It’s unlikely you’ve heard of a skin condition called lichen sclerosus if you don’t have it or aren’t close to someone who does. That’s because it primarily affects the genitals which, of course, are usually covered by clothing.

Lichen sclerosus is a rare skin disease that causes itchy and painful patches of thin, white, wrinkled-looking skin. In women, these may occur on the vulva and/or the skin around the anus. In men, it typically affects the head of the penis. And in both men and women, it can result in painful sexual intercourse and difficulties when urinating, among other symptoms.

Lichen sclerosus is a chronic disease, meaning that once it develops, it lasts for a long time, often for the rest of a patient’s life. Though it often worsens over time, the good news is that treatments are now available that can reduce symptoms and stop progression of the disease.

“Lichen sclerosus may go undiagnosed for years because women are often too embarrassed to tell their doctor about their symptoms,” says Amanda Zubek, MD, a Yale Medicine dermatologist. “When caught early, prompt treatment can prevent a lifetime of debilitating itch and painful genital scarring.”

Lichen sclerosus is a chronic inflammatory skin disease that affects five to ten times as many women (most frequently postmenopausal) as men. However, it can develop in people of any age, including infants and children.

“It’s relatively rare—its exact prevalence is not known but estimated between 1 in 300 and 1 in 1000 individuals,” says Dr. Zubek.

The lesions caused by lichen sclerosus usually begin as small, pinkish or whitish bumps. Over time, they become patchy, wrinkly and white, often resembling tissue paper. These patches tend to be itchy and painful. Because the affected skin is delicate, even light itching may cause bleeding.

The condition can lead to scarring and tightening of the skin around the genitals and anus. Both men and women with symptomatic lichen sclerosus of the genitals are at slightly increased risk of developing skin cancer of the penis or vulva.

Though infrequent, lichen sclerosus can also occur on other parts of the body, including the thighs, buttocks, upper body, and wrists; rarely, some people may have lesions on the inside of the mouth. Around 15-20% of people who have genital lichen sclerosus also have lesions on other parts of the body. In 6% of cases, lichen sclerosus does not involve the genitals, but rather only develops on other parts of the body.

The cause of lichen sclerosus is unknown, though research has found links to genetic, environmental, hormonal, and immune-related factors.

For instance, some evidence suggests a genetic predisposition in some people, for whom the condition may be triggered by injury or trauma to the affected area.  

Other studies suggest that some cases of lichen sclerosus reflect an autoimmune disorder, meaning that the body’s immune system attacks healthy cells which ultimately leads to the development of skin lesions and other symptoms of the condition.

Yet other studies suggest that low levels of the hormone estrogen may be a cause of lichen sclerosus, though the relationship between estrogen and the disease is not fully understood.  

Doctors can often diagnose lichen sclerosus through a physical examination and a review of the patient’s medical history. In some cases, a doctor may perform a skin biopsy to aid in diagnosis. 

While there is no cure for lichen sclerosus, various treatments are available:

• Topical Steroids: Prescription topical steroid ointments that are applied to the affected skin can reduce inflammation and itching. These are usually the first treatment option for lichen sclerosus. The most commonly prescribed topical steroid is cobetasol propionate.

• Topical Calcineurin Inhibitors: By suppressing the response of the immune system, these creams and ointments can reduce skin inflammation.
• Topical and Oral Retinoids: A class of medications derived from vitamin A called retinoids are sometimes used to treat lichen sclerosus.
• Phototherapy: Exposure of affected areas of skin to certain wavelengths of ultraviolet light can reduce inflammation and itching.
• Oral Immunosupressants: This class of medications, which includes methotrexate and mycophenolate mofteil, suppress the immune system and reduce inflammation.

Surgery may be recommended as a treatment for some people. Treatment for uncircumcised men with lichen sclerosus, for instance, may include circumcision. Surgery may also benefit a woman whose vulva has become scarred.

Lichen sclerosus is a life-long condition for which there is no cure. If the disease progresses or is left untreated, it may cause serious, debilitating symptoms.

Fortunately, treatment generally reduces and, in some cases, eliminates symptoms, though relapses still tend to occur periodically. People with lichen sclerosus should regularly visit their physician to ensure that they are following an appropriate treatment regimen to monitor any signs of progression of the condition.

"A long-term maintenance treatment regimen is very important, and not just to prevent or treat symptom recurrence. Treatment to maintain normal skin color and texture reduces scarring and may prevent progression to skin cancer," says Yale Medicine dermatologist Alicia Little, MD, PhD.

At Yale Medicine, we take a proactive approach in treating lichen sclerosus. “We start with potent topical steroids and educate the patient on the importance of continued treatment. Even if they don’t have symptoms, preventing severe scarring or cancer is a priority,” Dr. Zubek says. “Yale Medicine Dermatology has experts in autoimmune disease and sclerosing disorders who are performing research, furthering our understanding of these diseases, and advancing treatment technologies.”