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Kraurosis vulvae is a cutaneous condition characterized by atrophy and shrinkage of the skin of the vagina and vulva often accompanied by a chronic inflammatory reaction in the deeper tissues.
Vulvitis is inflammation of the vulva, the external female mammalian genitalia that include the labia majora, labia minora, clitoris, and introitus (the entrance to the vagina). It may co-occur with vaginitis, inflammation of the vagina, and may have infectious or non-infectious causes.
LS can occur without symptoms. White patches on the LS body area, itching, pain, pain during sex (in genital LS), easier bruising, cracking, tearing and peeling, and hyperkeratosis are common symptoms in both men and women. In women, the condition most commonly occurs on the vulva and around the anus with ivory-white elevations that may be flat and glistening.
In males, the disease may take the form of whitish patches on the foreskin and its narrowing (preputial stenosis), forming an "indurated ring", which can make retraction more difficult or impossible. In addition there can be lesions, white patches or reddening on the glans. In contrast to women, anal involvement is less frequent. Meatal stenosis, making it more difficult or even impossible to urinate, may also occur.
On the non-genital skin, the disease may manifest as porcelain-white spots with small visible plugs inside the orifices of hair follicles or sweat glands on the surface. Thinning of the skin may also occur.
Vulvitis, inflammation of the vulva, can have a variety of etiologies in children and adolescents, including allergic dermatitis, contact dermatitis, lichen sclerosus, and infections with bacteria, fungi, and parasites. Dermatitis in infants is commonly caused by a soiled diaper being left on for an extended period of time. Increasing the frequency of diaper changes and topical application of emollients are sufficient to resolve most cases. Dermatitis of the vulva in older children is usually caused by exposure to an irritant (e.g. scented products that come into contact with the vulva, laundry detergent, soaps, etc.) and is treated with preventing exposure and encouraging sitz baths with baking soda as the vulvar skin heals. Other treatment options for vulvar dermatitis include oral hydroxyzine hydrochloride or topical hydrocortisone.
Lichen sclerosus is another common cause of vulvitis in children, and it often affects an hourglass or figure eight-shaped area of skin around the anus and vulva. Symptoms of a mild case include skin fissures, loss of skin pigment (hypopigmentation), skin atrophy, a parchment-like texture to the skin, dysuria, itching, discomfort, and excoriation. In more severe cases, the vulva may become discolored, developing dark purple bruising (ecchymosis), bleeding, scarring, attenuation of the labia minora, and fissures and bleeding affecting the posterior fourchette. Its cause is unknown, but likely genetic or autoimmune, and it is unconnected to malignancy in children. If the skin changes are not obvious on visual inspection, a biopsy of the skin may be performed to acquire an exact diagnosis. Treatment for vulvar lichen sclerosus may consist of topical hydrocortisone in mild cases, or stronger topical steroids (e.g. clobetasol propionate). Preliminary studies show that 75% of cases do not resolve with puberty.
Organisms responsible for vulvitis in children include pinworms ("Enterobius vermicularis"), "Candida" yeast, and group A hemolytic "Streptococcus". Though pinworms mainly affect the perianal area, they can cause itching and irritation to the vulva as well. Pinworms are treated with albendazole. Vulvar "Candida" infections are uncommon in children, and generally occur in infants after antibiotic therapy, and in children with diabetes or immunodeficiency. "Candida" infections cause a red raised vulvar rash with satellite lesions and clear borders, and are diagnosed by microscopically examining a sample treated with potassium hydroxide for hyphae. They are treated with topical butoconazole, clotrimazole, or miconazole. "Streptococcus" infections are characterized by a dark red discoloration of the vulva and introitus, and cause pain, itching, bleeding, and dysuria. They are treated with antibiotics.
Distress due to the discomfort and pain of Lichen Sclerosus is normal, as are concerns with self-esteem and sex. Counseling can help.
According to the National Vulvodynia Association, which also supports women with Lichen Sclerosus, vulvo-vaginal conditions can cause feelings of isolation, hopelessness, low self-image, and much more. Some women are unable to continue working or have sexual relations and may be limited in other physical activities. Depression, anxiety, and even anger are all normal responses to the ongoing pain LS patients suffer from.
