Vaginal lichen planus

Dr. Lynne Margesson, a dermatologist, is certainly knowledgeable about oral lichen planus, but her expertise is treating women with vulva/vaginal lichen planus. Lichen planus may affect many lining mucosa areas. The vaginal/vulva location is especially serious for women who suffer from lichen planus, and Dr. Margesson believes that dentists and dental hygienists are the first line of defense in obtaining care for women.

Dental professionals often do not approach the subjectbecause of apprehension in asking questions about such personal locations. She knowsthat the detrimental effects of scar tissue in this area is preventable when treated early, but it is devastating for women when treatment is delayedor unrecognized. I interviewed her on the subject, and she has some excellent advice to share. 

Burkhart: Where is your practice, and what types of patients do you see in your dermatology practice?

Margesson: I am a dermatologist who is practicing in New Hampshire. I have practiced dermatology for 43 years. I have been doing vulvovaginal dermatology since the 1980s and set up the first combined vulvar clinic at Queens University in Kingston, Ontario, in Canada in the mid-1980s. I moved to United States in 1998. I have had a vulvovaginal clinic monthly at Dartmouth Medical Center with Dr. Deborah Birenbaum in the Department of Obstetrics and Gynecology since 1999. I retired from private practice just over a year ago.

Presently, I still attend the teaching clinic at Dartmouth Medical Center and a new clinic monthly at Bedford Women’s Care, a gynecological practice, in Manchester, N.H. Hopefully, I will join another clinic in Portland, Maine, in the next year. I have continued to teach and lecture. 

Burkhart: What are the primary issues related to women who have cutaneous lichen planus? Which surfaces appear to have the most visible problems? 

Margesson: Lichen planus typically onsets at 30 and 40 years of age. It is more common in women than men. Lichen planus affects 1% of the adult population. Women can present with the skin changes of lichen planus, which are small red bumps or spots on the skin. These are classically purple-red, raised and flat topped, and quite firm. Looking carefully at the lesions, one can see fine white lines in a lacy pattern across the surface such as Wickham’s striae.

These lesions can range in size from small pinpoint spots to more than a centimeter each. Typically, they are clustered in groups around the wrists, the mid-lower back, and the ankles. If the lesions are scratched—typically, they are quite itchy—they can spread and become conjoined together forming plaques. There are rare variations of the skin spots ranging from very thick lesions to scattered small lesions all over the body.

They can be treated and often will resolve in 18 months. When they resolve, there may be a grayish brown discoloration of the skin left behind that can take a year or two to fade.

Burkhart: What do you think causes lichen planus?

Margesson: We are still not sure. It is described as a chronic inflammatory rash. The immune cells of the body, T cells, are turned on to attack and destroy the lining cells of the body, which are the skin cells or the mucous membrane, lining cells. Why this reaction pattern happens we are not sure.

We do know that lichen planus can occur as a reaction to a drug. There is a long list of medications that can cause a lichen planus-type reaction on the skin and mucous membranes. Examples are common drugs like aspirin, ibuprofen, sulfa antibiotics, and even tetracycline. The lichen planus described here is the body’s response to a foreign antigen (the drug).

What other foreign antigens or proteins could do this and create the same reaction pattern on the skin and/or mucous membrane? Are there genetic factors playing a role? Probably. Lichen planus is seen with other disease conditions. In Europe, it is seen with hepatitis C infections.

I tell my patients that lichen planus is due to an overactive immune reaction where our own immune system is attacking our body skin and mucous membranes. The immune system refers to a collection of white cells in our body that protect us from infections and cancer by destroying foreign invaders. I am sure there is a foreign protein, drug, or chemical that starts this immune reaction, injuring the skin and/or mucous membrane. Lichen planus is more common in those that have conditions where the body attacks its own system referred to as autoimmune conditions such as vitiligo with white patches on the skin, alopecia areata where there is hair loss, etc. 

