Fig. 19.1
The vulva with longer existing lichen sclerosus. The labia minora have almost completely disappeared. Hyperkeratotic area of the clitoris. Narrowing of the introitus. Excoriations on the vulva and red skin surrounding the introitus, as a consequence of scratching, also signs of lichenification (lichen simplex chronicus) as a result of scratching. Sometimes a superimposed yeast infection may be present, also presenting as red areas and symptoms of scratching
Fig. 19.2
Lichen sclerosus of the vulva. The labia minora have partly disappeared and have a white atrophic aspect. A small hemorrhage or fissure on the labium minor is on the left side. The skin of the vulva is red, with signs of lichenification, which may be a result of scratching or superponed yeast infection
19.4.2 What Is Known About the Course of Lichen Sclerosus in Women?
19.5 Etiology and Pathogenesis
19.5.1 What Is Known About the Etiology/Pathogenesis of Lichen Sclerosus?
The etiology of LS is unknown, but there is a strong association with autoimmune diseases. Thyroid disease, alopecia areata, vitiligo, and pernicious anemia are the most commonly seen. Genetic factors are implicated, and cases of familial lichen sclerosus have been reported [14]. An infective etiology has been postulated, but there are no clear data to show that lichen sclerosus is related to an infectious disease [15]. Women with genital LS can develop squamous cell carcinoma (SCC) of the vulva, although the risk seems to be very small (5 %) [16].
19.5.2 What Are the Effects and Impact of Lichen Sclerosus on Quality of Life and Sexuality in Women?
LS can have a huge impact on a woman’s quality of life [17–19]: for example, some have persistent itching and pain (despite medication) and in some women LS interferes with function (particularly sexual functioning) also despite successful control of the itching. Many affected women feel embarrassed and do not talk about these problems. We will discuss the itching and pain complaint in more detail.
19.5.2.1 Itch and Scratching
The prominence of “itching” as a symptom of LS quite often leads to patients being treated incorrectly for a yeast infection. In those women with itch, this is often worse at night and may be sufficiently severe to disturb sleep. Some women with vulvar LS have histologic evidence of epidermal thickening at the time of diagnosis. The thickening is believed to be related to superimposed lichen simplex chronicus (LSC). LSC is the lichenification that occurs as a result of scratching. Affected women report that “itching is much more distressing than the experience of pain.”
19.5.2.2 Pain
In those with pain during intercourse, the pain can be a consequence of (1) the erosions and fissures but can also be a direct consequence of (2) anatomical changes in the vulvar area, i.e., narrowing of the introitus and fusion of the labia minora, or (3) as a consequence of fear of pain. In the last case, the fear of pain during intercourse may result in decreased sexual arousal during sexual activity and thus in vaginal dryness and/or increased pelvic floor muscle tone (as a protective reaction to anticipated or actual pain). The combination of vaginal dryness and increased pelvic floor muscle tone cause friction between the penis and vulvar skin, which may result in pain and even tissue damage. Skin damage itself may result in pain or may further increase already existing pain. In some patients with LS, a provoked vestibulodynia (PVD) also is seen (see Chap. 18). Often it is not one factor that causes the pain, but a combination of the different factors as discussed earlier.
19.6 Specific Diagnostic Aspects
19.6.1 History Taking
The most common location of LS is the anogenital region. The clinical picture of LS is variable: complaint-free periods alternate with exacerbations. The course of the disease is chronic. Vulvar and/or perineal itching is the main symptom, but the absence of itching does not exclude LS. Furthermore, a burning sensation, dyspareunia, and dysuria are frequently reported symptoms. So history taking also includes specific questions related to sexual function (Table 19.1) [7].