Labial fusion is never present at birth, but rather acquired later in infancy, since it is caused by insufficient estrogen exposure and newborns have been exposed to maternal estrogen "in utero". It typically presents in infants at least 3 months old. Most presentations are asymptomatic and are discovered by a parent or during routine medical examination. In other cases, patients may present with associated symptoms of dysuria, urinary frequency, refusal to urinate, or post-void dribbling. Some patients present with vaginal discharge due to pooling of urine in the vulval vestibule or vagina.
VVS is characterized by severe pain with attempted penetration of the vaginal orifice and complaints of tenderness with pressure within the vulval vestibule. Usually there are no reports of pain with pressure to other surrounding areas of the vulva. The feelings of irritation and burning can persist for hours or days following sexual activity, engendering a sense of hopelessness and depression. VVS also can often cause dyspareunia.
The pain may be provoked by contact with an object, such as with the insertion of a tampon or penis or with the pressure from sitting on a bicycle seat, "provoked vestibulodynia", or it may be constant, as in the case of unprovoked, generalized vestibulodynia. Some women have had pain since their first penetration (primary vulvar vestibulitis) while some have had it after a period of time with pain-free penetration (secondary vulvar vestibulitis).
Relationship problems often occur as the result of chronic frustration, disappointment, and depression associated with the condition.
Genital symptoms include dryness, itching, burning, soreness, pressure, white discharge, malodorous discharge due to infection, painful sexual intercourse, bleeding after intercourse. In addition, sores and cracks may occur spontaneously. Atrophic vaginitis is one possible cause of postmenopausal bleeding (PMB).
Urinary symptoms include painful urination, blood in the urine, increased frequency of urination, incontinence, and increased likelihood and occurrence of infections.
Pain is the most notable symptom of vulvodynia, and can be characterized as a burning, stinging, irritation or sharp pain that occurs in the vulva and entrance to the vagina. It may be constant, intermittent or happen only when the vulva is touched, but vulvodynia is usually defined as lasting for years.
Symptoms may occur in one place or the entire vulvar area. It can occur during or after sexual activity, when tampons are inserted, or when prolonged pressure is applied to the vulva, such as during sitting, bike riding, or horseback riding. Some cases of vulvodynia are idiopathic where no particular cause can be determined.
The term Vulvar intraepithelial neoplasia (VIN) refers to particular changes that can occur in the skin that covers the vulva. VIN is not cancer, and in some women it disappears without treatment. If the changes become more severe, there is a chance that cancer might develop after many years, and so it is referred to as a precancerous condition.
Labial fusion is a medical condition of the female genital anatomy where the labia minora become fused together. It is generally a pediatric condition.
The condition is known by a number of names, including labial fusion, labial adhesion, labial synechiae, labial agglutination, labial adherence, gynatresia, vulvar fusion, and vulvar synechiae.
Diagnosis is readily made by the cotton-swab test, in which pressure is applied in a circular fashion around the vulvar vestibule to assess complaints of pain. Laboratory tests are used to exclude bacterial or viral infection, and a careful examination of the vulvo/vaginal area is conducted to assess whether any atrophy is present.
Vulvar vestibulitis syndrome (VVS), vestibulodynia, or simply vulvar vestibulitis or "localized (to the vestibule) provoked vulvodynia" refers to pain localized to the vestibular region. It tends to be associated with a highly localized "burning" or "cutting" type of pain. The pain of vulvodynia may extend into the clitoris; this is referred to as clitorodynia.
Vulvar vestibulitis syndrome is the most common subtype of vulvodynia that affects premenopausal women – the syndrome has been cited as affecting about 10%–15% of women seeking gynecological care.
Oral lichen planus (also termed "oral mucosal lichen planus"), is a form of mucosal lichen planus, where lichen planus involves the oral mucosa, the lining of the mouth. This may occur in combination with other variants of lichen planus. Six clinical forms of oral lichen planus are recognized:
- "Reticular", the most common presentation of oral lichen planus, is characterised by the net-like or spider web-like appearance of lacy white lines, oral variants of Wickham's straiae. This is usually asymptomatic.
