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Kraurosis Vulvae

Kraurosis vulvae, now more commonly known as lichen sclerosus, is a chronic skin disease of unknown etiology that principally develops in the anogenital area, including the vulva. Pruritus, significant discomfort, burning pain, and the appearance of pale discoloration and atrophy of the skin accompanied by white coalescing papules and plaques with scarring are some of the main features. The diagnosis rests on clinical criteria, sometimes supported by a skin biopsy.


Presentation

Lichen sclerosus/Kraurosis vulvae is a chronic, potentially debilitating skin disease of undefined etiology, that is predominantly seen in women, although patients of both genders and all ages might be affected [1] [2]. Several studies have associated genetic factors (up to 12% of patients have a positive family history) [3], infectious agents (Borrelia burgdorferi and human papillomavirus, or HPV), autoimmune mechanisms (as 20-30% of women have been reported to suffer from an autoimmune disease) and mechanical irritation (having in mind the fact that Koebner phenomenon, ie. lesions appear after direct trauma) with Kraurosis vulvae [2] [4]. It is estimated that up to 1.7% of women are suffering from this condition [5]. The clinical presentation starts as a sharply defined erythema, followed by whitish (ivory-colored) discoloration of the vulva and white polygonal papules and plaques that exhibit a coalescing pattern [1] [6] [7]. Local edema is present in the majority of cases [1] [6]. As the skin becomes atrophic and fragile (one of the hallmarks of this disease), its rupture in the form of erosions, lacerations, and fissures is rather common, particularly during sexual intercourse [6]. Eventual progression of these lesions to scarring occurs, which may lead to significant damage to the vulvar structures (including the labia minora and the sealing of the clitoral hood) [6]. Most important symptoms, in addition to skin changes, are pruritus, a sensation of pain and soreness, dyspareunia, dysuria, and constipation [1] [2] [4] [5]. Kraurosis vulvae depict a relapsing and remitting clinical course, as targeted treatment is yet to be discovered [2].

Reiter's Syndrome
  • Namey 1 doctor agreed: 8 8 Does reiters syndrome cause dysmetria like symptoms in leg? Dr. Thomas Namey Dr. Namey 1 doctor agreed: Not true dysmetria![healthtap.com]
  • Ulcerative vulvitis in Reiter's syndrome. Br J Vener Dis 1982; 58: 405-7. Hazen HH. An anomalous case of white-spot disease. JAMA 1913; 61: 393-6. Brain RT. Lichen sclerosus et atrophicus. Br J Dermatol 1950; 62: 449-51.[cirp.org]
Dysmetria
  • Namey 1 doctor agreed: 8 8 Does reiters syndrome cause dysmetria like symptoms in leg? Dr. Thomas Namey Dr. Namey 1 doctor agreed: Not true dysmetria![healthtap.com]
Ataxia
  • It is a type of ataxia . It is sometimes described as an inability to judge distance or scale. However, symptoms like dysmetria can occur if you have pain in the feet, typical for reiter's. ...Read more See 1 more doctor answer Dr. Natalie Hodge Dr.[healthtap.com]
Vaginal Discharge
  • discharge Vaginitis Atrophic vaginitis Bacterial vaginosis Candidal vulvovaginitis Hydrocolpos Sexual dysfunction Dyspareunia Hypoactive sexual desire disorder Sexual arousal disorder Vaginismus Urogenital fistulas Ureterovaginal Vesicovaginal Obstetric[en.wikipedia.org]
  • A trichomoniasis vaginalis parasitic infection can lead to frothing, greenish-yellow, malodorous vaginal discharge . Women may have pain w. ...Read more See 1 more doctor answer Dr. Eric L. Weiss Dr.[healthtap.com]
  • Symptoms include pruritis, burning pain, dyspareunia, dysuria, vaginal discharge, anal or genital bleeding, 41, 51,52 labial stenosis or fusion, 53,54 and, especially in children, constipation. 55,56 Most cases reported in men occur on the glans penis[cirp.org]
Cervical Incompetence
  • incompetence Cervical polyp Cervicitis Female infertility Cervical stenosis Nabothian cyst General Hematometra / Pyometra Retroverted uterus Vagina Hematocolpos / Hydrocolpos Leukorrhea / Vaginal discharge Vaginitis Atrophic vaginitis Bacterial vaginosis[en.wikipedia.org]
Pruritus Vulvae
  • Dr. 1 doctor agreed: 14 14 What is the symptom of vulva itching (or pruritus vulvae( indicative of? Dr.[healthtap.com]
Vulvar Burning
  • Harris Sometimes : Vulvar burning and itching are very common and the skin can become very thin and have a whitish appearance sometimes. ...Read more Dr. Luis Villaplana Dr. Villaplana 11 11 Can vaginal infection mimic the symptoms of cystitis? Dr.[healthtap.com]

Workup

Clinical criteria are sufficient for the diagnosis of Kraurosis vulvae, implying that the role of a proper physical examination is essential [2]. Physicians should first obtain a detailed patient history, including the onset of symptoms and their progression, as well as the assessment of potential risk factors (family history, recent sexual intercourse that may have led to skin damage, and the presence of underlying autoimmune diseases). But as soon as the genital inspection is conducted, the identification of characteristic whitish skin, coalescing papules and plaques, atrophy, and scarring, the initial diagnosis of kraurosis vulvae must be suspected [2] [7]. In the case of an inconclusive clinical picture, a biopsy sample of the affected skin under local anesthesia and subsequent histopathological examination is recommended [2] [6]. However, the main histological features of kraurosis vulvae are nonspecific (luminal hyperkeratosis, acanthosis, infiltration of lymphocytes, thickening of the basement membrane, hypergranulosis of the adnexal structures, blood vessel dilation under the basement membrane and dermal edema) [6], and some authors recommend repeat biopsies in order to solidify the diagnosis [2]. Because of the limited role of biopsy, clinical signs and symptoms remain the cornerstone in confirming kraurosis vulvae.

