White Patches and Plaques: Lichen Sclerosus, Vitiligo, Postinflammatory Hypopigmentation and Lichenified Diseases
Lichen sclerosus (LS) is an uncommon skin disease that has a predilection for the genital skin. It is an important condition of the vulva with a reported prevalence between 1 in 300 and 1 in 1000 women and 1 in 900 girls.
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Gayle Fischer
34.1 Lichen Sclerosus 34.1.1 Introduction Lichen sclerosus (LS) is an uncommon skin disease that has a predilection for the genital skin. It is an important condition of the vulva with a reported prevalence between 1 in 300 and 1 in 1000 women and 1 in 900 girls.
34.1.2 Aetiology The true aetiology of LS remains unknown; however, there is a well-documented association with autoimmune disease, particularly thyroiditis with about 15% of patients having thyroid autoantibodies. Other associated autoimmune conditions include alopecia areata, vitiligo, pernicious anaemia and Addison’s disease. Antibodies to extracellular matrix protein 1 (ECM-1), and basement membrane zone (BMZ) components, have also been found in patients with LS, but the significance of it is unclear as there is no clinical correlation with the presence of these autoantibodies. Lichen sclerosus has been reported to run in families, and the HLA class II antigen HLA-DQ7 G. Fischer (*) Dermatology, Sydney Medical School Northern, The University of Sydney, Sydney, NSW, Australia e-mail: [email protected]
has the strongest association. Whilst these documented HLA associations are of interest, there remains a lack of clinical significance. Vulvar LS can affect the perianal region, clitoris, internal surface of the labia majora, labia minora and the vaginal introitus. Patients with significant involvement have a figure of eight patterns encircling the vagina and anus. Early and usually before there are significant symptoms, white polygonal papules with central indentation appear which over time coalesce into plaques. The typical plaque of LS develops a smooth, atrophic, porcelain-white, cigarettepaper surface. Oedema, telangiectasia, purpura and fissures may be seen at any time (Fig. 34.1). More severe inflammation leads to blistering with haemorrhagic bullae or erosions. With time, scarring appears with loss of vulval architecture including disappearance of the labia minora and clitoris (Fig. 34.2). The appearance in children is the same as in adults, and atrophy as well as loss of vulvar architecture also occurs in children. Symptomatically LS has a different presentation in children to adults. The average age of presentation is about 5 years of age and there is often a delay in diagnosis. Although, as in adults, itching and soreness are common, constipation and urinary symptoms are also a form of presentation that may result in children being referred to gastroenterology or urology. As in adults, some children are asymptomatic.
© Springer International Publishing AG, part of Springer Nature 2019 J. Bornstein (ed.), Vulvar Disease, https://doi.org/10.1007/978-3-319-61621-6_34
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Fig. 34.1 Lichen sclerosus: typical morphology with well demarcated, white wrinkled plaque with telangiectasia
The appearance of a well-defined white plaque with an atrophic wrinkled surface and areas of purpura and erosion is classical. With time and sometimes alarmingly quickly, change in the shape of the vulva occurs in ab
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