis of ulcus vulvae acutum was made, and treatment was started with clobetasol 0.05% ointment (4 times daily) and lidocaine gel as needed. Four days later, the patient reported marked symptomatic improvement. Physical examination showed near resolution of the large vulvar ulceration (Figure, panel B). The patient tapered use of clobetasol ointment over the next several days until the ulcerations healed completely. Two months after her initial episode, the patient again had 3 small vulvar erosions after symptoms that included low-grade fever, malaise, and vomiting. She did not receive oseltamivir for this illness; clobetasol ointment was applied 4 times daily, and the vulvar erosions ameliorated within a few days. Her constitutional symptoms resolved without treatment. The patient has not experienced any further episodes of vulvar ulcerations in the 18 months after the most recent treatment.

Dermatological conditions of the vulva.

Management of vulval conditions in the elderly will present no special problems if the following points are borne in mind: 1. The patient's condition will often be multifactorial; the components should be unravelled and the patient followed until the picture is clear. 2. Point 1 is particularly important because infections and neoplasia can easily go unrecognized. 3. Biopsy will often be needed. 4. Management of chronic dermatological conditions (especially where systemic treatment is indicated) is usually best carried out by the dermatologist. 5. It is vital to consider the area as one would any other as regards diagnosis of cutaneous and mucosal lesions. With this approach the vast majority of lesions can be accurately named and reasonable treatment given. To this end the naked-eye morphology, and the histopathological evidence which will sometimes be a necessary supplement, must be accurately described and understood. 6. The study of vulval conditions is interdisciplinary. 7. Much old and contentious terminology is now obsolete. The new classification is simple and should be universally applied by gynaecologists, dermatologists and pathologists so that other involved clinicians are helped rather than confused.

Vulvar Langerhans cell histiocytosis: a case report and review of the literature.

Langerhans cell histiocytosis (LCH) of the female genital tract is rare. Only, seven cases of primary vulvar LCH have been previously reported in the medical literature. We describe an additional case of LCH in which the disease was confined to the vulva. A 33-year-old gravida 0, para 0 Ethiopian woman presented with a nodular lesion on her left vulva. The lesion was biopsied, and the results were consistent with LCH. A metastatic workup did not reveal any evidence of disease beyond the vulva. The patient was initially treated with radiotherapy to the vulva. She was diagnosed with recurrent disease in the vulva 21 months after the completion of radiotherapy. At that time, she underwent a wide local excision. Five months later, we found a lesion on her right labium majus that was consistent with a recurrence. The patient's vulva was treated with a higher dose of radiotherapy than it had been the first time. Six months later the patient again experienced a local recurrence. She underwent a wide radical vulvar excision of diffuse bilateral lesions and was free of disease for approximately 3 months, after which she experienced another recurrence and underwent treatment with thalidomide. Within 2 months of starting thalidomide therapy, the patient experienced resolution of her symptoms and of her vulvar lesions. Primary LCH of the vulva is very rare. Its etiology and pathophysiology, as well as the most effective modes of therapy, remain elusive. We propose that thalidomide is a useful alternative for patients with this disease.

Effectiveness of treating non-specific pruritus vulvae with topical steroids: a randomized controlled trial.

To determine the effectiveness of triamcinolone cream in the treatment of non-specific pruritus vulvae. A total of 50 patients, aged between 8 and 55 years, with non-specific pruritus vulvae were consecutively included in a double-blind randomized controlled trial by their general practitioner in 25 general practices in the Netherlands. Analysis was by "intention to treat". An infectious cause was excluded by a vaginal and cervical swab. After randomization, 27 patients were treated with triamcinolone cream and 23 patients with a placebo cream. The effectiveness was assessed by: change in the severity of itch visual analogue scale (VAS) score; the percentage of patients with complete recovery; and changes in general health according to COOP/WONCA charts. The mean decrease in severity of itch was 2.08 points in the triamcinolone group compared to 3.26 points in the placebo group. Forty-two per cent of the women in the triamcinolone group completely recovered versus 35% in the placebo group. There was no difference in general health perception between the two groups. After 12 weeks, more than 80% in both groups reported recurrent vulval itch. No significant differences in effectiveness were found between the two groups, because both treatments were ineffective. We can conclude that triamcinolone cream is therefore not the treatment of first choice for non-specific pruritus vulvae.

Oral and Vulvar Lichen Sclerosus.

