What is Lichen Sclerosus?
Lichen sclerosus (LS) is a long-term skin condition that causes inflammation. It was first identified by a scientist named Hallopeau in 1881. Over the years, it has been known by different names like leukoplakia, kraurosis vulvae, balanitis xerotica obliterans, and lichen sclerosis et atrophicus. In 1976, a group focused on studying diseases related to the vaginal area decided to use the term lichen sclerosus.
LS is a type of autoimmune disorder, which means the body mistakenly attacks its own cells. In the case of LS, it targets the skin and mucous membranes – the moist, inner lining of some organs and body cavities. The condition is known for causing the skin to lose its pigment (hypopigmentation) and thinning of the skin (atrophy). It usually affects the genital area but can also appear in other areas of the body. Women are more likely to have LS than men.
LS can cause tightening of the foreskin in males (phimosis) or scarring at the vaginal opening in females. Diagnosis is usually based on the visible signs but often a biopsy (taking a small piece of skin for testing) is done to confirm it. If not treated, LS can lead to damage of body structures, troubles with bodily functions, and may even increase the risk of cancer. Therefore, getting treatment and regular check-ups over the long term are very important.
What Causes Lichen Sclerosus?
Lichen Sclerosis (or LS) is believed to be a condition caused by the body’s immune system attacking its own cells, but the exact cause is still a mystery. It is often seen with other diseases where the body’s immune system is overactive, like alopecia areata (hair loss), vitiligo (loss of skin color), autoimmune thyroiditis (when the immune system targets the thyroid), and pernicious anemia (a type of vitamin B12 deficiency). The causes are probably multiple, and could include different things for different people.
Some people might be more likely to get LS because of their genes, while for others, it could be due to infections caused by spirochetes, a type of bacteria. Other possible causes might be a response to sex hormones or due to the Koebner phenomenon, which is when skin diseases show up at the site of a skin injury. Additionally, recent studies have shown that LS and a skin disorder named morphea may occur together more often than previously thought.
Risk Factors and Frequency for Lichen Sclerosus
Lichen sclerosis (LS), a relatively rare condition, affects about 1 in 1000 to 1 in 300 people. Its exact prevalence is still unclear because many people with LS don’t show any symptoms, and it often gets misdiagnosed. LS can affect anyone, regardless of gender, race, or age. But, it is more common among women, with the women to men ratio varying from 1:1 to 10:1.
- LS is more prevalent among women, with two noticeable surges: one among girls aged 8 to 13 and the other during the 50s and 60s age range.
- The average age for diagnosing LS tends to be between 52 and 60 years.
- About 85% to 98% of LS cases affect the genital skin, and 15% to 20% cases occur in the skin areas outside the genitals, also known as extragenital LS.
- Extragenital LS during childhood is quite rare.
- LS can also occur in the mouth, a variant known as oral lichen sclerosus, but this type is extremely rare.
Signs and Symptoms of Lichen Sclerosus
Lichen sclerosus is a skin condition that typically affects the genital area but can sometimes also occur in other parts of the body. It begins with a clearly defined redness that gradually turns into thin, colorless, shiny, and hard patches of skin. These patches may become thicker due to constant scratching. Itching, often more intense at night, is the main symptom.
Other manifestations may include visible veins, target-shaped or rounded spots, cracks, open sores, and swelling. Those affected may experience itching, a local burning sensation, and pain. They may have trouble passing stool, especially in children, though this symptom is less common in adults. Sometimes the condition may not cause any symptoms at all.
- Clear redness that turns thin and colorless
- Visible veins
- Target-shaped or round spots
- Itching, particularly at night
- Local burning sensation
- Pain
- Open sores
- Swelling
In females, the condition typically begins around the hood of the clitoris and can spread to large areas that include the vulva, the skin between the vagina and anus, and the area around the anus. This condition usually doesn’t affect the vagina and cervix. In males, it occurs on the foreskin, the head of the penis, and the ridge around the head of the penis. When not in the genital area, the disease can occur on any nearby skin but is usually symptomless. Common areas of occurrence include the area under the breasts, neck, wrists, thighs, upper back, and shoulders. Lichen sclerosus can also appear as blue-white spots on the cheek lining or beneath the tongue.
Testing for Lichen Sclerosus
To diagnose a condition like lichen sclerosus (a skin disease that can cause discomfort or itching), doctors will need to know your medical history – this includes any autoimmune diseases you or your family may have had. Autoimmune diseases are conditions where your body’s immune system mistakenly attacks your own cells. Then, your doctor will examine your mucous membranes (the soft, moist areas inside your body like the inside of your nose or mouth), skin areas elsewhere on your body, and conduct a gynecological exam, if applicable.
