Lichen Sclerosus et Atrophicus Clinical Presentation
- Author: Jeffrey Meffert, MD; Chief Editor: William D James, MD more...
Extragenital lichen sclerosus may be asymptomatic or it may itch, although itching is not usual. Vulvar lichen sclerosus usually presents with progressive pruritus, dyspareunia, dysuria, or genital bleeding. Penile lichen sclerosus usually is preceded by pruritus but may present with sudden phimosis of previously retractable foreskin, and urinary obstruction can result.
Pertinent physical findings are limited to the skin.
Skin primary lesion
Lichen sclerosus usually begins as white, polygonal papules that coalesce into plaques. Evenly spaced dells or comedolike plugs correspond to obliterated appendiceal ostia. These may be easily identified with dermoscopy, keeping in mind that other conditions such as chronic cutaneous lupus may also show follicular plugs. With time, the plugs and dells will disappear and leave a smooth, porcelain-white plaque. Skin color is white, often with a shiny porcelain appearance. Telangiectases and follicular plugs may be seen. The size of the plaque or plaques may vary widely from a few millimeters resembling lichen nitidus to the entire upper trunk.
Vulvar lichen sclerosus may progress to gradual obliteration of the labia minora and stenosis of the introitus. The most common variation occurs when the inflammation is intense enough to cause separation of a large area of epidermis, creating blisters or large, occasionally hemorrhagic, bullae. Because this occurs more often in genital cases, it may be confused with the trauma of sexual abuse or other genital ulcerative disease.
Given the high frequency of genital mucosal disease, it is surprising that more oral cases have not been reported. Those rare cases reported are usually seen in patients with widespread, generalized lichen sclerosus. Some believe that many cases of clinically diagnosed lichen planus may actually be lichen sclerosus and that isolated oral mucosal lichen sclerosus may not be as rare as is thought.
Extragenital lesions may occur anywhere on the body, although the back and shoulders are reported most commonly. Note the image below:
Female genital lesions may be confined to the labia majora but usually involve and eventually obliterate the labia minora and stenose the introitus. Often, an hourglass, butterfly, or figure-8 pattern involves the perivaginal and perianal areas, with minimal involvement of the perineum in between. Note the images below:
Male genital lesions usually are confined to the glans penis and the prepuce or foreskin remnants. Penile shaft involvement is much less common, and scrotal involvement is rare. The initial manifestation may be a sclerotic ring at the prepuce edge. Note the image below:
The isomorphic (Koebner) phenomenon is described in this condition, with the resultant lesions in old surgical scars, burn scars, sunburned areas, and areas subject to repeated trauma. Distribution of lichen sclerosus along the lines of Blaschko was described in an extragenital case.
The etiology and pathogenesis of lichen sclerosus is unknown but may include genetic, infectious, environmental, and hormonal factors.
Several older studies have linked borrelial or other infections with lichen sclerosus, yet most other studies have disputed this, with polymerase chain reaction–based studies showing no increased incidence of borrelial infection.
Genetic and autoimmune factors have been explored without identification of consistent, reproducible patterns, although autoantibodies to ECM1 have now been reported by several independent authors. Increased circulating autoantibodies may be as high as 28%, comparable to the rate seen in bullous lichen planus. Baldo et al reported that more than 40% of vulvar lichen sclerosus and lichen planus patients have reactive T cells to the NC16A domain of bullous pemphigoid antigen 180 ; however, other studies suggest that the level of autoantibodies is poorly correlated to disease activity and response to treatment. Women with lichen sclerosus have a higher rate of associated autoimmune disease (odds ratio, 4.3), especially for autoimmune thyroid disease, compared with men.
Local irritation or trauma seems to play a role in some cases of lichen sclerosus, especially in genetically predisposed individuals. However, the sequence of events that leads to the altered fibroblast function, microvascular changes, and hyaluronic acid accumulation in the upper dermis continues to be researched.
Oral contraceptives in premenopausal women have been shown to give a relative risk of 2.5, which suggests an altered hormonal axis as a possible contributory factor.
Lichen sclerosus may occur in association with other inflammatory conditions including psoriasis. This is surprising because the histopathological findings of lichen sclerosus and psoriasis are so dissimilar.
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