Lichen Sclerosis of Vulva Case File
Eugene C. Toy, MD, Patti Jayne Ross, MD, Benton Baker III, MD, John C. Jennings, MD
CASE 60
A 51-year-old parous woman complains of a 4-year history of vaginal and vulvar itching. She scratches the area nearly every day and reports that the itching is worse at nighttime. She has diabetes, well controlled, is postmenopausal of 3 years, denies any sexually transmitted diseases or abnormal Pap smear history, and has four children delivered vaginally. Her blood pressure is 130/70 mm Hg and heart rate is 80 bpm. On inspection and examination of the external female genitalia, the following is revealed: atrophic-appearing external female genitalia, tissue over the labia minora is white and thin, the clitoris is hard to appreciate, excoriations are noted on bilateral labia majora, and some small bruising noted at the vaginal introitus. She is very tender on examination and speculum insertion is difficult, because the introitus is constricted. The cervix is visualized and no discharge is noted. Bimanual examination reveals a small uterus and no adnexal masses are appreciated.
» What is the most likely diagnosis?
» What is the next step in making the diagnosis?
» What is the most likely therapy?
ANSWER TO CASE 60:
Lichen Sclerosis of Vulva
Summary: A 51-year-old woman is noted to have vaginal and vulvar itching for 4 years. Inspection of the external female genitalia reveals atrophic, white, thin excoriated tissue and retraction of the clitoris and constriction of the vaginal introitus with some bruising.
- Most likely diagnosis: Lichen sclerosis (LS).
- Next step: Biopsy of the affected areas.
- Most likely therapy: Corticosteroid ointment each evening.
ANALYSIS
Objectives
- Describe the characteristics of patients that present with lichen sclerosis, and the natural history of the disease.
- Recognize the anatomical boundaries of the vulva and aspects of good vulvar hygiene.
- Identify current treatment regimes for lichen sclerosis and the follow-up that is requisite.
- Describe some of the other common vulvar diseases and their treatment.
Considerations
This postmenopausal woman is suffering from lichen sclerosis given her history and physical finding. The diagnosis is confirmed with biopsy of the affected vulvar tissue, revealing a thinned epidermis, hyperkeratosis, and elongation of the rete pegs. Lichen planus can also present similarly, but usually involves the vagina which LS does not. An experienced dermatopathologist should be able to differentiate the two on biopsy specimen. Long-standing candidal infection of the vulva may lead to similar symptoms. Since our patient is postmenopausal, therefore lacking estrogen, the pH of the area is raised and not amenable to candidal infection unless she has poorly controlled diabetes or is immunosuppressed. Sometimes vaginal atrophy in the postmenopausal patient can lead to pruritus, but usually not to this extent. Psoriasis may present with pruritus but not usually, and the lesions are classically described as silver scales, and are also present on the extensor surfaces of the extremities. Cancer of the vulva or vulvar intraepithelial neoplasia commonly presents with pruritus and is often associated with LS, which is why biopsy of the affected area and frequent surveillance of the vulva is warranted.
APPROACH TO:
Vulvar Disorders
DEFINITIONS
LICHEN SCLEROSIS: Chronic, inflammatory dermatologic disease characterized by pruritus and pain, which mainly affects the anogenital region.
VULVA: The external genitalia of the female comprised of the mons pubis, the labia majora and minora, the clitoris, the vestibule of the vagina and its glands, and the opening of the urethra and of the vagina.
CLINICAL APPROACH
The anatomic boundaries of the vulva extend from the mons pubis superiorly to the anus inferiorly and the genitocrural folds laterally. It is made up of the labia majora and minora, mons pubis, clitoris, vestibule of the vagina, urethral meatus, Skene glands, vaginal orifice, hymen, and Bartholin glands.