- "Erosive/ulcerative", the second most common form of oral lichen planus, is characterised by oral ulcers presenting with persistent, irregular areas of redness, ulcerations and erosions covered with a yellow slough. This can occur in one or more areas of the mouth. In 25% of people with erosive oral lichen planus, the gums are involved, described as desquamative gingivitis (a condition not unique to lichen planus). This may be the initial or only sign of the condition.
- "Papular," with white papules.
- "Plaque-like" appearing as a white patch which may resemble leukoplakia.
- "Atrophic," appearing as areas. Atrophic oral lichen planus may also manifest as desquamative gingivitis.
- "Bullous," appearing as fluid-filled vesicles which project from the surface.
These types often coexist in the same individual. Oral lichen planus tends to present bilaterally as mostly white lesions on the inner cheek, although any mucosal site in the mouth may be involved. Other sites, in decreasing order of frequency, may include the tongue, lips, gingivae, floor of the mouth, and very rarely, the palate.
Generally, oral lichen planus tends not to cause any discomfort or pain, although some people may experience soreness when eating or drinking acidic or spicy foodstuffs or beverages. When symptoms arise, they are most commonly associated with the atrophic and ulcerative subtypes. These symptoms can include a burning sensation to severe pain. Lichen planus, particularly when concomitant oral or genital lesions occur, significantly affects patients’ quality of life.
Bowenoid papulosis is a cutaneous condition characterized by the presence of pigmented verrucous papules on the body of the penis. They are associated with human papillomavirus, the causative agent of genital warts. The lesions have a typical dysplastic histology and are generally considered benign, although a small percentage will develop malignant characteristics.
It is considered as a pre-malignant condition. Other terms used to describe the condition are: Erythroplasia of Queyrat, Squamous cell carcinoma in situ and Bowen’s disease. The term "Bowenoid papulosis" was coined in 1977 by Kopf and Bart and is named after dermatologist John Templeton Bowen.
The term “intraepithelial neoplasia” defines a premalignant intraepithelial change.
On the vulva it is termed VIN (vulvar or vulval intraepithelial neoplasia); on the penis, PIN (penile intraepithelial neoplasia); and on or around the anus, AIN (anal intraepithelial neoplasia). The terminology has been very confusing and it is now recommended that the terms Bowen’s disease, erythroplasia of Queyrat, and bowenoid papulosis should not be used for lesions in the anogenital area. However, dermatologists still recognize a distinct clinical variant, bowenoid papulosis, characterized by discrete papules in a younger age group and a tendency for spontaneous regression. Additionally, some authorities believe that erythroplasia of Queyrat and Bowen’s disease remain useful terms in men.
Lichen planus affecting mucosal surfaces may have one lesion or be multifocal. Examples of lichen planus affecting mucosal surfaces include:
- "Esophageal lichen planus", affecting the esophageal mucosa. This can present with difficulty or pain when swallowing due to oesophageal inflammation, or as the development of an esophageal stricture. It has also been hypothesized that it is a precursor to squamous cell carcinoma of the esophagus.
- "Genital lichen planus," which may cause lesions on the glans penis or skin of the scrotum in males, and the vulva or vagina in females. Symptoms may include lower urinary tract symptoms associated with stenosis of the urethra, painful sexual intercourse, and itching. In females, "Vulvovaginal-gingival syndrome," is severe and distinct variant affecting the vulva, vagina, and gums, with complications including scarring, vaginal stricture formation, or vulva destruction. The corresponding syndrome in males, affecting the glans penis and gums, is the "peno-gingival syndrome". It is associated with HLA-DQB1.
Medically speaking, the term denotes a squamous intraepithelial lesion of the vulva that shows dysplasia with varying degrees of atypia. The epithelial basement membrane is intact and the lesion is thus not invasive but has invasive potential.
The terminology of VIN evolved over several decades. In 1989 the Committee on Terminology, International Society for the Study of Vulvar Disease (ISSVD) replaced older terminology such as vulvar , Bowen's disease, and Kraurosis vulvae by a new classification system for "Epithelial Vulvar Disease":
- Nonneoplastic epithelial disorders of vulva and mucosa:
- Lichen sclerosus
- Squamous hyperplasia
- Other dermatoses
- Mixed neoplastic and nonneoplastic disorders
- Intraepithelial neoplasia
- Squamous vulvar intraepithelial neoplasia (VIN)
- VIN I, mildest form
- VIN II, intermediate
- VIN III, most severe form including carcinoma in situ of the vulva
- Non-squamous intraepithelial neoplasia
- Extramammary Paget's disease
- Tumors of melanocytes, noninvasive
- Invasive disease (vulvar carcinoma)
The ISSVD further revised this classification in 2004, replacing the three-grade system with a single-grade system in which only the high-grade disease is classified as VIN.
