Kraurosis Vulvae

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].

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.

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.

• Kraurosis Vulvae
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