Lichen sclerosus (LS) is a chronic, inflammatory, mucocutaneous disorder of genital and extragenital skin (1). Simultaneous involvement of the oral mucosa is extremely rare, but it may be the only affected area (2). A 55-year-old woman was referred to the Department of Oral Medicine, School of Dental Medicine University of Zagreb due to whitish lesions on the right ventrolateral part of the tongue and buccal mucosa with desquamative gingivitis (Figure 1, a-c). The lesions were asymptomatic but indurated on palpation. Histology was conclusive for oral lichen sclerosus (OLS). The lesions on gingiva were successfully treated with betamethasone ointment, three times a day for two weeks. One year earlier, she had been referred to the Department of Dermatology and Venereology with progressive pruritus and dyspareunia, white patches, obliteration of the labia minora, and stenosis of the introitus (Figure 2). Histology was conclusive for vulvar LS (Figure 3, a and b). She was successfully treated for 5 months with clobetasol propionate 0.05% ointment. The patient was taking levothyroxine to treat hypothyroidism associated with Hashimoto's thyroiditis and was otherwise healthy. Oral LS is clinically characterized by the appearance of white macules, papules, or plaques mostly appearing on labial mucosa but also on buccal, palate mucosa and on the lower lip (2,3). On the genitals, it typically manifests as atrophic white plaques, which may be accompanied by purpura or fissuring (1). While vulvar LS is often associated with pruritus, dyspareunia, and dysuria, OLS is often asymptomatic, although pain, soreness, pruritus, and tightness when opening the mouth can be present (1,2). Oral manifestations of LS, as well as association of anogenital and oral LS, are rarely reported in the literature (4-6). Tomo et al. searched the Medline database for papers reporting oral LS cases with histological diagnosis confirmation from 1957 to 2016 and found only 34 cases of oral LS with histopathologic confirmation of the diagnosis (4). Kakko et al. reported 39 histologically proven cases of OLS (2). Attilli et al. (5) reviewed the clinical and histologic features of 72 cases of LS with oral/genital involvement. They reported that LS was diagnosed with exclusive genital lesions in 45, exclusive lip involvement in 20, and orogenital involvement in only 7 cases (5). Some believe that many cases of clinically diagnosed lichen planus may actually be LS and that isolated oral mucosal LS may not be as rare as is generally thought (2). While vulvar LS can occur at any age with increasing incidence with age, the median age of patients with OLS was 34 years and most of the patients were female (1,2,5). Due to the small number of patients in the literature, treatment recommendations for OLS are not available. In case of symptomatic oral lesions, topical or intralesional corticosteroids are considered to be the first-line treatment (2). First-line treatment for anogenital LS is a potent to very potent topical corticosteroid ointment, and second-line therapies include topical calcineurin inhibitors 1% pimecrolimus and 0.1% and 0.03% tacrolimus (1). For treatment-resistant genital LS, oral retinoids, methotrexate, and possibly local steroid injections for single lesions are mainly applicable for women (1). There is limited evidence for systemic treatments for both conditions. If it is not treated, genital LS is associated with a greater degree of scarring and an elevated risk of progression to squamous cell cancer; however, malignant transformation of OLS has not been reported (1-6). Due to the very rare presentation in the oral cavity, it is important to notice these lesions during a dental exam.

[The vulvar clinic].

Until recently, vulvar diseases have not been given sufficient attention by gynecologists. This might be attributable to the timidity of patients with chronic vulvar pruritus, tumor or dyspareunia, which delays diagnosis and treatment. In addition, the nomenclature of vulvar disease was complicated by having been adapted from various dermatological disorders. Only following the establishment of the International Society for the Study of Vulvar Disease (ISSVD) and of specialized clinics, has awareness increased and more attention is now being paid to vulvar disorders. The findings in 242 patients seen during the first year of operation of a vulvar clinic were analyzed. The most common presenting symptom was chronic vulvar pruritus. Non-neoplastic epithelial disorder (dystrophy) was the most common histological finding in the 79 women with pruritus, while 2 were diagnosed as having vulvar intraepithelial neoplasia, and 1 had basal cell carcinoma. In contrast, vulvar vestibulitis was the main diagnosis in 34 women referred for vulvar pain (vulvodynia). Vulvodynia was also associated with other inflammatory processes and with human papilloma virus (HPV) lesions (condylomata). The primary finding in 26 women referred for evaluation of a vulvar "tumor" was epidermal cyst. In only 55 (75.5%) of the 73 referred for suspected HPV lesions was the histologic diagnosis confirmatory. In addition, a few vulvar ulcers were associated with HPV. A single treatment with carbon dioxide laser eradicated the HPV lesions in 93% of the cases. The vulvar clinic contributes to the ambulatory gynecological service by concentrating diagnosis and treatment in a single specialized unit and increases understanding of, and interest in vulvar disease.

Treatment of recurrent vulvar Paget disease with imiquimod cream: a case report and review of the literature.

Extra-mammary Paget disease is one of the rare neoplastic conditions of the skin. The most common site of involvement is the vulva and presents itself with erythematous plaques. Surgery is the most important treatment option. In the recent years, there are publications of the topical use of imiquimod cream in extra-mammary cutaneous Paget disease. We report the case of a woman with recurrent vulvar Paget disease, who underwent successful treatment with imiquimod cream. We also review the reports of other patients with vulvar Paget disease who were treated with topical imiquimod cream. A 65-year-old woman presented to the Oncology Outpatient Clinic with an itchy lesion in her vulva for 2 years. In the gynecologic examination, a hyperkeratotic erythematous lesion was found starting from the right labium to involve clitoris, with a size of 4 × 3 cm. Pathology result was reported as Paget disease. She was operated and wide vulvar excision was performed with a safety margin of 2 cm. Then recurrence two times occurred. Because she refused surgery, imiquimod cream 5% was applied for treatment. Imiquimod cream is an effective and safe therapeutic agent for both primary and recurring vulvar Paget disease.

Gynecology_Novak

When vulvar cancer arises in the presence of VIN, vulvar dystrophy, or some nonneoplastic epithelial disorder, treatment is inﬂuenced by the patient’s age. Elderly patients who often had many years of chronic itching may not be disturbed by the prospect of a vulvectomy. In younger women, it is desirable to conserve as much of the vulva as possible. Radical local excision should be performed for the invasive disease, and the associated intraepithelial disease should be treated in the manner most appropriate to the patient. For example, topical steroids may be required for lichen sclerosus or squamous hyperplasia, whereas VIN may require superficial local excision with primary closure or laser ablation.

