Alan G. Waxman
History of present illness
A 60-year-old postmenopausal white woman, gravida 2, para 2 presents with an exacerbation of intense vulvar itching and burning. The symptoms have been present intermittently for the past 11 years, but until a few months ago, they presented only a mild annoyance. Recently, the itching has become constant and more intense. She has been able to tolerate the itching during the day. At night, however, the itching is so bad that she finds herself scratching in her sleep. She has found sexual relations increasingly difficult, and in the past two months has been unable to attempt to have intercourse. She denies recent changes in weight, vaginal discharge, and she does not douche or use feminine hygiene products. She wears cotton underwear and denies recent changes in soaps or deter- gents. Further review of symptoms is notable only for seasonal rhinitis, which is not currently bothering her.
The patient reports that she has seen her primary provider for these symptoms and has been to various urgent care clinics as well. She has been treated with both oral and vaginal medications for “yeast infections,” and has also been pre- scribed vaginal metronidazole. She has also tried a number of over-the-counter treatments without success. Her nurse practitioner recently started her on estrogen cream. Most of these medications have given her transient relief, but the symptoms ultimately return.
Her past medical history is notable for osteoporosis, and her only surgery is the repair of a torn left rotator cuff10 years prior. Her current medications are limited to estrogen vaginal cream, which she uses twice weekly, a weekly bisphosphonate, a daily multivitamin, calcium and a baby aspirin.
She reports having had two vaginal deliveries, thefirst with midline episiotomy. Menopause was at age 52, and she denies vaginal bleeding since. Her most recent cervical screening tests were negative for intraepithelial lesion or malignancy and negative for high-risk human papillomavirus (HPV) DNA.
She is a married, recently retired middle school social studies teacher. She does not smoke. She drinks alcohol twice a week with dinner.
Physical examination
General appearance: Well-developed, well-nourished woman who appears her stated age
Vital signs: All within normal limits. BMI: 24 kg/m2 Skin: No lesions noted on scalp, face, extremities, chest, trunk, or buccal mucosa
HEENT: Normal
Chest: Clear to auscultation
Cardiac: Regular rate and rhythm, no murmurs or gallops Abdomen: Soft, nontender, no masses
Lymph nodes: None palpable
External genitalia: Diminution of the labia minora
bilaterally with the left narrowed and the right largely absent.
The skin of the labia minora and inner labia majora is shiny and white with evidence of excoriation in the interlabial fold.
There is a 1 cm ulcer on the left. The ulcer is tender with slightly raised, distinct white margins and a nonfriable base (Fig. 14.1)
Introitus: The introitus is narrow but accepts a Pederson speculum with some discomfort
Acute Care and Emergency Gynecology, ed. David Chelmow, Christine R. Isaacs and Ashley Carroll. Published by Cambridge University Press.
© Cambridge University Press 2015.
Fig. 14.1 The ulcer is tender with slightly raised, distinct white margins and a nonfriable base.
Vagina: The vaginal mucosa appears pink and well rugated, consistent with topical estrogen use. The vaginal discharge is a creamy white and minimal in quantity. The cervix appears multiparous and without lesions
Uterus and adnexa: Because of the narrowness of the introitus, bimanual examination can only be performed with onefinger. A rectovaginal examination facilitates outlining the uterus, which is unremarkable. No adnexal masses are appreciated
Laboratory studies:
Vaginal discharge: pH 4.2
Wet mount: Epithelial maturation and lactobacilli consistent with vaginal estrogen use. No yeast, trichomonas, or clue cells seen
How would you manage this patient?
The gross appearance is consistent with a clinical diagnosis of lichen sclerosus. The ulcer, while not uncommon in this set- ting, clearly poses a cause for concern of a secondary diagnosis.
A biopsy is necessary to rule out malignancy. This was per- formed in the office setting with local anesthesia and a 4 mm Keys punch biopsy at the margin of the ulcer, including the ulcerfloor. Biopsy of the margin is important to include both epidermis and dermis, which will be absent in the ulcer, itself.
The patient was started on clobetazol propionate ointment 0.05% with instructions to use a thin application twice a day for one month, tapered to once a day for a month, then on alternate days for two weeks followed by twice a week. Her itching largely resolved within thefirst two weeks of treatment.
By six weeks the ulcer was completely healed. This patient’s biopsy report confirmed lichen sclerosus with no evidence of malignancy.
This patient’s topical steroid regimen was ultimately changed to use of a less potent steroid, triamcinolone ointment 0.1% used 2–3 times a week to reduce the incidence offlares.
She manages the occasional episode of recurrent itching with a short course of clobetazol.
The patient is scheduled for semi-annual vulvar examin- ations to look for the development of plaques, ulcers, or other lesions that might require repeat biopsy to rule out vulvar intraepithelial neoplasia or malignancy.
Lichen sclerosus
Lichen sclerosus occurs in both men and women. It is, how- ever, more common in women, and it is most commonly seen on the genitalia. It has a bimodal age distribution peaking in times of low estrogen, notably in postmenopausal women in the fifth and sixth decades, but also not infrequently in prepubertal girls [1]. It is an inflammatory disorder with a presumed autoimmune etiology. The exact pathophysiology remains unknown. The condition is associated with auto- immune thyroiditis, pernicious anemia, alopecia areata, and
vitilago. There is a familial predisposition with an increased incidence in siblings and twins, but the inheritance pattern is not fully understood [1].
