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50-450: LS can occur without symptoms. White patches on the LS body area, itching, pain, dyspareunia (in genital LS), easier bruising, cracking, tearing and peeling, as well as 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

100-482: A stratum corneum or a stratum granulosum ), sometimes specialized texts give slightly different definitions of hyperkeratosis in the context of mucosae. Examples are "an excessive formation of keratin (e.g., as seen in leukoplakia)" and "an increase in the thickness of the keratin layer of the epithelium, or the presence of such a layer in a site where none would normally be expected." The word hyperkeratosis ( / ˌ h aɪ p ər ˌ k ɛr ə ˈ t oʊ s ɪ s / )

150-504: A 2011 Cochrane review concluded that clobetasol propionate , mometasone furoate, and pimecrolimus (calcineurin inhibitor) all are effective therapies in treating genital lichen sclerosus. However, randomized-controlled trials are needed to further identify the optimal potency and regimen of topical corticosteroids, and assess the duration of remission and/or the prevention of flares patients experience with these topical therapies. Continuous use of appropriate doses of topical corticosteroids

200-443: A higher prevalence of other autoimmune diseases such as diabetes mellitus type 1 , vitiligo , alopecia areata , and thyroid disease. Both bacterial and viral pathogens have been implicated in the etiology of LS. A disease that is similar to LS, acrodermatitis chronica atrophicans is caused by the spirochete Borrelia burgdorferi . Viral involvement of HPV and hepatitis C are also suspected. A link with Lyme disease

250-625: A number of physical factors that may cause sexual discomfort. Pain is sometimes experienced in the testicular or glans area of the penis immediately after ejaculation . Infections of the prostate , bladder, or seminal vesicles can lead to intense burning or itching sensations following ejaculation. Patients with interstitial cystitis may experience intense penis pain at the moment of ejaculation. Gonorrheal infections are sometimes associated with burning or sharp penile pains during ejaculation. Urethritis or prostatitis can make genital stimulation painful or uncomfortable. Anatomic deformities of

300-437: A patient must experience the symptoms for at least six months and experience "significant distress". The differential diagnosis for dyspareunia is long because of its complicated and multifactorial nature. Often there are physiologic conditions underlying the pain, as well as psychosocial components that must be assessed to find appropriate treatment. A differential diagnosis of underlying physical causes can be guided by whether

350-468: A premalignant condition. In 1927, Kyrle defined LS ("white spot disease") as an entity sui generis. In 1928, Stühmer described balanitis xerotica obliterans as a postcircumcision phenomenon. In 1936, retinoids (vitamin A) were used to treat LS. In 1945, testosterone was used in genital LS. In 1961, the use of corticosteroids started. Jeffcoate presented an argument against vulvectomy for simple LS. In 1971, progesterone

400-408: A second study, of 129 patients, cancer occurred in 11% of patients, none of whom were fully compliant with corticosteroid treatment. Both those studies, however, also said that a corticosteroid as powerful as clobetasol is not necessary in most cases. In a prospective study of 83 patients, throughout 20 years, eight developed cancer. Six already had cancer at presentation and had not had treatment, while

450-436: A series of extragenital cases of LS. In 1892, Darier formally described the classic histopathology of LS. In 1900, the concept was formed that scleroderma and LS were closely related, which continues to this day. In 1901, pediatric LS was described. From 1913 to present, the concept that scleroderma is not closely related to LS also was formed. In 1920, Taussig established vulvectomy as the treatment of choice for kraurosis vulvae,

500-453: A sex disorder, with Charles Allen Moser , a physician, arguing for the removal of dyspareunia from the manual altogether. The most recent version, the DSM 5, has grouped dyspareunia under the diagnosis of Genito-Pelvic Pain/Penetration Disorder. Hyperkeratosis Hyperkeratosis is thickening of the stratum corneum (the outermost layer of the epidermis , or skin ), often associated with

550-434: A white plug of encrusted sebum . When called phrynoderma, the condition is associated with nutritional deficiency or malnourishment. This condition has been shown in several small-scale studies to respond well to supplementation with vitamins and fats rich in essential fatty acids . Deficiencies of vitamin E , vitamin A , and B-complex vitamins have been implicated in causing the condition. Follicular hyperkeratosis

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600-460: Is 2.6–5.8%". There is a bimodal age distribution in the incidence of LS in women. It occurs in females with an average age of diagnosis of 7.6 years in girls and 60 years old in women. The average age of diagnosis in boys is from 9 to 11 years old. In men, the most common age of incidence is 21 to 30. In 1875, Weir reported what was possible vulvar or oral LS was "ichthyosis." In 1885, Breisky described kraurosis vulvae. In 1887, Hallopeau described