Occlusive irritant dermatitis: when is "allergic" contact dermatitis not allergic?

CASE 1: A 38-year-old teacher presented with a 3- to 4-week history of a linear, erythematous, vesicular, and pruritic eruption of her left wrist. She had been wearing a new elastic bracelet for 4 weeks before the onset of her eruption. Although there was no history of allergy to rubber products or jewelry, an allergic contact dermatitis to rubber was suspected. Patch testing to rubber chemicals and the elastic bracelet revealed no reactions at 48, 72, and 96 hours. She stopped wearing the bracelet and used a corticosteroid cream with rapid resolution of the problem. The patient resumed wearing the bracelet, and there has been no recurrence in the past 2 months. CASE 2: A 12-year-old boy presented with a 1-month history of an itchy, scaly, erythematous 1-cm patch over the midline of his lower lip. The patient complained of tiny blisters initially with persistent erythema, mild scaling, and associated pruritus. The patient plays the saxophone and he had been practicing more intensely (3 to 4 h/d) for a musical competition. Allergic reaction to his wood reed was suspected, but patch testing with a moistened portion of his reed and reed shavings in a drop of water revealed no reaction at 48 and 72 hours. Treatment with hydrocortisone 1% cream bid for 3 days led to complete resolution of the dermitis and pruritus. Playing the saxophone 1 h/d has not led to any recurrence. CASE 3: A 33-year-old woman presented with erythema, scaling, and pruritus of 1 month's duration beneath her engagement and wedding rings, which were worn together on her left fourth finger (Figure 3). Although she had no history of previous sensitivity to earrings, watch clasp, blue jean rivets, or other jewelry, allergic contact dermatitis to nickel was suspected. Patch testing was performed to the common metal allergens nickel, cobalt, chromium, and gold. Readings at 48 hours and 1 week revealed no positive reactions. The patient wore her rings on the right hand for 1 week and used fluocinonide 0.5% cream twice daily for 1 week with resolution of the dermatitis. She has subsequently begun wearing the rings again on her left hand with care to dry her hands and rings after washing, and there has been no recurrence of her dermatitis.

[Screening strategies for vulvar preneoplastic and neoplastic lesions].

Although they cover superficial areas, preneoplastic and neoplastic lesions of the vulva are often diagnosed late. The reasons for this delay is the low incidence of this invasive neoplasm, the advanced age of patients, the non-specific symptoms mainly taking the form of itch, burning, dyspareunia and blood loss, which are also compatible with a non-neoplastic infective pathology. The late diagnosis of carcinoma of the vulva may also be linked to the inadequate examination of the external genitals by doctors as a result of insufficient specific knowledge. Epidemiological data and the natural history of VIN lesions and carcinoma of the vulva argue that mass screening is not feasible, but an adequate programme of early diagnosis must be introduced. Early diagnosis is linked to three key elements: targeted anamnesis, clinical examination and the appropriate use of the various diagnostic procedures. Vulvoscopy represents the most reliable method, above all because it allows a biopsy to be taken of any suspected lesion. From January 1992 to December 1998, a total of 1678 vulvoscopies were performed at the Institute of Clinical Obstetrics and Gynecology of the University of Catania in patients aged between 16 and 82 years old. Biopsies were taken of all suspected lesions. Sixty-nine cases of VIN (4.11%) were diagnosed: 28 VIN1, 24 VIN2 and 17 VIN3. Lesions were only symptomatic in 39.1% of cases. The association of vulvoscopy with biopsy of suspected lesions, even in the absence of vulvar symptoms, represents the most efficacious method for the diagnosis of intraepithelial lesions.

Trichotillomania: Bizzare Patern of Hair Loss at 11-Year-old Girl.