Usually, these steps are enough to diagnose lichen sclerosus. However, in some cases, your doctor might need to take a small sample of your skin (a biopsy) for further examination. Even if the results of this skin test come back as atypical, or not normal, it doesn’t necessarily mean you don’t have lichen sclerosus.
Your doctor might perform a biopsy if:
1. Your symptoms or condition don’t match the usual signs of lichen sclerosus.
2. They suspect you might have skin cancer.
3. The first-line treatment (the first medicine or therapy given for your condition) isn’t working after a suitable length of time.
Along with these, your doctor will also likely check how your thyroid is functioning, as problems with this gland can be connected to lichen sclerosus. Depending on your symptoms, they might also check for other autoimmune diseases.
Treatment Options for Lichen Sclerosus
The goal of treatment for this condition is to decrease the symptoms, stop thinning of the body tissue, prevent the formation of scars and changes in body structure, as well as avoiding the disease’s conversion into a more severe form like cancer. The treatment might involve general care, creams applied locally, medications taken by mouth, or possibly surgery. It’s really important that the patient doesn’t use anything that could irritate the skin, such as soap, and instead they should use skin moisturizers to stop itchiness and prevent further irritation.
When this disease affects the genital area, the main treatment involves applying a strong kind of steroid cream like clobetasol propionate for three months. If this doesn’t work, other creams like calcineurin inhibitors or imiquimod can be used. In men, a procedure called circumcision might be recommended early. Surgery is only recommended, if there are complications from lichen sclerosus, which is the technical term for this disease. When this disease affects areas other than genitals, the treatment options are fewer and may include light therapy, strong kinds of steroid creams, a type of ointment named tacrolimus, and oral medications such as steroids or methotrexate. Patients with this disease need to have regular doctor appointments indefinitely to monitor the condition. [6]
What else can Lichen Sclerosus be?
There are a variety of skin conditions that might share similar symptoms. Here are some of them:
- Lichen planus
- Lichen simplex chronicus
- Vitiligo
- Morphea
- Eczema
- Contact dermatitis
- Psoriasis
- Child sexual abuse
What to expect with Lichen Sclerosus
Generally, the outlook for children with genital Lichen Sclerosus (LS), a skin condition, is good. Many times, the disease can improve or even go away on its own. LS usually responds well to local treatments with corticosteroids, a type of anti-inflammatory medication.
However, the outlook isn’t as positive for LS that affects areas outside of the genital and for chronic atrophic genital disease, a long-standing thinning condition of the skin in the genital region.
If LS is not treated, it can lead to complications. That is why catching and treating LS early is important in preventing these complications.
While LS is not considered a condition that can potentially lead to cancer, it’s important to note that patients with genital LS have a slightly higher chance of developing squamous cell carcinoma, a type of skin cancer.
Possible Complications When Diagnosed with Lichen Sclerosus
Lichen Sclerosis (LS) is a health condition that keeps coming back if not treated, and can eventually cause scarring leading to severe alterations to the women’s vulvar region. In some extreme cases, it may even result in the total loss of the standard vulvar structure. In men, it can lead to a tightening of the urethra that makes it hard to urinate. Scarring and skin hardening can cause tissue tearing and a loss of sexual function. Some people might experience difficulty urinating, constipation, itching, and discomfort.
Additionally, there is a risk that LS of the vulva can progress to a type of skin cancer known as squamous cell carcinoma in the affected area. The risk is estimated at up to 5%, though it’s not clear if there’s a connection between LS and this type of cancer in men. LS away from the genitals isn’t linked with this cancer risk. In certain instances, cases of melanoma and basal cell carcinoma, two other types of skin cancer, have been reported among those with LS.
Common Side Effects:
- Scarring leading to alterations to the vulvar region or urethra tightening
- Tissue tearing and a loss of sexual function
- Difficulty urinating
- Constipation
- Itching
- Discomfort
- Progression to squamous cell carcinoma
- Unknown connection with squamous cell carcinoma in men
- Cases of melanoma and basal cell carcinoma in certain instances
Preventing Lichen Sclerosus
LS stands for “lichen sclerosus”, a skin condition that can cause scarring and changes to the normal structure of the genitals. Because of these changes, patients may experience sexual dysfunction. In these instances, patients might need further education or supportive care to understand and manage these changes.
Lichen sclerosus in the anogenital region (the area around the anus and genitals) can potentially turn into a type of skin cancer known as squamous cell carcinoma. Because of this risk, it’s vital for patients with this condition to have regular check-ups. This allows doctors to keep an eye on their condition and ensure any changes are caught early.