Lichen Sclerosis
Lichen sclerosis is a chronic progressive inflammatory medical condition of which there is no definitive cure. LS is more common in women than men, and the onset can occur at any age, peaking in the prepubertal and postmenopausal period. LS usually presents in the anogenital region, with extragenital disease only15% to 20% of the time. Women with the disease usually present with the complaint of itching which can be worse at night, and is described by the patient as vaginal itching. Appreciate that the itching is localized to the tissue of the vulva. Differential diagnosis of LS is lichen planus, psoriasis, vulvar intraepithelial neoplasia, and vitiligo. On examination of the external genitalia, a figure-eight pattern is seen around the vulva and anus. The skin is classically described as “cigarette paper” as it appears crinkled and is fragile, thinned, and atrophic. Abrasions may develop from scratching or attempted intercourse, and ultimately scarring may cause narrowing or a complete closure of the vaginal introitus, even in the parous woman. The labia minora may fuse burying the clitoris behind the fused clitoral hood. The scratching of the areas worsens the disease and can also lead to dyschezia, from constriction of the anus.
Counseling of the patient is important including discussing components of vulvar hygiene, avoiding irritants to the skin such as soaps and bubble baths, cessation of scratching the lesions, and wearing all cotton, white underwear. The patient should be made aware of the chronicity of the disease and the need for yearly surveillance. Treatment of the disease is aimed at preventing relapses of intense pruritus and the mainstay is corticosteroids. Initially, a potent steroid ointment, Clobetasol, may be necessary to provide relief, and should be used daily until symptoms abate and then tapered to intermittent use.
Bartholin Gland Abscess
The Bartholin or greater vestibular glands are located at the 5- and 7-o’clock locations of the labia majora. Usually, they are too small to palpate but with inflammation, they can be enlarged and painful. The treatment options include incision and placement of a small balloon catheter into the gland or marsupialization which is surgical fixation of the cyst wall everted against the mucosa of the vulva. The purpose of both of these techniques is to allow drainage of the infection for several weeks. A simple incision and drainage is prone to recurrence. Bartholin gland infections are usually polymicrobial and not usually sexually transmitted. Involvement in women over the age of 40 years can be associated with cancer and should have a biopsy.
Vulvar Cancer
Because vulvar cancer can present with no symptoms or with itching, any suspicious lesion of the vulva especially in a postmenopausal woman should undergo biopsy. Unfortunately, delay in diagnosis is usually the rule due to lack of clinical suspicion and prescription of various topical agents. Younger women such as those in their 30s may develop vulvar cancer due to human papillomavirus; smoking is also a risk factor. Again, biopsy is the rule. Regardless of the age, if vulvar cancer is diagnosed, then the patient should have surgical staging, with the primary lesion removed and the adjacent (ipsilateral) inguinal lymph nodes. Most vulvar cancers are squamous cell, but melanoma, basal cell carcinoma, and other subtypes can occur. Thus, pigmented lesions of the vulva should be carefully considered for biopsy.
|
CASE CORRELATION
|
COMPREHENSION QUESTIONS
Match the vulvar lesion (A-E) to the clinical presentation (60.1-60.5).
A. Lichen sclerosisB. PsoriasisC. Vulvar cancerD. Vulvar candidiasisE. Postmenopausal vulvar atrophy
60.1 A 60-year-old postmenopausal woman is recently remarried and has pain with intercourse.
60.2 A 52-year-old postmenopausal woman complains of intense itching around her vagina and anus which makes intercourse and defecating painful.
60.3 A 45-year-old woman with poorly controlled diabetes reports that she has tears on her vagina causing pain with intercourse and defecation.
60.4 A 59-year-old postmenopausal woman presents with a 10-year history of vaginal itching, which she scratches frequently, and a bump near her clitoris.
60.5 A 54-year-old postmenopausal woman complains of itching in her vagina and, the physician notices scaly lesions on both of her elbows.
60.6 A 34-year-old G2P2 woman is noted to have a painful mass of the vulvar area at the 5:00 location. An incision and drainage procedure is performed. If culture is done, which of the following organisms is most likely to be found?