Trichotillomania (TTM) is defined by the Diagnostics and Statistic Manual of Mental Disorders, 4th edition (DMS-IV) as hair loss from a patient`s repetitive self-pulling of hair. The disorder is included under anxiety disorders because it shares some obsessive-compulsive features. Patients have the tendency towards feelings of unattractiveness, body dissatisfaction, and low self-esteem (1,2). It is a major psychiatric problem, but many patients with this disorder first present to a dermatologist. An 11-year-old girl came to our department with a 2-month history of diffuse hair loss on the frontoparietal and parietotemporal area (Figure 1). She had originally been examined by a pediatrician with the diagnosis of alopecia areata. The patient`s personal history included hay fever and shortsightedness, and she suffered from varicella and mononucleosis. Nobody in the family history suffered from alopecia areata, but her father has male androgenetic alopecia (Norwood/Hamilton MAGA C3F3). The mother noticed that the child had had changeable mood for about 2 months and did not want to communicate with other persons in the family. The family did not have any pet at home. At school, her favorite subjects were Math and Computer Studies. She did not like Physical Education and did not participate in any sport activities during her free time. This was very strange because she was obese (body-mass index (BMI) 24.69). She was sometimes angry with her 13-year-old sister who had better results at school. The girl had suddenly started to wear a blue scarf. The parents did not notice that she pulled out her hair at home. Dermatological examination of the capillitium found a zone of incomplete alopecia in the frontoparietal and parietotemporal area, without inflammation, desquamation, and scaring. Hairs were of variable length (Figure 1). There was a patch of incomplete alopecia above the forehead between two stripes of hair of variable length (Figure 2). The hair pull test was negative along the edges of the alopecia. Mycological examination from the skin capillitium was negative. The trichoscopy and skin biopsy of the parietotemporal region of the capillitium (Figure 3) confirmed trichotillomania. Laboratory tests (blood count, iron, ferritin, transferrin, selenium, zinc, vitamin B12, folic acid, serology and hormones of thyroid gland) were negative. We referred the girl for ophthalmologic and psychological examination. Ophthalmologic examination proved that there was no need to add any more diopters. The psychological examination provided us with a picture in which she drew her family (Figure 4). The strongest authority in the family was the mother because she looked after the girls for most of the day. She was in the first place in the picture. The father had longer working hours and spent more time outside the home. He worked as a long vehicle driver. He was in the second place in the picture. There was sibling rivalry between the girls, but the parents did not notice this problem and preferred the older daughter. She was successful at school and was prettier (slim, higher, curly brown hair, without spectacles). Our 11-years-old patient noticed all these differences between them, but at her level of mental development was not able to cope with this problem. She wanted to be her sister's equal. The sister is drawn in the picture in the third place next to father, while the patient's own figure was drawn larger and slim even though she was obese. Notably, all three female figures had very nice long brown hair. It seemed that the mother and our patient had better quality of hair and more intense color than the sister in the drawing. The only hairless person in the picture was the father. The girl did not want to talk about her problems and feelings at home. Then it was confirmed that our patient was very sensitive, anxious, willful, and withdrawn. She was interested in her body and very perceptive of her physical appearance. From the psychological point of view, the parents started to pay more interest to their younger daughter and tried to understand and help her. After consultation with the psychiatrist, we did not start psychopharmacologic therapy for trichotillomania; instead, we started treatment with cognitive behavioral therapy, mild shampoo, mild topical steroids (e.g. hydrocortisone butyrate 0.1%) in solution and methionine in capsules. With parents' cooperation, the treatment was successful. The name trichotillomania was first employed by the French dermatologist Francois Henri Hallopeau in 1889, who described a young man pulling his hair out in tufts (3-5). The word is derived from the Greek thrix (hair), tillein (to pull), and mania (madness) (5). The prevalence of TTM in the general adult population ranges from 0.6% to 4%, and 2-4% of the general psychiatric outpatient population meet the criteria for TTM (2-5). The prevalence among children and adolescents has been estimated at less than 1% (5). The disease can occur at any age and in any sex. The age of onset of hair pulling is significantly later for men than for women (3). There are three subsets of age: preschool children, preadolescents to young adults, and adults. The mean age of onset is pre-pubertal. It ranges from 8 to 13 years (on average 11.3 years) (2-5). The occurrence of hair-pulling in the first year of life is a rare event, probably comprising <1% of cases (5). The etiology of TTM is complex and may be triggered by a psychosocial stressor within the family, such as separation from an attachment figure, hospitalization of the child or parent, birth of a younger sibling, sibling rivalry, moving to a new house, or problems with school performance. It has been hypothesized that the habit may begin with "playing" with the hair, with later chronic pulling resulting in obvious hair loss (2). Environment is a factor because children usually pull their hair when alone and in relaxed surroundings. The bedroom, bathroom, or family room are "high-risk" situations for hair-pulling (5). Men and women also differed in terms of the hair pulling site (men pull hair from the stomach/back and the moustache/beard areas, while women pull from the scalp) (3). Pulling hair from siblings, pets, dolls, and stuffed animals has also been documented, often occurring in the same pattern as in the patient (5). Genetic factors contributing to the development of TTM are mutations of the SLITRK1 gene, which plays a role in cortex development and neuronal growth. The protein SAPAP3 has been present in 4.2% of TTM cases and patients with obsessive-compulsive disorder (OCD). It may be involved in the development of the spectrum of OCD. A significantly different concordance rate for TTM was found in monozygotic (38.1%) compared with dizygotic (0%) twins in 34 pairs (3). The core diagnostic feature is the repetitive pulling of hairs from one`s own body, resulting in hair loss. The targeted hair is mostly on the scalp (75%), but may also be from the eyebrows (42%), eyelashes (53%), beard (10%), and pubic area (17%) (3,5). There are three subtypes of hair pulling - early onset, automatic, and focused. Diagnostic criteria for TTM according to DSM-IV criteria are (2,3,5): 1) recurrent pulling of one`s hair resulting in noticeable hair loss; 2) an increasing sense of tension immediately prior to pulling out the hair or when attempting to resist the behavior; 3) pleasure, gratification, or relief when pulling out the hair; 4) the disturbance is not better accounted for by another mental disorder and is not due to a general medical condition (e.g., a dermatologic condition); 5) the disturbance causes clinically significant distress or impairment in social, occupational, or other important areas of functioning. The differential diagnosis includes alopecia areata (Table 1) (6), tinea capitis, telogen effluvium, secondary syphilis, traction alopecia, loose anagen syndrome, lichen planopilaris, alopecia mucinosa, and scleroderma (2-5). Biopsy of an involved area (ideally from a recent site of hair loss) can help to confirm the diagnosis (5). On histologic examination, there are typically increased numbers of catagen and telogen hairs without evidence of inflammation. Chronic hair pulling induces a catagen phase, and more hairs will be telogen hairs. Pigment casts and empty anagen follicles are often seen. Perifollicular hemorrhage near the hair bulb is an indicator of TTM (2). Complications of TTM are rare, but they comprise secondary bacterial infections with regional lymphadenopathy as a result of picking and scratching at the scalp. Many patients play with and ingest the pulled hairs (e.g. touching the hair to lips, biting, and chewing). Trichophagia (ingestion of the hair) can lead to a rare complication named trichobezoar (a "hair ball" in stomach). This habit is present in approximately 5% to 30% of adult patients, but it is less frequent in children. Patient with trichophagia present with pallor, nausea, vomiting, anorexia, and weight loss. Radiologic examination and gastroscopy should not be delayed (2,4,5). The management of the disease is difficult and requires strong cooperation between the physician, patient, and parents. The dermatologist cannot take part in the therapy, strictly speaking, but without the psychological, psychopharmacologic, and topic dermatologic treatment a vicious circle will be perpetuated.

