Insights from a joint pediatric dermatology-gynecology vulvar clinic: A retrospective study.

BACKGROUND/OBJECTIVES: Pediatric vulvar disease has not been widely explored in the medical literature. Few studies focus on vulvar disease in skin of color. The vulvar disease can be distressing for young patients given the sensitive location, and providers may lack experience in diagnosing and managing vulvar dermatoses. We sought to characterize the conditions seen, diagnostic challenges encountered, and the racial and ethnic factors associated with vulvar diseases in our multidisciplinary pediatric dermatology-gynecology vulvar clinic at Children's National. METHODS: Medical records of 220 patients who presented to our multidisciplinary pediatric dermatology-gynecology clinic were reviewed retrospectively. RESULTS: Lichen sclerosus (LS) (36%, n = 80), inflammatory vulvitis (11%, n = 23), and vitiligo (9%, n = 19) were the three most frequent conditions observed. These conditions were often misdiagnosed as one another. There was a mean delay in diagnosis after symptom onset in LS patients of 16.43 months. CONCLUSIONS: LS, inflammatory vulvitis, and vitiligo are common vulvar diseases among pediatric patients. Accurate diagnosis is important because LS must be treated aggressively to prevent sequelae. Further studies are warranted to help differentiate LS and vitiligo with consideration of skin tone.

Female Genital Itch.

Vulvar pruritus is a common complaint among young girls and women presenting to primary care physicians, gynecologists, and dermatologists. Female genital itch is especially disruptive because of its interference with sexual function and intimacy. Causes of vulvar itch are vast and may be inflammatory, environmental, neoplastic, or infectious, often with several causes coexisting simultaneously. Diagnosis may be difficult because of the unique anatomy and inherent properties of genital and perianal skin. Treatment is aimed at eliminating outside irritants, restoring epidermal barrier function, and suppressing inflammation.

«Perineal groove», ese gran desconocido / Perineal groove, the great unknown

El perineal groove, o surco perineal, es una malformación benigna y poco frecuente que tiende a la resolución espontánea. De forma general, se caracteriza por un surco húmedo en la línea media perineal que se extiende desde la horquilla vulvar hasta el ano. El surco perineal se presenta más frecuentemente en niñas, aunque también se ha descrito en varones. Se ignora su incidencia real, debido en parte al desconocimiento del mismo por parte de los profesionales. Presentamos el caso de una recién nacida de 2 días de vida en la que se detecta una lesión lineal en el periné. Es importante su reconocimiento dado que entraña un amplio diagnóstico diferencial de lesiones, incluidas algunas con importantes connotaciones legales por la zona en la que se presenta (AU)

The perineal groove, or perineal sulcus, is an uncommon and benign anomaly which tends to resolve spontaneously. In general, it has been described as a wet sulcus in the midline perineum which extends from the fourchette to the anus. Although the perineal groove appears more frequently in girls, it has been described in males too. The incidence of perineal groove is ignored, it is due to the lack of knowledge of it by health professionals. We report one case of a 2-days-old baby girl. There was found a lineal lesion in the perineum during her clinical examination. Its recognition is important since it involves a wide differential diagnosis of injures, including some of them with important legal implications because of the area in which it is presented (AU)

Symptoms in plasma cell vulvitis: first observational cohort study on type, frequency and severity.

BACKGROUND: Studies assessing symptoms of plasma cell vulvitis (PCV) are lacking. OBJECTIVES: To assess the prevalence and severity of PCV-related symptoms and identify possible associations between patient characteristics, clinical features of PCV and treatments administered before a definitive diagnosis. METHODS: Thirty-six patients affected with PCV were included. Data were collected by direct interview and clinical examination. RESULTS: Thirty patients (83.3%) complained of symptoms: burning was the most frequent (80.6%) while dyspareunia was the severest. Of the symptomatic patients, 73.3% experienced severe symptoms. Severity of symptoms was not associated with age at onset and duration of PCV. Almost 70% of the patients had previously undergone treatments. CONCLUSIONS: Symptoms in PCV are frequent and more than often severe. Neither age at onset nor duration of the disease nor the extent of vulvar involvement were associated with symptom severity. Both the delay in diagnosis and the inappropriate previous treatments seem to indicate frequent misdiagnosis.

Granulomatous cheilitis with granulomatous vulvitis: a rare association.

Granulomatous cheilitis and granulomatous vulvitis are rare disorders characterized by painless swelling of lips and vulva, respectively. Histopathology of both conditions show non-caseating epithelioid cell granulomas in the dermis. Both disorders have been associated with Crohn's disease rarely. Occurrence of the two conditions in the same patient is extremely infrequent. We hereby report, the association of granulomatous cheilitis with granulomatous vulvitis in a 30-year-old female.

[A woman with cayenne pepper spots]. / Een vrouw met roestbruine plekjes op de vulva.

A 52-year-old female with dyspareunia presented with red-brown maculae at the vulva.Biopsy revealed an inflammatory cell infiltrate mostly consisting of plasma cells, and the diagnosis 'vulvitis circumscripta plasmacellularis' was established.A biopsy is mandatory to confirm this rare benign inflammatory disorder of the vulva.