Burkhart: Do you usually see oral lesions as well as vulva/vaginal lesions? Can vulva/vaginal lichen planus be diagnosed clinically or are there other disease states that appear similar? I am thinking of lichen sclerosis. 

Margesson: Oral lichen planus is one of the most common presentations, and 80-90% of the cases involve the buccal mucosa with the spiderweb like network of white lines, also described as fern-like. These are painless.

Less commonly, there are painful, persistent erosions and ulcers with varying degree of diffuse redness involving not only the buccal mucosa but along the edges of the gums and even the tongue. Approximately, 25-30% of women who have oral involvement will have lesions in the vulva and/or vagina. Oral lesions of lichen planus proceed the ones in the genital area in one-third of patients. Both areas, however, can onset together. 

The diagnosis of lichen planus in the vulva and vagina can be made clinically when it presents with a typical lacy or fern-like pattern as is seen in the buccal mucosa of the mouth. A biopsy of this area will confirm the diagnosis easily. Genital lichen planus can affect anywhere such as the labia majora, labia minora, and the vagina. Often painful, persistent erosions and glazed red areas can be seen.

A biopsy of these areas is often nonspecific. There can be scarring with loss of the labia minora and shrinking of the opening of the vagina referred to as the introitus. In the vagina, there can be scarring so that the vagina is shorter and narrower. There may be adhesions as well. The surface may be eroded, be very raw, and bleed easily. Because of the erosions and open lesions, the whole area is very painful and burning. Women often will present because they cannot have sexual intercourse due to the pain on touching these areas.

Rarely, the vagina can be scarred closed. Additionally, 40% of vulvar cases can have oral involvement; 25% of oral lichen planus patients can have vulvar involvement. With vulvar and oral involvement, skin lesions are seen in about 20% of cases. 

The diagnosis of vulvar or vaginal lichen planus must always be considered with any scarring genital condition. The usual condition to mimic vulvar lichen planus is vulvar lichen sclerosus. Both conditions can present with a scarring pattern on the vulva with whiteness. Lichen sclerosus almost never involves the vagina. If there is typical oral lichen planus and scarring in the genital area, then lichen planus must be considered. Unfortunately, both lichen planus and lichen sclerosus can be seen in the same patient who may have oral lichen planus and vulvar lichen sclerosus or even both conditions on the genital area. A biopsy can be helpful to make a definitive diagnosis. Unfortunately, there is a lack of agreement on the diagnostic criteria for genital lichen planus, and biopsies are often read as nonspecific. Lichen sclerosus is typically scarring on the vulva with white areas that have a shiny surface whereas lichen planus is less distinct, not white but more pale and smudgy looking with the common tendency to have erosions and persistent red sore areas. Scarring may be dramatic in both conditions with loss of the labia as mentioned above and with scarring over the clitoral area so that clitoris is buried. 

Burkhart: What segment of this population complain about esophageal lichen planus? 

Margesson:Esophageal lichen planus is a rare presentation. It can present all by itself. The upper part of the esophagus is usually involved in 90% of cases. As of 2015, only 90 cases had been documented in the literature. Unfortunately, the diagnosis is often missed. It is more common in women and the onset is around 70 years of age.

One hopes that patients with lichen planus on the skin, in the mouth, or genital area are asked about problems with the esophagus as too often esophageal lichen planus is mistaken for acid reflux or a yeast infection of the esophagus. The patients complain usually of problems swallowing or pain on swallowing. Diagnosis is made on clinical presentation and biopsy. 

There are other rare forms of lichen planus. It can involve the eyes so that there are problems with inflammation along the eyelids and in the tear ducts. It can involve the larynx although that really is quite rare. The skin of the scalp, ears, nails, and even the hands and feet can be involved. 

Burkhart: Approximately how long does the average patient take to find someone knowledgeable in treatment both cutaneous, vulva or oral lesions? Just a rough estimate from dialogue in the past years. 

