Lichen Sclerosus - Causes, Symptoms, Diagnosis, Treatment, and Complications

What Is Lichen Sclerosus?

Lichen sclerosus, also known as BXO (balanitis xerotica obliterans) and white spot disease, is an ailment characterized by persistent lesions affecting mostly the genitals or the anus. The disease may lead to skin lesions, including white spots, irritation, dryness, pain, and itching.

In some patients, the skin may thin, making it more prone to damage. The cause of this illness is not well understood, although it might have something to do with immunity and hormonal imbalance. Lichen sclerosus is not contagious and does not pass from person to person.

It is more common among females compared to males; in fact, close to 85 percent to 90 percent of all recorded cases involve women.

Epidemiology and Disease Pattern

It occurs more often in women than in men, with a ratio between females and males of 3:1 to 10:1. The disease is more prevalent in postmenopausal women but has been known to affect children less than 10 years of age. Prevalence rates range from 0.1 percent in children to nearly 3 percent in women aged more than 80 years.

Patients often go undiagnosed for as long as five years due to the symptom onset. One characteristic of the disease is the figure-of-eight appearance, in which white patches appear on both the vulva and the perianal regions.

What Causes Lichen Sclerosus?

The actual cause of lichen sclerosus has not been identified yet.

1. It is often attributed to autoimmunity, in which the body mistakenly attacks its own cells and tissues in the skin.

2. The involvement of hormonal factors may also be possible; for example, low levels of estrogen following menopause may put a woman at risk of this disorder.

3. Those suffering from autoimmune diseases such as hypothyroidism, vitiligo, or type 1 diabetes are more susceptible to lichen sclerosus.

4. Having a personal history of lichen sclerosus or autoimmune diseases increases the risk of developing this condition.

5. Trauma or irritation on the skin caused by repetitive rubbing in the genital region could be among the triggers.

6. The presence of preexisting skin damage or scars at the affected site may predispose to lichen sclerosus. This condition is not classified as a sexually transmitted disease (STD).

What Are the Risk Factors Associated with Lichen Sclerosus?

• Postmenopausal women are at increased risk.

• Children under the age of 10 can suffer from this disease.

• Women who have hypothyroidism and other autoimmune conditions can be at increased risk for this disease.

• Umbilical foreskin circumcision increases the risk of developing genital lichen sclerosus.

• A family history of lichen sclerosus predisposes to the disease.

• Individuals with autoimmune diseases are more susceptible to this disease.

• This is due to a hormonal imbalance that occurs in some people.

• Prior skin trauma may cause the disease.

What Are the Symptoms of Lichen Sclerosus?

The main symptoms of lichen sclerosis are:

• White, raised areas on your vulva, anus, foreskin, or head of your penis.

• They make you feel itchy, sore, uncomfortable, or burning.

Other symptoms are:

• Sores or ulcers.

• Inflammation.

• Scar tissue.

• Cracking.

• Burning when you pee (dysuria).

• Discomfort during sex.

• Weak urinary stream.

• You might have urinary dribbling, where your pee doesn’t flow as a stream but sprays instead.

• Foreskin tightening.

• Being unable to retract your foreskin (phimosis).

• Penile discharge (fluid coming from your penis that is neither urine nor semen).

Occasionally, lichen sclerosis may affect other areas of the body, such as:

• Your neck.

• Your chest.

• Your torso.

• Your upper back.

• Your wrists.

• Your mouth.

When to See a Doctor?

You should consult your physician if you have itching, burning sensations, pain, and/or whitish patches on or around your genitals or anus. Consultation is also advised if there is thinning and cracking of skin, bleeding, and discomfort when urinating or engaging in sexual intercourse.

Children who have itching and irritation around their genitals must be seen, especially if the condition recurs. Persons with lichen sclerosus must go for checkups regularly since longstanding cases may result in scars and other problems.

How is Lichen Sclerosus Diagnosed?

Lichen sclerosus is often diagnosed based only on clinical appearance by a skilled practitioner. More insights can be obtained using dermoscopy.

Skin biopsies are often advised. Histopathology might support the lichen sclerosus diagnosis that has been suggested. Lichen sclerosus may be diagnosed with or coexist with another skin disorder.

A biopsy may be performed on a focused location to check for SIL or malignancy. During a follow-up, a biopsy could also be advised to assess problems or explain the treatment's insufficient effectiveness.

Differential Diagnosis

• Lichen planus - Wickham Striae and painful erosions.

• Vitiligo - Leukoderma without scars.

• Eczema - Red, itchy, and inflammatory.

• Psoriasis - Tubular plaques with silvery scales.

What Is the Treatment for Lichen Sclerosus?

Patients with lichen sclerosus should see a specialist for an accurate diagnosis and treatment recommendations. They should get familiar with the position and look of their lichen sclerosus. When using topical treatment, women should utilize a mirror. Photographs may aid in the monitoring of activities and therapy.

General Measures that Need to Be Followed Are:

• Once or twice a day, gently wash your hands.