Vulvovaginitis infantil / Child vulvovaginitis

La vulvovaginitis es el problema ginecológico más frecuente en las niñas prepúberes, favorecido por la menor protección del introito vaginal a los agentes externos y la presencia de factores anatómicos (proximidad del ano, labios mayores y menores menos desarrollados, etc.) y hormonales (hipoestrogenismo). A esto se suma el efecto de productos irritantes locales y la coexistencia en ocasiones de malos hábitos higiénicos. La mayoría de las vulvovaginitis en las niñas son inespecíficas, aunque en un porcentaje variable pueden aislarse bacterias patógenas específicas (entéricas, respiratorias, o de transmisión sexual), o ser secundarias a otros procesos como oxiurasis o cuerpos extraños intravaginales. En el presente artículo analizaremos la etiología, la patogénesis, la clínica y el manejo de esta entidad en la infancia, y realizaremos algunas consideraciones clínicas y microbiológicas sobre distintos cuadros con etiología específica(AU)

Vulvovaginitis is the most common gynecological problem in prepubertal girls, helped by lower introitus protection to external agents and the presence of anatomical (proximity of anus, minor and major labia less developed,...) and hormonal factors (low estrogen concentration) This is compounded by the effect of local irritants and sometimes co-existence of poor hygiene. Most vulvovaginitis in girls are nonspecific, although specific pathogenic bacteria (enteric, respiratory, or sexually transmitted) can be isolated and they can be also secondary to other causes as oxiurasis, or intravaginal foreign bodies. In this article we will analyze the etiology, pathogenesis, clinical features and management of this disorder in childhood, and we will have some clinical and microbiological considerations of different diseases with specific etiology(AU)

Vulvovaginal-gingival syndrome.

Vulvovaginal-gingival syndrome is a distinct variant of classic lichen planus frequently associated with mucocutaneous scarring and vaginal stricture formation, therefore early diagnosis and treatment through a multidisciplinary approach is essential. Management is challenging and is characterised by wide variation in treatments and lack of evidence-based therapeutic approaches. Literature review with particular regard to therapeutic approaches is performed with a view towards a cooperative treatment study.

A case of idiopathic granulomatous cheilitis and vulvitis.

A 36-year-old woman presented with chronic recurring dermatitis of the vulva, perineum, and lips. The genital lesions had a 3-year history and were associated with slight pruritus and occasional pain. The lesions of the upper lip had a history of 6 months. The patient had been treated with cycles of antimycotics and topical steroids which only partially controlled the symptoms during treatment. Dermatologic examination showed erythematous, infiltrative dermatitis with edema of the labia majora and persistent edema of the upper lip (Fig. 1a,b). Routine blood chemistry, urine analysis, and chest X-ray were normal. Microscopic examination and cultures of vaginal swabs did not reveal any pathogenic bacteria or fungi. Histologic examination of a biopsy of vulval lesional skin showed lichenoid lymphocytic infiltration of the papillary dermis and small, nonnecrotic epithelioid granulomas in the deep dermis (Fig. 1c,d). No microorganisms, including acid-fast bacilli or fungi, were identified. Culture was negative for fungi. Polymerase chain reaction was negative for the mycobacterial genome. Histologic examination of a biopsy from the upper lip showed similar results. The pathology reports of both regions were compatible with a diagnosis of granulomatous cheilitis and vulvitis. To investigate concomitant asymptomatic inflammatory bowel disease, the patient underwent colonoscopy with retrograde ileoscopy and gastroscopy, which were both negative. The patient refused radiographic examination of the small intestine with a barium meal. The patient was treated with systemic metronidazole (500 mg/day). After 6 months of therapy, the upper lip showed significant improvement and erythema and desquamation in the genital area showed slight improvement, but genital edema was unaffected.

Itch and burning pain in women with partial vaginismus with or without vulvar vestibulitis.

Fifty-three women with partial vaginismus with or without vulvar vestibulitis and 27 asymptomatic women estimated sensations of burning pain and itch at 20 standardized moments during a standardized penetration situation, including vaginal muscle contractions. Forty-three women with partial vaginismus (81.1%) reported burning pain, 23 (43.4%) itch, and 22 (41.5%) both complaints, compared to 0% of the asymptomatic women. In 17 of 22 cases, burning pain preceded the appearance of itch and in four cases the two complaints coincided. The median time from the moment when burning pain started until itch appeared was 150 seconds.

[Rosacea fulminans, pyostomatitis and pyovulvitis in Crohn's disease: dapsone as key factor in combination therapy]. / Rosacea fulminans, pyostomatitis und pyovulvitis bei morbus Crohn: dapson als entscheidender wirkstoff einer erfolgreichen kombinationstherapie?

Rosacea fulminans (also known as pyoderma faciale) has been reported to occur in association with Crohn's disease. It is still unclear whether the papulopustules and confluent nodules of rosacea fulminans represent a manifestation of mucocutaneous Crohn's disease or whether this association is a mere coincidence. A 46-year-old woman presented with the spontaneous outbreak of rosacea fulminans and pyostomatitis/pyovulvitis. Complete remission of the mucocutaneous symptoms was achieved with 2 months combination therapy with methylprednisolone, isotretinoin and dapsone. The patient's Crohn's disease, already diagnosed for 3 years, did not flare during this period.