The chronically symptomatic vulva: aetiology and management.

To determine the causes and management of chronic vulval symptoms and to compare the findings in patients first presenting to a gynaecologist with those in patients first presenting to a dermatologist. A prospective study of 144 patients, approximately half each being referred to a gynaecologist and a dermatologist. Diagnosis was based on clinical history, vulvoscopy, vulval biopsy and bacteriology. Biopsies were examined by a histopathologist experienced in dermatopathology and gynaecological pathology. The two patient groups were similar in both range and frequency of conditions. The commonest cause of chronic vulval symptoms was dermatitis, which was found in 64% of our patients. Dermatitis occurred alone in 55% and was found in association with histological evidence of human papilloma virus (HPV) in a further 9%. These patients responded to simple dermatological methods, mainly topical corticosteroids. Histopathological evidence of HPV was encountered in only 23% of our patients, and of these 36% also demonstrated dermatitis on biopsy. Most responded to topical corticosteroids. Another 7% had lichen sclerosus, and all responded to potent topical corticosteroid. The remaining 15% demonstrated a range of diagnoses, including psoriasis, dysaesthetic vulvodynia, vulval intraepithelial neoplasia (VIN) and chronic candidiasis. The majority of patients had a corticosteroid responsive dermatosis rather than a gynaecological condition. The majority of patients with a chronically symptomatic vulva who present to either a gynaecologist or a dermatologist have a dermatological condition that responds to simple dermatological treatments. We believe that the presence or absence of the human papilloma virus is not relevant to most patients with a chronically symptomatic vulva and treatments should not be aimed at eradicating this virus. Histopathologists and gynaecologists who have focused on gynaecological disorders have often missed simple dermatological conditions that are easily treatable.

Unwarranted hysterectomy in a case of oro-vaginal-vulvar lichen planus in a young woman: a case report.

Lichen planus is a rare autoimmune disease primarily affecting the skin and mucous membranes of the oral mucosa, vulva, and vagina. Diagnosis is difficult and often delayed as the clinicians do not associate the oral symptoms with the genital symptoms. This has a negative impact on the out-of-pocket expenditure and quality of life of the patients. We report this case, as only anecdotal cases have been reported so far from a developing country such as India. We highlight the unindicated hysterectomy that the patient had undergone because of lack of awareness regarding this condition. Our case report also highlights the importance of the multidisciplinary team approach to optimize outcomes and avoid unnecessary morbidity to such patients. We report a North-Indian patient with oro-vaginal-vulvar lichen planus who presented to us with complaints of recurrent vulvovaginal symptoms for the last 5 years. She had been previously treated with multiple courses of antibiotics, antifungals, and topical steroids over the course of 3 years and finally offered laparoscopic-assisted vaginal hysterectomy (LAVH) by a private practitioner but got no relief. She also had complained of oral symptoms in the form of a burning sensation after eating spicy food, but did not seek any treatment for this. After multidisciplinary team discussion, a final diagnosis of oro-vaginal-vulvar lichen planus was made at our institute based on the clinical and histopathological findings. The patient was immediately started on oral prednisolone to which she responded with improvement in her symptoms. Lichen planus is a chronic painful condition with significant impact on the quality of life. Women often suffer for several years before an accurate diagnosis is made. Treatment is challenging and needs to be individualized with a multidisciplinary approach to prevent progressive anatomical distortion and associated morbidity.

The chronically symptomatic vulva: prevalence in primary health care.

To examine within the primary health care setting, the frequency of chronic vulval disorders, their frequency, investigation, management and referral patterns. A questionnaire based survey at general practitioners surrounding a district general hospital. A total of 79 (72%) GPs replied to the questionnaire. Thirty-six (45%) GPs saw more than one patient per month with recurrent vulval symptoms and ten (13%) GPs saw more than three patients per month. The predominant symptom amongst patients with recurrent vulval symptoms was itching (55%) followed by soreness (26%), burning (14%) and dyspareunia (5%). Topical-antifungals and steroid medications were the commonest treatments prescribed for all women with vulval symptoms. Women who have vulval complaints commonly attend their GP for treatment. In many instances women with chronic vulval complaints are inadequately investigated and inappropriately managed.

Vulvar Leiomyosarcoma in Pregnancy.

Vulvar leiomyosarcoma is a rare smooth muscle malignant neoplasm but it is the commonest type of vulvar sarcomas. It may mimic benign tumors and misdiagnosis could delay proper management. We report a case of a 38-year-old pregnant woman with leiomyosarcoma of the vulva. The patient presented to her primary general practitioner with a small vulvar mass that she had first noticed one year prior. The tumor was suspected to be benign Bartholin's cyst and treated with antibiotics. The patient declined improvement and had many consultations to different clinics where she had been diagnosed and treated the same. The tumor size started to grow rapidly after she got pregnant, and the patient was referred to our hospital where she underwent tumor resection. Histopathology revealed leiomyosarcoma. The patient had further assessment and close follow-up and has had no recurrence for 12 months. There is little literature available on vulvar leiomyosarcoma, most of which are case reports, and most gynecologic oncologists will go through their whole careers without seeing a single case.