650-405: Is a growing body of evidence suggesting that prolonged exposure of susceptible tissues to the irritant effects of urine may contribute to the development of lichen sclerosus. Urine droplets that leak after urination can become trapped in the external genitalia (e.g., beneath the foreskin), creating an occluded environment that exacerbates irritation and inflammation. Several observations support

700-516: Is a major factor in the severity of symptoms in some cases. Corticosteroids applied topically to the LS-affected skin are the first-line treatment for lichen sclerosus in both women and men, with strong evidence showing that they are "safe and effective" when appropriately applied, even over long courses of treatment, rarely causing serious adverse effects. They improve or suppress all symptoms for some time, with high variance across patients, until it

750-417: Is also a symptom in inherited collagen-related diseases of Ehlers-Danlos syndromes and Bethlem myopathy . The term hyperkeratosis is often used in connection with lesions of the mucous membranes , such as leukoplakia . Because of the differences between mucous membranes and the skin (e.g., keratinizing mucosa does not have a stratum lucidum and non keratinizing mucosa does not have this layer or normally

800-465: Is based on the Ancient Greek morphemes hyper- + kerato- + -osis , meaning 'the condition of too much keratin'. Nasodigitic hyperkeratosis in dogs may be idiopathic, secondary to an underlying disease, or due to congenital abnormalities in the normal anatomy of the nose and fingertips. In the case of congenital anatomical abnormalities, contact between the affected area and rubbing surfaces

850-437: Is impaired. It is roughly the same with finger pads — in animals with an anatomical abnormality, part of the pad is not in contact with rubbing surfaces and excessive keratin deposition is formed. The idiopathic form of nasodigitic hyperkeratosis in dogs develops from unknown causes and is more common in older animals (senile form). Of all dog breeds, Labradors, Golden Retrievers, Cocker Spaniels, Irish Terriers, Bordeaux Dogs are

900-409: Is more likely to develop skin cancer. Women with lichen sclerosus may develop vulvar carcinoma . Lichen sclerosus is associated with from 3 to 7% of all cases of vulvar squamous cell carcinoma. In women, it has been reported that 33.6 times higher vulvar cancer risk is associated with LS. A study in men noted that: "the reported incidence of penile carcinoma in patients with balanitis xerotica obliterans

950-596: Is more painful. Fear of being in pain can make the discomfort worse. Pain may continue despite the original source being removed, due to the learned expectation of pain. Fear, avoidance, and psychological distress around attempting intercourse can become large parts of the experience of dyspareunia. Physical examination of the vulva (external genitalia) may reveal clear reasons for pain including lesions, thin skin, ulcerations or discharge associated with vulvovaginal infections or vaginal atrophy. An internal pelvic exam may also reveal physical reasons for pain including lesions on

1000-485: Is not a diagnosis of itself. It is combined with vaginismus into genito-pelvic pain/penetration disorder in the DSM-5 . Criteria for genito-pelvic pain/penetration disorder include multiple episodes of difficulty with vaginal penetration, pain associated with intercourse attempts, anticipation of pain due to attempted intercourse, and tensing of the pelvis in response to attempted penetration. To meet criteria for this disorder,

1050-416: Is painful sexual intercourse due to somatic or psychological causes. The term dyspareunia covers both female dyspareunia and male dyspareunia, but many discussions that use the term without further specification concern the female type, which is more common than the male type. In females, the pain can primarily be on the external surface of the genitalia , or deeper in the pelvis upon deep pressure against

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1100-438: Is required to ensure symptoms remain relieved over the patient's life time. If continuously used, corticosteroids have been suggested to minimize the risk of cancer in various studies. In a prospective longitudinal cohort study of 507 women throughout six years, cancer occurred for 4.7% of patients who were only "partially compliant" with corticosteroid treatment, while it occurred in 0% of cases where they were "fully compliant". In

1150-485: Is required to use them again. Methylprednisolone aceponate has been used as a safe and effective corticosteroid for mild and moderate cases. For severe cases, it has been theorized that mometasone furoate might be safer and more effective than clobetasol. Recent studies have shown that topical calcineurin inhibitors such as tacrolimus can have an effect similar to corticosteroids, but its effects on cancer risks with LS are not conclusively known. Based on limited evidence,

1200-558: Is shown by the presence of Borrelia burgdorferi in LSA biopsy tissue. Since LS in females is primarily found in women with a low estrogen state (prepubertal and postmenopausal women), hormonal influences have been postulated. To date though, very little evidence has been found to support that theory. Some findings suggest that LS can be initiated through scarring or radiation, although those findings were sporadic and very uncommon. The disease often goes undiagnosed for several years, because it