A. Treponema pallidumB. Neisseria gonorrheaC. Chlamydia trachomatisD. Haemophilus ducreyiE. Peptostreptococci
60.7 A 56-year-old woman is seen for a 2-cm ulcerating lesion of the right labia majora that has been present for 5 months. You perform a punch biopsy of the lesion which reveals moderately differentiated squamous cell carcinoma. Which of the following is the most likely location of the metastasis?
A. Left labia majoraB. Uterosacral ligamentC. Inguinal lymph nodesD. Pelvic lymph nodesE. Hypogastric arterial plexus
ANSWERS
60.1 E. Complaints of dyspareunia, or painful intercourse, are not uncommon in the postmenopausal state. In the first 5 years after menopause, atropic changes are not as common. This patient is 60 years old, and likely 10+ years postmenopausal. Without estrogen, the vaginal and vulvar tissue can atrophy leading to bruising, tearing, and even bleeding of the vulva vagina with intercourse. Topical estrogen can alleviate these symptoms. The vaginal and vulvar area should be inspected for lesions.
60.2 A. Pruritus of the vulva is not unique to lichen sclerosis, although the predilection for the vulva and anus is. Examination of the vulva and anus with indicated biopsies and topical steroid ointment is the treatment of choice.
60.3 D. Diabetes can lead to candidal infection of the vulva which can cause fissures in the labial folds, and the scratching of the disease can sometimes spread the infection. Women who present with vulvar candidiasis should be evaluated for diabetes.
60.4 C. Lichen sclerosis (LS) left untreated and with repeated scratching can predispose to carcinoma of the vulva. Nevertheless, this is fairly rare, since only about 5% of women with LS will ultimately develop vulvar cancer.
60.5 B. Psoriasis can affect the genital area, and the silver plaques on the elbow are a dead giveaway to the disease. Treatment of this disease may prove difficult, and consultation with an experienced dermatologist is requisite.
60.6 E. The most common bacteria found in a Bartholin gland abscess are polymicrobial such as skin organisms, Gram-negative rods, and anaerobes. It is rare to have STI-related organisms in these abscesses.
60.7 C. The most common location for spread of a squamous cell carcinoma of the labia majora is the ipsilateral inguinal lymph nodes. After spread to these nodes, the cancer may progress to the pelvic lymph nodes. A midline lesion may travel to bilateral inguinal nodes, but a lateral lesion will almost always be isolated to the ipsilateral nodes.
CLINICAL PEARLS
|
» Itching of the vulva, especially in a postmenopausal woman, should prompt a thorough history and examination with indicated biopsies of the affected areas. » Lichen sclerosis is a chronic condition characterized by thin, cigarette paper-like, crinkly epithelium. Frequent surveillance of the vulva is necessary as to prevent squamous cell carcinoma of the vulva. » Vulva cancer is staged surgically including dissecting the ipsilateral inguinal lymph nodes. » Bartholin gland cysts are treated by Word catheter or marsupialization so that drainage for several weeks can occur. Simple incision and drainage is associated with a high rate of recurrence. |
REFERENCES
American College of Obstetricians and Gynecologists. Diagnosis and management of vulvar skin disorders. ACOG Practice Bulletin 93. Washington, DC; 2008. (Reaffirmed 2013.)
Brown D. Non-neoplastic epithelial disorders of the skin and mucosa (vulvar dystrophies). In: Kaufman R, Faro S, Brown D, eds. Benign Diseases of the Vulva and Vagina. 5th ed. Philadelphia, PA: Elsevier; 2005:274-290.
FIGO Committee on Gynecologic Oncology. Revised FIGO staging for carcinoma of the vulva, cervix, and endometrium. Int J Gynecol Obst. 2012;119S2:S90-96.
Frumovitz M, Bodurka DC. Neoplastic diseases of the vulva. In: Katz VL, Lentz GM, Lobo RA, Gershenson DM, eds. Comprehensive Gynecology. 6th ed. St. Louis, MO: Mosby-Year Book; 2012:781-800.
0 comments:
Post a Comment
Note: Only a member of this blog may post a comment.