• If necessary, use a non-soap cleaner.

• Avoid wearing tight garments, rubbing, and itching.

• Activities like riding a bike might increase symptoms.

• Seek medical help and treatment.

• Emollients can be used to reduce dryness and irritation, as well as to function as a barrier to protect delicate skin in the vaginal and anal regions from urine and feces.

Topical Treatment:

• In postmenopausal women, use intravaginal estrogen cream or pessaries. These alleviate the symptoms of atrophic vulvovaginitis.

• Instead of or in place of topical steroids, use tacrolimus ointment and pimecrolimus cream as calcineurin inhibitors. They usually produce searing pain (at least for the first several days). There are early concerns that these drugs may hasten cancer formation.

• Topical retinoid is not well tolerated on vaginal skin but may be used on other lichen sclerosus-affected areas. It helps to minimize scaling and dryness.

• Phototherapy with ultraviolet B (UVB) light may be used in cases where extragenital lichen sclerosus involves a large area of skin and topical therapies are ineffective. Relief is expected within two months.

Surgery:

• For high-grade squamous intraepithelial lesions or malignancies, surgery is required.

• Circumcision is helpful for males with lichen sclerosus of the prepuce and glans of the penis. If topical steroids alone have not reduced symptoms and signs, this procedure should be performed as soon as possible. Reconstructive surgery may be required if the urethra is stenosed or scarred.

• Suppose dilators have not been beneficial in reducing urinary issues and allowing intercourse in females. In that case, the removal of vulval and vaginal adhesions and scarring from vulval lichen sclerosus may be performed on occasion.

• Healing after circumcision is normally rapid, with most patients experiencing improvement after about a week to two weeks. When urethroplasty surgery for narrowing of the urethra is required, the healing process can take a few weeks, and the patient will temporarily be using a catheter for about three to four weeks.

Among the procedures are:

• Simple perineotomy (adhesion division).

• Fenton technique (transversely repaired incision).

• Perineoplasty (excision of affected tissue and advancement of vaginal mucosa).

• Unfortunately, lichen sclerosus can block the vaginal opening again after surgery appears to be effective.

Oral Antibiotics:

Systemic treatment is infrequently used when lichen sclerosus is severe, acute, and not responsive to topical therapy.

Among the options are:

• Corticosteroids, intralesional, or systemic.

• Oral retinoids include Acitretin, Isotretinoin, Methotrexate, and Cyclosporin.

Topical Corticosteroids:

The initial drug prescribed is clobetasol propionate, which relieves symptoms in many patients in three months. For children, Mometasone furoate is usually prescribed. If there is no response to steroid treatment, tacrolimus 0.1% or 0.03% cream, or pimecrolimus 1% cream, can be administered.

Lifestyle and Personal Care Advice for Lichen Sclerosus:

There are some lifestyle choices that can be adopted to minimize the irritation and outbreaks among those living with lichen sclerosus. One should consider wearing loose-fitting cotton underpants and avoid using strong soaps with perfumes or irritants.

Most physicians also recommend taking a warm bath rather than a hot bath, and avoiding showers with scented bubble baths. In addition, one should try to change into dry swimsuits immediately after swimming.

What Are the Complications of Lichen Sclerosus?

The complications of lichen sclerosus are:

• LS is a continuously relapsing condition that, if left untreated, can result in scarring and a total loss of conventional vulvar architecture in women, including introital stenosis, fusion, and resorption of the labia minora, and urethral strictures in men.

• Tissue adhesion and sclerosis cause tearing and, as a result, loss of sexual function, dysuria, constipation, itchiness, and discomfort.

• Furthermore, vulvar lichen sclerosis may progress to vulvar squamous cell carcinoma in the afflicted area, with a risk of up to five percent; however, its relationship with penile SCC is unknown.

• This probability of transformation is not connected with extragenital lesions. There have been reports of melanoma and basal cell cancer.

• Penile scarring causes painful erections and painful sex.

Conclusion

LS is a mucocutaneous autoimmune condition characterized by skin shrinkage and hypopigmentation. Women are more likely than men to have LS. In children, the prognosis for genital LS is favorable. Occasionally, the illness will resolve on its own.

LS often responds well to topical corticosteroids. Timely diagnosis and treatment could prevent the negative effects of the disease. About 10% of patients may have a familial history of lichen sclerosus.

This condition is linked with other autoimmune diseases such as hypothyroidism, vitiligo, and type 1 diabetes mellitus. To get personalized advice, consult a specialist for better care and follow-up.

Key Takeaways

• Lichen sclerosus is a chronic dermatological disease that mostly impacts the genital region and is prevalent in postmenopausal women.

• About one in every 70 women is likely to be affected by this disease, although it often goes unnoticed and may even take a long time before getting diagnosed.

• The usual symptoms associated with this disease include itchiness, whitening of the affected areas, pain, and skin thinning. With timely treatment, scar tissue formation can be avoided.

• Steroids constitute the most effective treatment option in most cases.