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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].

Burning Pain
  • 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.[symptoma.com]
  • Rowlett Not really: These infections can cause urinary symptoms (burning, pain) but not typically associated with infection per se. ...Read more Dr. Cynthia Frazier Dr. Frazier 4 4 What are the causes of pruritus vulvae or vulva itching? Dr.[healthtap.com]
Localized Edema
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]
Falling
  • Mathews 6 doctors agreed: Pelvic pressure: Pelvic pressure, pain, feeling like something is falling out of the vagina, constipation, incomplete emptying of the bladder. ...Read more See 2 more doctor answers Dr.[healthtap.com]
Skin Lesion
  • In advanced cases, the papules tend to coalesce (blend) into large, white patches of thin, itchy skin. Lesions often occur on the torso and, almost invariably, in the anogenital (relating to both the anal and the genital) regions.[healthcentral.com]
  • In some rare cases, skin lesions may also develop in the mouth. The lesions consist of bluish-white flat patchy areas on the inside of the cheeks and/or palate. The tongue may also be involved.[rarediseases.org]
Koebner Phenomenon
  • phenomenon, ie. lesions appear after direct trauma) with Kraurosis vulvae.[symptoma.com]
Irritability
  • […] 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 an autoimmune disease) and mechanical irritation[symptoma.com]
  • Any vaginal discharge or leakage of urine can irritate the vulva. Scratching can cause tissue irritation and is counterproductive. Contact dermatitis is also very common on the vulva.Best to see a specialist for evaluation.[healthtap.com]
  • Creams were applied with testosterone propionate (.2-.3% concentration) or metiltestosterone (.1-.25% concentration) resulting in a decrease of itching and improvement of skin irritation and atrophies.[popline.org]
  • […] immunoglobulin, colostrum’s immune factors are highly effective antibacterial and antiviral agents which can help to reduce, eliminate and control infections in external injuries. by Julia Busch — Real relief for vaginal thinning, burning, itching, irritation[naturalhealingnews.com]
  • Advice to patients: Wash with bland emollients - eg, aqueous cream; avoid topical irritants and tight clothing; use lubricants if necessary; give details of support groups.[patient.info]
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]
Pruritus Vulvae
  • Dr. 1 doctor agreed: 14 14 What is the symptom of vulva itching (or pruritus vulvae( indicative of? Dr.[healthtap.com]
Vaginal Discharge
  • 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]
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]
Pruritus Vulvae
  • Dr. 1 doctor agreed: 14 14 What is the symptom of vulva itching (or pruritus vulvae( indicative of? 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]

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]
  • 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]
  • (vulvectomy). gynecologist and treatment.[medicalformat.com]

Prognosis

  • 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]

Epidemiology

  • Epidemiology The true incidence of LS is unknown and is probably underestimated.[patient.info]
  • Balanitis xerotica obliyerans: epidemiologic distribution in an equal access health care system. South Med J. 2003;96:9-11. Tasker GL, Wojnarowska F. Lichen sclerosus. Clin Exp Dermatol. 2003;28:128-33. Neill SM, Tatnall FM, Cox NH.[rarediseases.org]
Sex distribution
Age distribution

Pathophysiology

  • Appropriately, many new sections have been added and many of the older sections are remodelled to accommodate the major changes in pathophysiology and management of gynaecological disorders.[books.google.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]
  • Doctors may also deliberately suppress the immune system before organ or stem cell transplants to prevent rejection.[cancer.ca]
  • 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