VIN is subdivided into: (Robbins Pathological Basis of Disease, 9th Ed)
Classic vulvular intraepithelial neoplasia: associated with developing into the warty and basaloid type carcinoma. This is associated with carcinogenic genotypes of HPV and/or HPV persistence factors such as cigarette smoking or immunocompromised states.
Differentiated vulvar intraepithelial neoplasia also known as VIN Simplex: is associated with vulvar dermatoses such as lichen sclerosus. It is associated with atypia of the squamous epithelium.
Atrophic vaginitis (also known as vaginal atrophy, vulvovaginal atrophy, or urogenital atrophy) is an inflammation of the vagina (and the outer urinary tract) due to the thinning and shrinking of the tissues, as well as decreased lubrication. These symptoms are due to a lack of the reproductive hormone estrogen.
The most common cause of vaginal atrophy is the decrease in estrogen which happens naturally during perimenopause, and increasingly so in post-menopause. However this condition can occur in other circumstances that result in decreased estrogen such as breastfeeding and the use of medications intended to decrease estrogen too, for example, treat endometriosis.
The symptoms can include vaginal soreness and itching, as well as painful intercourse, and bleeding after sexual intercourse. The shrinkage of the tissues and loss of flexibility can be extreme enough to make intercourse impossible.
In some instances nodular angiokeratomas can produce necrotic tissue and valleys that can harbor fungal, bacterial and viral infections. Infections can include staphylococcus. If the lesion becomes painful, begins draining fluids or pus, or begins to smell, consult a physician. In these instance a doctor may recommend excision and grafting.
People burdened with LSC report pruritus, followed by uncontrollable scratching of the same body region, excessively. Most common sites of LSC are the sides of the neck, the scalp, ankles, vulva, pubis, scrotum, and extensor sides of the forearms. However, due to the stigma associated with chronic scratching, some patients will not admit to chronic rubbing or abrasion. The skin may become thickened and hyperpigmented (lichenified) as a direct result of chronic exoriation. Typically this period of increased scratching is associated with stressors.
Presentation includes telangiectasia, acanthosis, and hyperkeratosis.
Presentation can be solitary or systemic.
Linear focal elastosis (also known as "Elastotic striae") presents with asymptomatic, palpable or atrophic, yellow lines of the middle and lower back, thighs, arms and breasts.
Warty dyskeratoma, also known as an Isolated dyskeratosis follicularis, is a benign epidermal proliferation with distinctive histologic findings that may mimic invasive squamous cell carcinoma and commonly manifests as an umbilicated (Having a central mark or depression resembling a navel) lesion with a keratotic plug, WD have some histopathologic similarities to viral warts but it's not caused by HPV and the majority of these lesions display overall histopathologic features consistent with a follicular adnexal neoplasm. usually limited to the head, neck, scalp or face and vulva. Lesions are generally and sporadic and may be associated with a follicular unit. Oral involvement, particularly the hard palate, and genital involvement have been reported. it can also be thought of as one of the manifestations of focal acantholytic dyskeratosis, an epidermal reaction pattern that can be seen in several disorders, including Darier's disease and Grover's disease. But the main Difference between Darier disease and Warty dyskeratoma, is that Darier disease inherited dermatosis (autosomal dominant) consisting of multiple keratotic papules on the face, trunk, and extremities, while WD occurs as an isolated, noninherited, single keratotic nodule mainly confined to the head and neck as mentioned earlier.
Vulvovaginal health is the health and sanitation of the human vulva and vagina.
Problems affecting this area include vulva diseases, vaginal diseases and urinary tract infections.
Warty dyskeratoma must be differentiated from vulvar dysplasia, Bowenoid papulosis, squamous carcinoma, condyloma, and other viral-induced squamous lesions.