1250-713: Is sometimes not recognized, and misdiagnosed as thrush or other problems, and not correctly diagnosed until the patient is referred to a specialist after the problem does not clear up. A biopsy of the affected skin can be done to confirm a diagnosis. When a biopsy is done, hyperkeratosis , atrophic epidermis, sclerosis of dermis and lymphocyte activity in dermis are histological findings associated with LS. The biopsies are also checked for signs of dysplasia . It has been noted that clinical diagnosis of balanitis xerotica obliterans can be "almost unmistakable," though there are other dermatologic conditions such as lichen planus , localized scleroderma , leukoplakia , vitiligo , and

1300-461: The DSM-IV , stated that the diagnosis of dyspareunia is made when the patient complains of recurrent or persistent genital pain before, during, or after sexual intercourse that is not caused exclusively by lack of lubrication or by vaginal spasm (vaginismus). After the text revision of the fourth edition of the DSM, a debate arose, with arguments to recategorize dyspareunia as a pain disorder instead of

1350-470: The cervix . Medically, dyspareunia is a pelvic floor dysfunction and is frequently underdiagnosed. It can affect a small portion of the vulva or vagina or be felt all over the surface. Understanding the duration, location, and nature of the pain is important in identifying the causes of the pain. Numerous physical, psychological, and social or relationship causes can contribute to pain during sexual encounters. Commonly, multiple underlying causes contribute to

1400-415: The intercellular matrix of the cells of the stratum corneum, promoting desquamation of scaly skin, eventually resulting in softening of hyperkeratotic areas. Follicular hyperkeratosis, also known as keratosis pilaris (KP), is a skin condition characterized by excessive development of keratin in hair follicles , resulting in rough, cone-shaped, elevated papules . The openings are often closed with

1450-400: The "urine occlusion hypothesis," including: Lichen sclerosus may have a genetic component. A high correlation of lichen sclerosus has been reported between twins and between family members. Autoimmunity is a process in which the body fails to recognize itself and therefore attacks its own cells and tissue. Specific antibodies have been found in LS sufferers. Furthermore, there seems to be

1500-406: The cervix or anatomic variation. When there are no visible findings on vulvar exam that would suggest a cause for superficial dyspareunia, a cotton-swab test may be performed. This is a test to assess for localized provoked vulvodynia . A cotton tip applicator is applied at several points around the opening of the vagina; the patient reports the resulting pain on a scale from 0–10. The cause of

1550-426: The condition, and uncomfortable levels of tension remain, a frenuloplasty procedure may be recommended. Frenuloplasty is an effective procedure, with a high chance of avoiding circumcision, giving good functional results and patient satisfaction. The psychological effects of these conditions, while little understood, are real, and are visible in literature and art. Dyspareunia is a condition that has many causes and

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1600-435: The cutaneous rash of Lyme disease can have a similar appearance. There is no definitive cure for LS. Behavior change is part of treatment. The patient should minimize or, preferably, stop scratching LS-affected skin. Any scratching, stress or damage to the skin can worsen the disease. Scratching has been theorized to increase cancer risks. Furthermore, the patient should wear comfortable clothes and avoid tight clothing, as it

1650-407: The disease and does not protect against the possibility of cancer. Circumcision does not prevent or cure LS. In fact, "balanitis xerotica obliterans" in men was first reported by Stühmer in 1928. as a condition affecting a set of circumcised men. Carbon dioxide laser treatment is safe and effective, and improves symptoms over the long term, but it does not lower cancer risks. Platelet-rich plasma

1700-441: The extreme rarity of serious adverse effects. With genital LS, appropriate lubrication should always be used before and during sex in order to avoid pain and the worsening the disease. Some oils, such as olive oil and coconut oil, can be used to accomplish both the emollient and sexual lubrication function. In males, it has been reported that circumcision can have positive effects, but does not necessarily prevent further flare-ups of

1750-402: The foreskin retracts on entry to the vagina irrespective of lubrication. In one study frenulum breve was found in 50% of patients who presented with dyspareunia. During vigorous or deep or tight intercourse or masturbation, small tears may occur in the preputial frenulum and can bleed and be very painful and induce anxiety, which can become chronic if left unresolved. If stretching fails to ease

1800-409: 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 ( phimosis ). 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

1850-400: The most prone to hyperkeratosis. Since the deposition of excess keratin cannot be stopped, therapy is aimed at softening and removing it. For moderate to severe cases, the affected areas should be hydrated (moisturised) with warm water or compresses for 5-10 minutes. Softening preparations are then applied once a day until the excess keratin is removed. In dogs with severe hyperkeratosis and

1900-808: 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. 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 US 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

1950-438: The ongoing pain LS sufferers experience. Although it is not clear what causes LS, several theories have been postulated. Lichen sclerosus is not contagious and cannot be caught from another person. Several risk factors have been proposed, including exposure to the irritant effects of urine, autoimmune diseases , infections and genetic predisposition . There is evidence that LS can be associated with thyroid disease . There

