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 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]

• Localized Edema

  Local edema is present in the majority of cases. [symptoma.com]

• Sore Throat

  Most common are sore throat, fever, a non- itchy skin rash of red spots, and enlarged lymph nodes -- plus others. Most people with these symptoms have other viruses or non-infectious problems, even if at risk for HIV. [healthtap.com]

• Koebner Phenomenon

  phenomenon, ie. lesions appear after direct trauma) with Kraurosis vulvae. [symptoma.com]

  Bilateral Koebner phenomenon in lichen sclerosus et atrophicus. Int J Dermatol 1987; 26: 117-8. Gross P. Generalized lichen sclerosus et atrophicus associated with band-like scleroderma (Koebner phenomenon and lichen sclerosus). [cirp.org]

  Regular use of super-potent/potent topical corticosteroids after vulvar surgery is essential to prevent flares of lichen sclerosus (given the koebnerization phenomenon) and to minimize the risk of re-agglutination and stenosis. [dermatologyadvisor.com]

• Impulsivity

  The cause of the itch receptors are changes in the vulva, causing disturbances in conducting nerve impulses. [medicalformat.com]

• 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

  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]

• 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]

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]

  A possible role for Borrelia burgdorferi infection in the etiology of extragenital lichen sclerosus has been suggested in Europe based on polymerase chain reaction (PCR) amplification data. [dermatologyadvisor.com]

  In addition to the research on the relation of Borrelia burgdorferi to scleroderma are many reports linking LS to this spirochete. [cirp.org]

• Lymphocytic Infiltrate

  This homogenized collagen typically sits above the lymphocytic infiltrate. Use of topical corticosteroids results in normalization of dermal collagen hyalinization and resolution of the lymphocytic inflammation (Figure 6). Figure 6. [dermatologyadvisor.com]

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.