Vulvar dermatoses: a cross-sectional 5-year study. Experience in a specialized vulvar unit.

Vulvar diseases are common in the general population and have a negative impact on the quality of life. To describe our experience as dermatologists in the management of vulvar dermatosis consultations. A retrospective observational study was conducted with patients who attended monographic vulvar consultations over a 5-year period. Clinical information was obtained from the patient's charts. 148 women were studied. Their mean age was 43.24 years (standard deviation: 15.15 years), with ages ranging from 4 months to 80 years. 53.4% of patients took between 2 and 5 years to seek medical attention for the first time. The most frequent diagnosis was lichen sclerosus (41.9%), irritative eczema of the vulva (14.9%), and lichen simplex chronicus (10.1%). 83.8% reported anogenital itching, 66.2% pain, and 45.9% dyspareunia. The most frequently prescribed treatment was ultra-potent topical corticosteroids (clobetasol propionate; 41.2%). Patients with lichen sclerosus were significantly older than those who presented with any of the other diseases. No differences were found in terms of either the time of disease evolution or in symptom presentation. Retrospective study. Vulvar diseases with an infectious cause are usually managed in primary care, therefore, were not included. All patients were recruited from a single private hospital which limits the comparisons with the public health system. Vulvar diseases frequently occur and are associated with high morbidity. It is essential to promote the development of specific vulvar consultations in hospitals. Specialties such as dermatology, gynecology, urology, or physiotherapy must be part of these units.

Lung Metastasis in a Case of Recurrent Poorly Differentiated Leiomyosarcoma of the Bartholin Gland: A Case Report and Review of the Literature.

Vulvar neoplasms represent four percent of all gynecological cancers. While most cases of vulvar neoplasms are benign, two percent of patients present with malignant disease. We present the case of a 37-year-old premenopausal female who presented to an outside institution with a lump in her left vulva, which had progressively enlarged to the size of an egg. A wide local excision of the left vulva was performed, and the pathology revealed a high-grade sarcoma, not otherwise specified (NOS), with negative margins. Imaging showed enlarged bilateral external iliac lymph nodes, likely metastatic. After discussion at a multidisciplinary gynecology oncology tumor board, she was treated with gemcitabine/docetaxel chemotherapy, followed by a left inguinal lymph node dissection and a left radical vulvectomy after being referred to our centre. The final pathology at that time showed a residual sarcoma of 3.5 mm in the left vulva with no lympho-vascular invasion (LVI) and negative margins, with the closest, laterally, at 2 mm. A total of three lymph nodes were negative. She received additional chemotherapy postoperatively. Approximately one year later, she returned to her gynecologist with a 1 cm mass on the left vulva. She underwent a left hemi-vulvectomy and lymph node dissection, and pathology confirmed the presence of a high-grade sarcoma with close margins. She received adjuvant radiotherapy. Three months later, she presented with persistent cough and pneumonia. Imaging revealed a 10 cm lung mass, which was believed to be metastasis from the vulva. This was confirmed with biopsy and was completely resected. Any mass in the Bartholin gland area should be investigated carefully. Poorly differentiated vulvar leiomyosarcoma in the Bartholin gland can recur locally but may also lead to distant metastasis. Despite surgical and systemic treatment, as well as adjuvant radiation, the tumor recurred. Due to the rarity of this condition, there are no clear recommendations for treatment of this disease. To our knowledge, this is the first report of vulvar leiomyosarcoma of the Bartholin gland with metastasis to the lung.

Women's experiences of general practitioner management of their vaginal symptoms.

Little is known about the management of vaginal symptoms despite their frequency. Most vaginal symptoms in non-menopausal women are managed as thrush, although bacterial vaginosis is commoner. The aim of this study was to measure the experiences of women attending their general practitioner with vaginal symptoms including self-reporting of symptoms, duration and severity, informal support, over-the-counter remedies, sources of information, gender of doctor, expected and actual vaginal examination, and explanations and knowledge of common vaginal infections. A postal questionnaire survey was conducted of 490 patients presenting with vaginal symptoms aged between 18 and 48 years who had attended 10 general practices within the previous 3 months in the East Midlands of England. A total of 85% of patients had suffered a previous episode, with 39% having had three or more infections in the previous year. Overall, 68% consult with each episode, and most consult within 7 days of onset of symptoms (median = 4 days). In all, 68% discussed their symptoms with partners, families or friends, but 32% relied solely on their doctor. Some 33% bought over-the-counter remedies. A total of 65% informed themselves further from encyclopaedias, leaflets and women's magazines, but there was a strong request for more information. Out of those questioned, 75% expected a vaginal examination, whereas 57% had such an examination performed. Most were told their symptoms were caused by thrush (78%), but patients' ideas on causation were varied. Most believed sexual transmission played a role in transmission of symptoms. Women were socially embarrassed by their symptoms, with 46% admitting to having the condition on their minds all or most of the time. Twenty-eight per cent of women wished to see a female doctor, with gender being unimportant to the remainder. Vaginal symptoms were commonly recurrent, socially embarrassing and managed as candidiasis. Just over half the patients had a vaginal examination. There is a shortage of suitable information on vaginal infections available to patients, many of whom used over-the-counter medications.