Borrelia Burgdorferi
  • Several studies have associated genetic factors (up to 12% of patients have a positive family history), infectious agents (Borrelia burgdorferi and human papillomavirus, or HPV), autoimmune mechanisms (as 20-30% of women have been reported to suffer from[symptoma.com]
  • In addition to the research on the relation of Borrelia burgdorferi to scleroderma are many reports linking LS to this spirochete.[cirp.org]

Treatment

  • Please Note: The information provided here should not be used for diagnosis or treatment of any medical condition. A medical practitioner should always be consulted for diagnosis and treatment of all medical conditions.[hospitalhelp.co.uk]
  • (vulvectomy). gynecologist and treatment.[medicalformat.com]
  • Itching may be intense, but tends to lessen in time.One reason why suitable treatment is important is that, if untreated or improperly treated, there is the possibility that a cancerous condition may be developed from kraurosis vulvae.[disease-treatment-prevention.blogspot.com]
  • Estrogen containing creams are not sufficient for a complete long-term hormonal replacement and are only recommanded as an initial additional treatment.[link.springer.com]

Prognosis

  • Prognosis Prognosis is good for more acute genital cases of lichen sclerosus (LS), especially for those in the pediatric age group, in whom it may resolve spontaneously.[emedicine.medscape.com]
  • Prognosis Symptom remission can be achieved in 98% of compliant and 75% of non-compliant women by using potent topical steroids. [ 12 ] In males, a course of steroids may prevent the need for circumcision.[patient.info]

Etiology

  • Kraurosis vulvae, now more commonly known as lichen sclerosus, is a chronic skin disease of unknown etiology that principally develops in the anogenital area, including the vulva.[symptoma.com]
  • Definition (NCI) A chronic inflammatory disorder of unknown etiology that affects the vulva. It is characterized by the development of white elevated plaques in the vulva. Histologically there is marked subepithelial fibrosis.[fpnotebook.com]
  • The etiology and pathogenesis of lichen sclerosus (LS) is unknown but may include genetic, infectious, environmental, and hormonal factors.[emedicine.medscape.com]
  • Leukoplakic vulvitis and cancer of the vulva (etiology, histopathology, treatment, five-year results). Am J Obstet Gynecol 1929; 18: 472-503. Taussig FJ. Cancer of the vulva. Am J Obstet Gynecol 1940; 40: 764-79. Parks J.[cirp.org]

Epidemiology

  • Epidemiology The true incidence of LS is unknown and is probably underestimated.[patient.info]
  • Epidemiology Frequency Lichen sclerosus (LS) is a relatively common dermatosis, although the true prevalence of lichen sclerosus is unknown and likely underestimated.[emedicine.medscape.com]
  • However, firm epidemiological data are lacking for many reasons. Gynecologists and urologists do not generally perform a complete cutaneous examination, and dermatologists often exclude a genital examination.[cirp.org]
Sex distribution
Age distribution

Pathophysiology

  • Complications include pain, sexual and/or urinary dysfunction, reduced quality of life, and an increased risk of squamous cell carcinoma ( Pathophysiology Inflammation and altered fibroblast function in the papillary dermis lead to fibrosis of the upper[emedicine.medscape.com]

Prevention

  • Early detection and adequate therapy kraurosis vulva can prevent progression of the disease. In the process of development kraurosis vulva are three stages.[medicalformat.com]
  • These packs often allay inflammation and relieve itching. 2.Take a diet rich in vitamins, particularly vitamin A, which has considerable preventative value, and which may be curative in early cases. 3.If possible, consult a skin specialist, because there[disease-treatment-prevention.blogspot.com]
  • Estrogens with or without progestins should not be used for the prevention of cardiovascular disease or dementia.[pfizerpro.com]
  • Prognosis Symptom remission can be achieved in 98% of compliant and 75% of non-compliant women by using potent topical steroids. [ 12 ] In males, a course of steroids may prevent the need for circumcision.[patient.info]

References

Article

  1. Kunstfeld R, Kirnbauer R, Stingl G, Karlhofer FM. Successful treatment of vulvar lichen sclerosus with topical tacrolimus. Arch Dermatol. 2003;139(7):850-852.
  2. Kirtschig G. Lichen Sclerosus—Presentation, Diagnosis and Management. Dtsch Arztebl Int. 2016;113(19):337-343.
  3. Sherman V, McPherson T, Baldo M, Salim A, Gao XH, Wojnarowska F. The high rate of familial lichen sclerosus suggests a genetic contribution: an observational cohort study. J Eur Acad Dermatol Venereol. 2010;24(9):1031-1034.
  4. Powell JJ, Wojnarowska F. Lichen sclerosus. Lancet. 1999;353(9166):1777-1783.
  5. Doulaveri G, Armira K, Kouris A, Karypidis D, Potouridou I. Genital Vulvar Lichen Sclerosus in Monozygotic Twin Women: A Case Report and Review of the Literature. Case Rep Dermatol. 2013;5(3):321-325.
  6. Fistarol SK, Itin PH. Diagnosis and Treatment of Lichen Sclerosus: An Update. Am J Clin Dermatol. 2013;14(1):27-47.
  7. Drummond C. Common vulval dermatoses. Aust Fam Physician. 2011;40(7):490-496.

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Last updated: 2018-06-22 08:12