History
Extragenital lichen sclerosus (LS) may be asymptomatic (approximately one third of cases) or it may itch or be tender. The most common complaint in vulvar lichen sclerosus is itch and sexual/urinary dysfunction in penile lichen sclerosus.
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.
Physical Examination
Pertinent physical findings are limited to the skin.
Skin primary lesion
Lichen sclerosus (LS) 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 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 usually are 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.
Skin distribution
Extragenital lesions may occur anywhere on the body, although the back and shoulders are reported most commonly. Note the image below:
Extragenital lichen sclerosus demonstrating coalescing pitted white papules. Courtesy of Wilford Hall Medical Center slide files.
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:
Lichen sclerosus demonstrating classic hourglass or figure 8 vulvar and perianal distribution. Courtesy of Wilford Hall Medical Center slide files.
More advanced vulvar lichen sclerosus; eroded areas need to be carefully examined and a biopsy sample should be taken to exclude coexistent squamous cell carcinoma. Courtesy of Wilford Hall Medical Center Dermatology slide files.
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:
Male genital lichen sclerosus may present with a sclerotic ring at the edge of the prepuce or anywhere on the glans itself. Advanced disease at the urethral os may lead to urinary obstruction. Courtesy of Wilford Hall Medical Center Dermatology Slide files.
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 has been described in extragenital cases. [19]
Complications
Following are male genital, female genital, and extragenital complications of lichen sclerosus (LS):
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Male genital: Complications can include painful erections, urinary obstruction, an inability to retract the foreskin, and squamous cell carcinoma (rare).
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Female genital: Complications include dyspareunia, urinary obstruction, secondary infection from chronic ulceration, secondary infection related to steroid use, and squamous cell carcinoma (rare, but not as rare as male cases). Some estimates are as high as 5% for the lifetime risk of vulvar squamous cell carcinoma in patients with lichen sclerosus. [20] The epidemiology of lichen sclerosus patients who develop squamous cell carcinoma shows that older age, longer duration of lichen sclerosus, and evidence of hyperplastic/early vulvar carcinoma in situ changes to be significant risk factors. A 2009 study linked coexistent chlamydia infection and lichen sclerosus with increased risk of squamous cell carcinoma, but other sexually transmitted illnesses, such as human papillomavirus infection, were not completely addressed. [21]
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Extragenital: Complications include cosmetic concerns, but only in extensive cases.
The following issues also may arise:
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Incomplete diagnosis: The patient may have lichen sclerosus, but a persistently ulcerated area or an area not responsive to therapy may be a malignancy. Another biopsy or additional biopsies may be necessary.
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Inappropriate surveillance: Repeatedly refilling a patient's topical corticosteroids without reexamining them may allow a malignancy to spread or may allow steroid adverse effects to develop.
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Child abuse issues: Lichen sclerosus, especially when bullous and hemorrhagic or erosive, may be confused with child abuse. On the other hand, one case report suggested lichen sclerosus either coexisted with child abuse or was related to the trauma associated with the repeated sexual attacks.
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Suboptimal therapy: Topical testosterone, despite the extensive literature describing its use, may not be more effective than placebo and can be associated with virilization.
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New problem: Allergic contact dermatitis may develop with any topical therapy, including steroids. Irritant dermatitis may likewise develop. Consider these when a patient who previously was doing well suddenly seems to worsen.
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Lichen sclerosus demonstrating classic hourglass or figure 8 vulvar and perianal distribution. Courtesy of Wilford Hall Medical Center slide files.
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Extragenital lichen sclerosus demonstrating coalescing pitted white papules. Courtesy of Wilford Hall Medical Center slide files.
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Typical lichen sclerosus histology demonstrating homogenized edematous papillary (upper) dermis and effaced epidermis.
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More advanced vulvar lichen sclerosus; eroded areas need to be carefully examined and a biopsy sample should be taken to exclude coexistent squamous cell carcinoma. Courtesy of Wilford Hall Medical Center Dermatology slide files.
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Male genital lichen sclerosus may present with a sclerotic ring at the edge of the prepuce or anywhere on the glans itself. Advanced disease at the urethral os may lead to urinary obstruction. Courtesy of Wilford Hall Medical Center Dermatology Slide files.
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Late lichen sclerosus may show less edema in the upper dermis and more sclerosis throughout the dermis. Involvement of the lower dermis or fat may occur in lichen sclerosus/scleroderma overlap presentations.