2000-456: The other two were not taking corticosteroids often enough. In all three studies, every single cancer case observed occurred in patients who were not taking corticosteroids as often as the study recommended. Continuous, abundant usage of emollients topically applied to the LS-affected skin is recommended to improve symptoms. They can supplement, but not replace, corticosteroid therapy. They can be used much more frequently than corticosteroids due to

2050-440: The pain help to understand potential causes and treatments. Some describe superficial pain at the opening of the vagina or surface of the genitalia when penetration is initiated. Others feel deeper pain in the vault of the vagina or deep within the pelvis upon deeper penetration. Some feel pain in more than one of these places. Determining whether the pain is more superficial or deep is important in understanding what may be causing

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2100-483: The pain is deep or superficial: The treatment for pain with intercourse depends on what is causing the pain. After proper diagnosis one or more treatments for specific causes may be necessary. For example: In addition, the following may reduce discomfort with intercourse: The word "dyspareunia" comes from Greek δυσ- , dys- "bad" and πάρευνος , pareunos "bedfellow", meaning "badly mated". The previous Diagnostic and Statistical Manual of Mental Disorders ,

2150-470: The pain may be anatomic or physiologic, including but not limited to lesions of the vagina, retroversion of the uterus , urinary tract infection, lack of lubrication, scar tissue, abnormal growths, or tender pelvic sites. Some cases may be psychosomatic, which can include fear of pain or injury, feelings of guilt or shame, ignorance of sexual anatomy and physiology, and fear of pregnancy. Common causes for discomfort on vaginal penetration include: There are

2200-419: The pain. Some patients have always experienced pain with intercourse from their very first attempt, while others begin to feel pain with intercourse after an injury or infection or cyclically with menstruation. Sometimes the pain increases over time. Pain may distract from feeling pleasure and excitement. Both vaginal lubrication and vaginal dilation decrease. When the vagina is dry and undilated, penetration

2250-635: The pain. The pain can be acquired or congenital . Symptoms of dyspareunia may also occur after menopause. Diagnosis is typically by physical examination and medical history . Underlying causes determine treatment. Many patients experience relief when physical causes are identified and treated. In 2020, dyspareunia has been estimated to globally affect 35% of women at some point in their lives. Those who experience pelvic pain upon attempted vaginal intercourse describe their pain in many ways. This reflects how many different and overlapping causes there are for dyspareunia. The location, nature, and time course of

2300-483: The penis, such as exist in Peyronie's disease , may also result in pain during coitus. One cause of painful intercourse is due to the painful retraction of a too-tight foreskin , occurring either during the first attempt at intercourse or subsequent to tightening or scarring following inflammation or local infection. Another cause of painful intercourse is due to tension in a short and slender frenulum, frenulum breve , as

2350-512: The presence of an abnormal quantity of keratin , and is usually accompanied by an increase in the granular layer. As the corneum layer normally varies greatly in thickness in different sites, some experience is needed to assess minor degrees of hyperkeratosis. It can be caused by vitamin A deficiency or chronic exposure to arsenic . Hyperkeratosis can also be caused by B-Raf inhibitor drugs such as Vemurafenib and Dabrafenib . It can be treated with urea-containing creams , which dissolve

2400-606: Was linked with Borrelia infection. Lichen sclerosus et atrophicus was first described in 1887 by François Henri Hallopeau . Since not all cases of lichen sclerosus exhibit atrophic tissue, the use of et atrophicus was dropped in 1976 by the International Society for the Study of Vulvovaginal Disease (ISSVD), officially proclaiming the name lichen sclerosus . Medical pictures Dyspareunia Dyspareunia ( / d ɪ s p ə r ˈ u n i ə / dis-pər-OO-nee-ə )

2450-423: Was reported to be effective in one study, producing large improvements in the patients' quality of life, with an average IGA improvement of 2.04 and DLQI improvement of 7.73. The disease can last for a considerably long time. Occasionally, "spontaneous cure" may occur, particularly in young girls. Lichen sclerosus is associated with a higher risk of cancer. Skin that has been scarred as a result of lichen sclerosus

2500-535: Was used in LS. Wallace defined clinical factors and the epidemiology of LS. In 1976, Friedrich defined LS as a dystrophic and not an atrophic condition, and "et atrophicus" was dropped. The International Society for Study of Vulvar Disease classification system established that "kraurosis" and "leukoplakia" were no longer to be used. In 1980, fluorinated and superpotent steroids were first used to treat LS. In 1981, studies into HLA serotypes and LS were published. In 1984, etretinate and acetretin were used in LS. In 1987, LS

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