Lichen Sclerosus

CHAPTER 14 Lichen Sclerosus



Lichen sclerosus is the classic, pruritic, chronic dermatosis of the postmenopausal vulva.



Epidemiology and clinical manifestations


Lichen sclerosus is recognized most often on the vulva of postmenopausal women, with prepubertal girls representing a significant minority. Lichen sclerosus is seen in all age groups, but it is often less symptomatic in premenopausal and postpubertal females who do not have the additive effect of an atrophic, hypoestrogenic vagina. Lichen sclerosus occurs very occasionally on the glans penis of men, and it occurs rarely as an isolated finding on extragenital skin in either gender.


Lichen sclerosus is a common disease, reported in about 3% of incontinent women in a nursing-home environment1. Another survey discovered that 1.7% of women presenting to a gynecologist’s office were found to have lichen sclerosus2. In addition, a vulvar clinic in England found lichen sclerosus to be the most common disease evaluated and treated, occurring in 39% of those patients3. There is a slight familial tendency, but the risk for the development of lichen sclerosus in family members is not known.


Itching is the most common presenting symptom of women with lichen sclerosus. Pruritus is frequently excruciating, and scratching often produces tearing and purpura because the affected skin is extremely fragile. The patient then experiences pain from the erosions, particularly with urination, sexual activity, or with attempted sexual activity. Pain with defecation due to fissured perianal lichen sclerosus, and resulting fecal retention and constipation are common, particularly in young girls.


Classically, well-developed lichen sclerosus presents as sharply demarcated white plaques encompassing the modified mucous membranes of the vulva, perineal body, and perianal skin (Figure 14.1). Most often, lichen sclerosus begins around the clitoral hood but, despite prominent involvement of the perineal body and perianal skin, it does not generally affect the keratinized, hair-bearing labia majora (Figures 14.2 and 14.3). Distant extragenital disease has been reported in a minority of women. A series of 250 women examined in this author’s office showed 6% with extragenital disease of keratinized skin and no oral or vaginal lesions (abstract still in press; presented at XIX World Congress of International Society for the study of Vulvovaginal Disease, July 2007, Alaska). The most common locations for extragenital disease are upper arms, back, and chest (Figure 14.4). Lichen sclerosus usually, but not always, spares the mucous membrane of the vestibule, and it has been reported only rarely in the mouth4. There is only one report of vaginal lichen sclerosus5. Lesions of the mouth or the vagina suggest an alternative or additional diagnosis.






Although many skin diseases present with white skin on moist mucous membranes or modified mucous membranes, the hallmark of lichen sclerosus is a characteristic texture change of the white plaques. Although the pathognomonic texture change is that of a crinkling or cellophane paper-type appearance, some lesions exhibit a smooth, waxy surface, and others manifest nonspecific irregular, hyperkeratotic white skin (Figures 14.514.7). Rubbing and scratching sometimes produce thickening of the skin (lichenification) and superimposed lichen simplex chronicus can obscure diagnostic texture changes of the lesions (Figure 14.8). Fragility is a hallmark of lichen sclerosus, manifested by purpura, erosions, and fissuring (Figures 14.914.12). Extragenital lesions show even more striking texture changes because of the dry nature of the skin. Sometimes, follicular plugging is visible in these lesions.










Long-standing and severe disease is associated with resorption of vulvar architecture, with loss of the labia minora, and the clitoris is buried under the scarred clitoral hood (Figures 14.13 and 14.14). Sometimes, side-to-side anterior and/or posterior adhesions eventuate in narrowing of the introitus (Figure 14.15). Squamous cell carcinoma occurs in up to 5% of women with untreated lichen sclerosus (Figure 14.16)6. The primary risk factors for the development of squamous cell carcinoma are elderly age of patients (probably an indication of longer duration of disease) and the presence of hyperkeratotic lesions (Figure 14.17)7.







Lichen sclerosus is sometimes associated with patchy hyperpigmentation of the modified mucous membranes and vestibule (Figure 14.18). This pigment change ranges from mild, poorly demarcated, tan patches to wild, irregular, variegate brown and black patches indistinguishable from malignant melanoma. Pigmentary changes are nearly always benign, but biopsy and follow-up are prudent, since case reports of atypical nevi and melanoma in the setting of lichen sclerosus suggest that there may be an association810. Also, benign pigmented lesions in the setting of vulvar lichen sclerosus sometimes appear atypical histologically, confounding the differentiation of benign from malignant11.



Women with lichen sclerosus have an increased prevalence of hypothyroidism12, and many clinicians find that their lichen sclerosus patients appear to be more likely to exhibit concomitant vitiligo or lichen planus, although data are scant13,14.



Diagnosis and differential diagnosis


Lichen sclerosus can be confused with other white diseases of the vulva as well as other diseases that exhibit tendency to scar. Lichen simplex chronicus and lichen planus are diseases that sometimes present with white vulvar plaques, and lichen planus produces resorption of vulvar landmarks indistinguishable from lichen sclerosus. The presence of prominent vestibular erosions and the common occurrence of accompanying oral and vaginal lesions suggest the diagnosis of lichen planus. Vitiligo is often mistaken for lichen sclerosus, but this disease presents with depigmentation only, with no symptoms, texture change, scale, erosions, or evidence of rubbing or scratching.


Genital warts and vulvar intraepithelial neoplasia 3 are sometimes white, but these are usually well-formed papules that are less symmetrical than lichen sclerosus. Cicatricial pemphigoid and pemphigus vulgaris are erosive diseases that produce resorption of vulvar architecture that is indistinguishable from lichen sclerosus, but white color and texture change are usually absent or subtle.


Although white plaques of the vulva are relatively common and nonspecific, and although scarring of the vulva that is identical to lichen sclerosus can occur with other diseases, the diagnosis of lichen sclerosus can often be made on the grounds of morphology. The characteristic crinkled or cellophane paper change, when present, is diagnostic, particularly in the setting of hypopigmentation and agglutination. When the diagnosis is not perfectly clear, a biopsy is indicated.


Apr 29, 2016 | Posted by in Dermatology | Comments Off on Lichen Sclerosus

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