Lichen Sclerosus

Rare or Underrecognized?

February 28 was Rare Diseases Day, and this year I was shocked to learn that Lichen Sclerosus is classified as a rare disease.

You’d never know it from my waiting room.

I see a lot of LS in my colposcopy clinic. On questioning my gynecologist colleagues, they see many LS patients as well. So, is it really rare or just underdiagnosed?

I have a very low threshold for suspicion of LS. People who eventually receive an LS diagnosis come to me referred with painful intercourse, chronic yeast infection, and itching.

People with LS describe itching more inside than outside, occasional cuts on the labia minora or perineum, whitening of the inner vulva, and shrinking of the labia. They often have other autoimmune diagnoses such as hypothyroidism. If you notice these types of changes getting worse over months, ask your doctor to consider referral to a Colposcopy Clinic for evaluation.

Back in the old days, everyone got a vulvar biopsy. This practice has largely fallen out of favour, as many patients with obvious LS have non-specific biopsy results

In young people, symptoms of yeast infection and LS can be similar. Vaginal swabs that are negative during symptoms of yeast infection should prompt a search for an alternate explanation. Ask your family members if they have similar issues! I often have siblings and their parents in my practice.

In postmenopausal people, vaginal yeast infections are quite rare. The vagina changes with low estrogen and people stop getting yeast overgrowth. The exceptions to this are Diabetes (high sugar levels in blood cause increased sugar in the vagina) and people taking vaginal estrogen. Chronic itch in a healthy postmenopausal person should be investigated.

Since biopsy is often unhelpful, I tend to offer a trial of therapy. If I’m right about the diagnosis and the patient has LS , they can use potent steroids for months and just keep feeling better and better. A patient who doesn’t have LS will feel great for the first couple of weeks, but by the end of the three weeks they start to feel raw and sore from steroid overuse.

Most people with LS should be on a maintenance dose of steroid every Monday/Wednesday/Friday forever. I tell patients they should use their cream until they die or I die because if I’m gone there will be no one to nag them about it! Very rarely I’ll have a patient who needs to decrease to once or twice per week. The goal is to prevent recurrent symptoms.

The lifetime risk of vulvar cancer in untreated LS patients is about 3%, so we recommend a yearly vulvar exam to screen for pre-cancerous changes. In patients who use their steroids three times weekly, cancer incidence is much lower than that. In fact, in 15 years of practice, I’ve never even had a precancerous lesion show up in patients who take their medication conscientiously. In contrast, I’ve seen many cancers in elderly patients who were told they had chronic yeast infections throughout their postmenopausal years, and likely had undiagnosed LS.

If you’ve already been diagnosed with Lichen Sclerosus, make sure you stay on your medication and have yearly checkups!