Margesson:This a difficult question. For cutaneous lichen planus most patients can find a dermatologist to make that diagnosis. In my area in New England, my vulvovaginal patients have seen three to five caregivers before the diagnosis is made. Vulvovaginal lichen planus unfortunately is too often missed as is so poorly recognized. Patients usually see their primary care physician and are sent to a gynecologist who may have had no training in vulvar disease. Most dermatologists have little to no training in genital dermatology.

There are, however, more programs on vulvar disease for caregivers than ever before. There are more centers in the United States than ever before—but still not enough. The International Society for the Study of Vulvovaginal Disease has lists of appropriate caregivers.

Burkhart: What treatment do you usually recommend? What points are important for a patient with vulva/vaginal lichen planus to know? 

Margesson:Treatment is aimed at stopping the immune system from destroying the skin/mucous membrane. Treatment is to control lichen planus. It is true we still do not have a cure. However, with aggressive treatment and a good response, patients can go into remission. This can be permanent, or they may relapse. I do not have any statistics on that. 

Treatment usually involves the use of strong steroid ointments, solutions, or suppositories. Systemic cortisone may be needed if there are severe flares. Treatment offers relief of pain, itching and discomfort, and it can prevent further scarring. 

I start with gentle genital care. For cleansing only, gentle hygiene is recommended using hands only and a mild hypoallergenic bar, unscented soap like Dove or Cetaphil Gentle Skin Cleanser. 

For the vulva, start with a topical corticosteroid ointment such as clobetasol or halobetasol 0.05% ointment once or twice a day. That is tapered down over time depending on the response. Only a very small amount is recommended.

I may have to add an anti-yeast treatment if they have a tendency towards yeast. If they are scratching, I may have to give them an antihistamine at night so they do not scratch. 

I add topical estrogen cream as indicated to the vulva and vagina to improve barrier function. 

For the vagina, there are almost no available commercial products. I will have compounded a corticosteroid vaginal cream at the appropriate strength that will be used nightly and then taper depending on the response. There are compounded corticosteroid suppositories also. Tacrolimus is an ointment that can be very useful on the vulva instead of cortisone, but, unfortunately, it has a tendency to burn and is expensive. It can be compounded into a solution or vaginal suppository to use. 

If the patient has a very severe disease, they will need systemic medications. Corticosteroids can be given as pills in the form of prednisone or as injections of triamcinolone. I tried to limit these to avoid side effects. I will combine other medications to suppress inflammation such as methotrexate and mycophenolate mofetil. If the vagina is narrowed, a dilator may be used. Surgery is needed to open a closed vagina. 

Burkhart: How often do you recommend that they come back for follow up? 

Margesson: Follow-up depends on the severity. I usually see my patients every three to four weeks initially and then hopefully every three to six months. When they are very controlled or seem to be in remission, I see them yearly. 

Dr. Margesson made this final comment: “I recommend that a dentist or hygienist who sees oral lichen planus should ask the lady to see her gynecologist for vulvovaginal checkup looking for lichen planus. I disagree with them avoiding this ‘because they don’t feel comfortable approaching this subject.’ This is so important, and they are the first-line people. Women can have lichen planus in the vagina and completely lose the vagina and not know. This can be prevented. Women suffer too needlessly with this condition. 

As always, continue to listen to your patients and always ask good questions.

NANCY W. BURKHART, AFAAOM, BSDH, EdD, is an adjunct associate professor in the Department of Periodontics-Stomatology, College of Dentistry, Texas A&M University, Dallas, Texas. Dr. Burkhart is founder and cohost of the International Oral Lichen Planus Support Group (https://www.dentistry.tamhsc.edu/olp/ and coauthor of General and Oral Pathology for the Dental Hygienist-In its 3rd ed. Nancy was awarded an Affiliate Fellow status in the American Academy of Oral Medicine in 2016. She was given the Dental Professional of the Year in 2017 through The International Pemphigus and Pemphigoid Foundation and is a 2017 Sunstar Award of Distinction Recipient. She can be contacted at [email protected]