Synchronous of breast and vulvar Paget's disease: a case report.

Synchronous Paget's disease of breast and vulva is extremely rare and has only been reported in the literature in one other case. A 58-year-old postmenopausal woman was found to have crusting, bleeding, and discharge from left nipple, as well as vulvar pruritis at the same time. Biopsy of breast lesion demonstrated Paget's disease with an underlying foci of ductal carcinoma in-situ that required total mastectomy of left breast with sentinel node biopsy and breast reconstruction. For vulvar symptoms, the patient was initially diagnosed with dermatitis and topical ointment was prescribed. However, her symptoms persisted for the next several months, and she underwent vulvar biopsy that demonstrated Paget's disease. She underwent partial vulvectomy. Multiple episodes of recurrent vulvar Paget's disease were noted in the postoperative course that medical therapy with Imiquimod and a second partial vulvectomy was performed. Synchronous of breast and vulvar Paget's disease is presented. There was a delay in diagnosing vulvar Paget's disease in this experienced case. While coincidence of breast and vulvar Paget's disease is likely, ectopic mammary tissue in vulvar as well as secondary metastasis from a focal lesion of breast Paget's disease needs to be carefully evaluated whenever the patient complains of vulvar symptoms in the setting of breast Paget's disease.

The vulvar clinic: an eight-year appraisal.

One thousand forty-three patients made 2,750 visits to the Vulvar Clinic at the Milwaukee County Medical Complex from July, 1970, to July, 1978. This experience is analyzed and the various diseases are grouped in order of frequency. The results shed light on those vulvar problems most likely to be encountered in general office practice and point the way toward future areas of research.

Unusual presentation of lichen sclerosuis in an adolescent

Background: The presentation of lichen sclerosus has been described in detail in the adult literature. Typically present with symptoms of itching and soreness in the vulvar area at which time a vulvar evaluation reveals a specific appearance. The presentation is believed to be similar in prepubertal children and adolescents. In this case report we encountered an unusual initial presentation of this disease.Methods: Case presentation.Results: An 18-year-old female presented for the first time to her gynecologist with complaint of difficulty with complete emptying of bladder and dribbling. She had noted the onset of these symptoms two months prior to presentation. She denied any long-term history of vulvar itching or irritation. Her menses were normal with no complaints of dysmenorrhea. Onset of menarche and pubertal development were also normal. She denied any pre-pubertal history of labial adhesions or lichen sclerosis. The patient was not sexually active. She was diagnosed with labial adhesions and her first course of treatment included topical estrogen therapy for 8 weeks. Her second course of therapy included topical testosterone for 6 weeks without any improvement or side effects. On evaluation at our institution the posterior aspect of the labia minora could not be seen and the area of the vaginal introitus was completely obstructed (see picture). The clitoral hood could not be retracted and the surrounding vulva appeared atrophic and white. The degree of obstruction was such that the urethra could not be seen. In the operating room the labia minora were manually separated. The patient applied clobetasol.05% ointment for the next two weeks to the vulva and then switched to a less potent steroid. Follow-up evaluation 2 and 4 weeks after the procedure did not show any adhesions. Punch biopsy was consistent with diagnosis of lichen sclerosis.Conclusion: The presentation of lichen sclerosis may be variable in adolescents; thus, a high index of suspicion must be maintained to make this diagnosis.

Vulvar skin disorders throughout lifetime: about some representative dermatoses.

The objective of this paper is to present general considerations which should be kept in mind by clinicians in charge of women with vulvar diseases. Four representative vulvar dermatoses are described. Lichen simplex chronicus is a pathological condition related to chemical and mechanical irritant agents. Detrimental effects of these irritants, in the presence of other dermatoses, have to be considered when therapeutic responses are unsatisfactory. Lichen sclerosus is the most common vulvar dermatosis in elderly. However, it should be kept in mind that it may be diagnosed at any age. Lichen planus, in spite of sharing a similar range of etiological factors with lichen sclerosus, is a very distinct entity. Finally, Paget's disease, although rare, is also described especially because of the challenge it represents both clinically and therapeutically.

Vulvar carcinoma in pregnant women aged less than 40 years: case report.

Invasive squamous cell carcinoma of the vulva is primarily a disease of postmenopausal women and thus is rarely associated with pregnancy. We have reported on a young woman under 40 years old with vulvar carcinoma, which occurred during the pregnancy but optimal treatment was delayed to the postpartum period. This 37-year-old woman was diagnosed with 3x3 cm vulvar lesion, 2 weeks after cesarean section, subsequent biopsy revealed squamous cell carcinoma. She had a history of an ulcer on her left labia minor at the third month of the pregnancy. She was treated by a modified radical vulvectomy and bilateral groin lymphadenectomy. She did not receive any additional treatments. Now after two years, she has had no recurrence of the disease. This case emphasizes on the need to consider malignancy as a differential diagnosis in vulvar lesions of pregnant young women.

Vulval disease from the 1800s to the new millennium.

The study of vulval disease has become important over the last few decades. Although several inflammatory dermatoses were described at the end of the 19th century, vulval involvement in these conditions was only realized some time later. Indeed, the vulva may be a site of predilection of some inflammatory dermatoses such as lichen sclerosus. There are now groups of interested dermatologists, gynecologists, and genitourinary physicians that have cooperated to study patients with vulval disease. Hopefully, this will increase our knowledge over the next century. This review article examines vulval disease from an historical viewpoint and highlights important developments that have increased our understanding of the disorders that specifically affect the vulva.