Table 19.1
Clinical features of LS and diagnosis
|
Clinical features |
|
Symptoms |
|
Itch/irritation |
|
Pain (burning) |
|
Dyspareunia |
|
Urinary symptoms |
|
Constipation, can occur if there is perianal involvement |
|
Can be asymptomatic |
|
Physical signs |
|
Patchy pale, white atrophic areas affecting the vulva |
|
Purpura (ecchymosis) is common |
|
Erosions, but blistering is very rare |
|
Fissuring |
|
Hyperkeratosis can occur caused by itching |
|
Changes may be localized or in a “figure of eight” distribution including the perianal area |
|
Loss of architecture may be manifest as loss of the labia minora and/or midline fusion. The clitoral hood may be sealed over the clitoris so that it is buried |
|
Effects of LS |
|
Development of clitoral pseudocyst |
|
Development of squamous cell carcinoma estimated to be 3–5 % (actual risk uncertain but small) |
|
Sexual dysfunction |
|
Dysesthesia |
|
Diagnosis |
|
Characteristic clinical appearance |
|
Histology of vulval biopsya: thinned epidermis with subepidermal hyalinization and deeper inflammatory infiltrate. In early disease histology can be difficult |
Case History: Continued
Anne Salmon reports to have irregular pain during intercourse (half of the time). The pain is located at the entrance of the vagina and is described as a burning pain. During the periods of dyspareunia, she recognizes that her lubrication was diminished and that she is tensing her pelvic floor muscles in response to the pain. She has no problems with orgasm, except during the periods when penetration was so painful. Despite the pain she suffers sometimes, she forces herself to have intercourse because she finds it difficult to discuss the problem with her husband. During the periods of what she calls the “chronic yeast infections,” she is scratching a lot, mostly during the night. Furthermore, she reports that she is using regular vaginal douches (as she feels so dirty).
19.6.2 Physical Examination
In women, the entire vulva can be affected, but also the perineum (like a figure of eight). On inspection LS is characterized by hypopigmentation, fissures, hyperkeratotic areas, and ecchymoses. Small hemorrhages can occur spontaneously as a consequence of scratching. When scratching has taken place, excoriations are frequently seen in addition to small hemorrhages. When LS is present on the vulva and the anus, fissures can occur. With long-standing LS, an atrophic (parchment-like) and/or sclerotic picture can predominate. During the course of LS, the labia minora can disappear completely. Also fusion of the labia around the clitoris can lead to a hidden clitoral glans (fusion of the preputium) and narrowing of the vaginal introitus is seen. The vaginal mucosa is not involved in LS.
Extragenital lesions, characterized by hypopigmented maculae or papules/plaques that are accompanied by atrophy and or hyperkeratosis, can be found in 15–20 % of cases, particularly on the torso, upper legs, neck, and wrists. In rare cases, LS has been described as occurring on the head. Extragenital lesions are not generally linked with itching. In part, because of the sometimes look-a-like clinical picture, genital LS is considered a disorder in the same spectrum as lichen planus (LP). One important distinction is the involvement of the vaginal epithelium with LP, while, with LS, the vaginal epithelium is not affected. In addition, LP can occur mucosally (orally and vaginally), while LS does not affect the oral mucosa. Furthermore, the main symptom of LP is pain and not itch [20].
Case History: Continued
On inspection, fissures on the vulva and anus, hypopigmentation, atrophy of the labia minora, and some narrowing of the vaginal introitus are seen. Furthermore, some epidermal thickening of the labia majora and small hemorrhages are seen, which could be the consequence of regular scratching.
19.6.3 Diagnosis
The diagnosis of anogenital LS can generally be easily made on the basis of medical history and physical examination if it involves a classic presentation (see earlier section on Facts and Figures). A punch biopsy is recommended in the case of (1) diagnostic uncertainty or when there is a need to confirm the clinical diagnosis, (2) suspicion of neoplasia, and (3) inadequate response to cortisone creams.
Case History: Continued
The diagnosis of LS is made on the basis of medical history and physical examination. The hypothesis is that the itching is a symptom of LS and not a symptom of a chronic yeast infection (as the patient believed). The pain during intercourse seems to be related to more than one factor: (1) the fusing of the labia and narrowing of the introitus, (2) lesions related to the scratching, (3) decreased sexual arousal due to (imminent threat of) pain experience, and (4) increased pelvic muscle tension in response to the pain.
19.7 Specific Therapeutic Aspects
19.7.1 Which Treatment Options Are Evidence-Based in Women with Lichen Sclerosus?
There is no known cure for LS; however, part of the symptoms can be treated well. These include the relief of subjective symptoms (itching and pain) [12]. Some clinical signs may be reversed (i.e., the effects of chronic scratching LSC), but any scarring that has occurred will remain [21, 22]. It is possible that treatment may prevent malignant transformation, but there is no evidence for this hypothesis. The most frequent interventions for LS and its consequences on sexual functioning and how to cope with these consequences are briefly described next.
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