The spectrum of skin changes seen in lichen sclerosus ranges from isolated to confluent white patches, and from thickened appearing skin to thinned crinkled parchment-like skin. It may involve the labia minora and majora alone or demonstrate a classical keyhole shape extending around the anus. Longstanding lichen sclerosus may create a thickened yellowish or dull gray coloration in the labia and interlabial folds. Patients may experience a spectrum of symptoms ranging from nothing, to tingling and slight burning of the skin, to intense itching and burning as with the patient illus- trated in this case. Chronic scratching and rubbing can create ecchymoses, excoriations, and ulcerations. Repeated scratching may thicken the epidermis resulting in adjacent lichen simplex chronicus, a condition of skin lichenification and swelling from chronic scratching. The“scratch–itch–scratch”cycle set up by lichen simplex chronicus can exacerbate and prolong the already intense itching of lichen sclerosis. Progression of the lichen sclerosis can result in loss of the architecture of the vulva with resorption or agglutination of the labia minora including the clitoral hood. In advanced cases, agglutination of the labia may lead to urinary retention requiring surgery.
Such progression, however, is not an inevitable development in women with lichen sclerosus, and the disease in some patients may be limited to small white patches on the labia that con- tinue without further progression. However, it is a chronic condition that will have recurrences and remissions through- out the patient’s life. The diagnosis is made clinically and confirmed with biopsy.
Histologically, the epidermis thins with or without hyperkeratosis or parakeratosis and there is flattening of the rete ridges. A pathognomonic feature is a band of dermal homogenization with an underlying inflammatory infiltrate that is predominantly lymphocytic (Fig. 14.2).
Lichen sclerosus responds very well to class 1 super potent steroids such as clobetazol propionate 0.05%. A common regimen used in the United States starts with twice daily applications as described in this case. There are no randomized clinical trials recommending one regimen over another. The British Association of Dermatologists guidelines recommend application once nightly for four weeks, tapering to alternate nights for four weeks and then twice weekly [2]. The steroid should be tapered to avoid a steroid rebound effect but may be restarted for recurrence of symptoms. Topical testosterone was widely used in the past, but has not been shown to have the efficacy of the super potent corticosteroids [2,3]. The potential for steroid toxicity may be reduced by limiting the steroid to a thin layer application and limiting the amount given to the patient. A 30 g tube should last 3 months.
While comfort measures are based on expert opinion, most clinicians advise empiric measures to protect the sensitive skin that is easily subject to contact dermatitis, allergic reactions, and irritation from excess moisture. Specifically patients
should be urged to avoid detergents or soaps with scents, and to consider using water alone to clean the vulva. A bland emollient may be used on the skin after bathing, and cotton underwear (or no underwear) should be encouraged, especially at night while sleeping. Many patients find warm soaks or use of cold gelpacks soothing and helpful to provide relief for the itching. In addition, patients may be started on hydroxyzine 50 mg at bedtime to relieve the itching and to help with sleep.
Squamous cell carcinoma is a rare cancer with an incidence of 1.5 cases per 100 000 women [1]. However, lichen sclerosus is present and adjacent to about 60% of all vulvar squamous cell cancers [1,4]. The squamous cell carcinoma associated with lichen sclerosis is HPV negative, and is associated with vulvar intraepithelial neoplasia, differentiated type. It has been estimated that the lifelong risk of a woman with lichen scler- osis to develop vulvar carcinoma is approximately 5% [1,4].
Long-term follow-up is therefore very important once the diagnosis of lichen sclerosis has been made. Most authorities recommend reevaluation of the patient every six months
looking specifically for new plaque-like lesions, white raised lesions, erythematous lesions, papillary tumors, ulcerations, or unexplained pain. Biopsies should be taken very liberally with any new clinicalfindings.
Key teaching points
Lichen sclerosus presents most commonly with chronic, severe vulvar pruritis.
While the diagnosis of lichen sclerosus is made clinically, biopsy confirmation should be obtained.
Super potent topical corticosteroids are the mainstay of treatment for lichen sclerosus.
Vulvar examination at six-month intervals is
recommended for women with lichen sclerosus because of the elevated risk of vulvar squamous cell carcinoma carcinoma.
Any new or different appearing lesion should be biopsied.
References
1. Murphy R. Lichen sclerosus.Dermatol Clin2010;28:707–15.
2. Neill SM, Lewis FM, Tatnall FM, et al.
British Association of Dermatologists’
guidelines for the management of
lichen sclerosus 2010.Brit J Derm 2010;163:672–82.
3. Ching-Chi C, Kirtschig G, Baldo M, et al. Systematic review and meta- analysis of randomized controlled trials on topical interventions for genital
lichen sclerosus.J Am Acad Dermatol 2012;67(2):305–12.
4. Gutierrez-Pascual M, Vincente-Martin FJ, López-Estebaranz JL. Lichen sclerosus and squamous cell carcinoma.
Acts Dermosifilogr2012;103(1):21–8.
Hyperkeratosis
Thinned epidermis with flaened rete ridges
Homogenous collagen band in dermis
Lymphocyc infiltrate
Fig. 14.2 Histology of lichen sclerosis.
Case 14: A 60-year-old woman with severe vulvar itching and an ulcer