Amelanotic vulvar melanoma: a case report.

A 14-year-old girl stated that she has vulvar pruritus, pain, and bleeding and underwent a complete gynecological evaluation during which a suspicious 2-cm achromic lesion on her vulva was observed. Initially, it was suspected to be a pyogenic granuloma, in relation to the patient's age. A biopsy was taken, and a histopathological diagnosis of amelanotic mucosal melanoma was made. Pyogenic granuloma is a reactive hyperproliferation vascular response to trauma or other stimuli. It predominantly occurs in the second decade of life in young females, in relation to the vascular effects of female hormones. Primary mucosal melanoma is a rare and aggressive neoplasm, characterized by a higher aggressiveness and a worse prognosis than her cutaneous counterpart. The female genital tract is the second most common site of onset of mucosal melanoma; it represents the 3% of melanomas diagnosed in women.

The commonest causes of symptomatic vulvar disease: a dermatologist's perspective.

A prospective study of 141 consecutive adult patients with chronic vulvar symptoms referred to a dermatologist was carried out to determine the commonest conditions seen. Eighty-nine per cent of patients underwent vulvar biopsy. The commonest cause of chronic vulvar symptoms in this group of patients was dermatitis, seen in 54% of patients. The other commonly seen conditions were lichen sclerosus (13%), chronic vulvovaginal candidiasis (10%), dysaesthetic vulvodynia (9%) and psoriasis (5%). Although 38% of patients had previously been diagnosed as suffering from human papillomavirus (HPV) vulvitis, histopathological evidence of HPV was seen in only 5%. All cases showing HPV also demonstrated spongiotic dermatitis on biopsy. In this study group, a majority (overall 72%) of patients with a chronic vulvar complaint had a corticosteroid responsive dermatosis rather than a gynaecological condition. The patients with HPV on biopsy also responded to topical corticosteroids, and it was concluded that their symptoms may have been due to dermatitis unrelated to the presence of HPV. In such patients, the assumption that 'subclinical HPV' is a cause of symptoms and the practice of focusing medical and particularly surgical treatment on eradication of the virus may be inappropriate. A review of the commonest vulvar conditions seen by the author is presented.

Vulvar Paget's Disease Presenting with Fever and Left Inguinal and Peritoneal Lymphadenopathies.

BACKGROUND Asymptomatic vulvar Paget's disease is rare and commonly presents with vulvar eczema, erosions, or pruritus. The time from onset to diagnosis of vulvar Paget's disease tends to be rather long because of difficulty making a correct diagnosis owing to similar skin findings with eczema or patients' reluctance to undergo physical examination of their pubic area because of embarrassment. CASE REPORT A 55-year-old woman experienced recurrent episodes of fever for 10 months. Her primary care physician indicated inguinal lymphadenopathy 2 months prior to presentation at our hospital. Contrast-enhanced abdominal computed tomography revealed multiple intra-abdominal lymphadenopathies. With the failure of finding the primary lesion after biopsy, and with a diagnosis of metastatic carcinoma, she was referred to our hospital. On admission, she did not report having vulvar symptoms. As imaging studies revealed no primary lesions, we subsequently added immunostaining to the lymph node biopsy specimens, which suggested Paget's disease. We finally performed a vulvar physical examination and identified eczema. We performed a skin biopsy and histopathological examinations, which provided the final diagnosis of vulvar Paget's disease. CONCLUSIONS We experienced a case of vulvar Paget's disease presenting with inguinal and intraperitoneal lymphadenopathies, without a patient report of vulvar symptoms. When identifying lymphadenopathies, it is crucial to obtain a careful history and perform appropriate physical examinations, suspecting diseases of the vulva or perineum. In addition, immunostaining of lymph node biopsy specimens could be useful in making a correct diagnosis.

Verrucous Carcinoma of the Vulva: A 20 Year Retrospective Study and Literature Review.

The aim of the study was to assess the clinicopathological characteristics of patients with verrucous carcinoma (VC) of the vulva. We reviewed data on the age, disease course, clinical manifestation, pathologic diagnosis, treatment, and follow-up of patients with vulvar VC who were treated at Tianjin Medical University General Hospital and cases that were reported in the Chinese and English literature during the past 20 years. Six cases were identified in Tianjin Medical University General Hospital, the mean age of patients was 55 years, and their mean disease course was 26 months. Primary symptoms were exophytic neoplasm with pruritus and/or pain. Surgical treatment included wide local excision, simple vulvectomy, and radical vulvectomy with or without lymph node dissection in the groin. Vulvar VC occurred simultaneously with vulvar intraepithelial neoplasia in 2 cases and well-differentiated squamous cell carcinoma in 2 cases. The mean follow-up was 16.8 months with no recurrence in those 6 cases. During the 20-year period, 20 and 41 cases were reported in Chinese and English literature, respectively. Three cases were misdiagnosed with giant condyloma acuminatum in China. One case (1/20) was reported with coexistent squamous carcinoma in Chinese literature and 8 cases (8/41) in the English literature. The recurrence rate was 12.5% (2/19) and 17.5% (7/40) in Chinese and English literature, respectively. Vulvar VC is a distinct type of slow-growing, nonmetastatic tumor with unclear etiology. These tumors should be distinguished from giant condyloma acuminatum and well-differentiated squamous cell carcinoma. Surgery is the most effective treatment.