What Is Lichen Sclerosus?

Lichen sclerosus is a disorder that mostly affects the genital and anal regions, resulting in skin that is thin, white, and wrinkled. It is brought on by skin changes and inflammation in the affected area. Typical signs and symptoms involve itching, irritability, and pain. There is no cure currently but symptoms can be managed using various forms of treatment.

Overview

Lichen sclerosus (LS) is an inflammatory skin condition affecting mostly the anal and genital areas. Extragenital involvement can also be present in a few cases. Lichen sclerosus can occur in all age groups and can affect both sexes. However, it predominantly affects postmenopausal people assigned female at birth (AFAB) and, to a lesser extent, people assigned male at birth (AMAB), prepubertal children, and adolescents. The exact cause of LS is still unknown. The typical clinical picture includes chronic white patches on the skin with itching and soreness. LS can affect anywhere on the body but is mostly seen in the vulvar, perianal and penile regions.¹ There is an underestimation of the true number of cases of LS. One-third of cases are asymptomatic, and LS is usually also misdiagnosed or goes unrecognised. The estimated prevalence of LS in patients referred to dermatologists ranges from 1 in 300 to 1000 patients and 1 in 30 older adult women are seen in general gynaecology clinics.² Although there is no cure currently, treatment may reduce the symptoms. In addition to an early diagnosis and the appropriate course of therapy, a long-term follow-up is essential for preventing complications. 

Causes of lichen sclerosus

The exact cause of lichen sclerosus is unknown. However, scientists believe that it involves the activation of many T-cells, enabling the release molecules that further activate cells found in connective tissue - known as fibroblasts.² 

Genetics can play a large role in the likelihood of having a disease - especially in immune-mediated diseases. A cell surface receptor protein known as HLA-DQ7 has been found more prevalent in both people AFAB and AMAB patients with lichen sclerosus.¹ 

Signs and symptoms of lichen sclerosus

Lichen sclerosus causes the formation of patches on the skin that usually appear as white and are itchy.¹. The patches can be smooth or wrinkled and are easily damaged and bleed when scratched.² These patches can appear anywhere but are mostly found in the genital and anal regions. In people AFAB, it affects the area around the opening of the vagina and in people AMAB, it can affect the foreskin and the end of the penis.^1,3 In people AMAB, there may also be difficulty in erections due to scarring and narrowing of the foreskin’s top aperture.¹ Lichen sclerosus causes irritation and itching on the foreskin of the penis in uncircumcised people AMAB.⁴ Other regions of the body can occasionally be affected by lichen sclerosus. Though less common, there have been several cases of oral lichen sclerosus which affects the mouth.⁵  Some individuals may not experience symptoms at all, with at least one third of patients being asymptomatic.¹ Other possible symptoms may include:^1,2

• Pruritus (extreme itching)
• Pain 
• Skin bruising and tearing, which can lead to bleeding.
• Blisters
• Skin becomes fragile and can bleed easily from minor rubbing. 
• Pain or bleeding during bowel movement.
• Discomfort or pain when urinating or defecating

Risk factors

There are several risk factors that increase your likelihood of developing lichen sclerosus. These risk factors can be divided into 4 categories:

• Hormonal
• Previous trauma (such as a history of sexual abuse)
• Other autoimmune diseases 
• A family history of lichen sclerosus

Postmenopausal people AFAB and prepubertal children are at a higher risk due to the decreased oestrogen in their bodies. Trauma and irritation both contribute significantly to the onset of LS. Occlusion, scratching, friction, and surgical treatments, for instance, might cause LS lesions to manifest. A history of sexual abuse can also increase the risk of LS. In older people AFAB, urinary incontinence, multiparous status, infrequent genital washing, and high BMI are linked to vulvar lichen sclerosus. Uncircumcised people AMAB are more likely to develop LS than their circumcised counterparts. There may be a greater risk for lichen sclerosus in people AFAB who have another autoimmune disease, such as autoimmune thyroid disease (like hypothyroidism), autoimmune-related anaemia, vitiligo, type-1 diabetes, and alopecia areata.¹ Family history of the condition can also influence the likelihood of developing the disease.

Diagnosis

In most cases, a diagnosis is made based on a physical examination of the affected skin present with signs and symptoms of LS.¹ To ensure that there are no other medical concerns, additional tests can be completed. Lichen planus, low oestrogen, vitiligo, or autoimmune thyroid issues are a few conditions that need to be ruled out. Therefore, additional tests are completed to rule these out. During early stages of the disease, it is difficult to diagnose. A biopsy can also be done to rule out malignancy. A biopsy is usually done if the skin does not respond to the topical steroid treatment.⁵ 

Complex cases may be referred to specialists such as dermatologists and gynaecologists.

Management and treatment for lichen sclerosus

Although, currently there is no cure for lichen sclerosus.There are several forms of management and treatment of the disease. One way is through the use of  steroid creams that help to reduce the symptoms like:³

• Reducing atrophy, 
• Preventing scarring, 
• Preventing anatomical distortion,
• Preventing malignant change. 

General care, topical therapies, systemic treatments, and surgical procedures are all included in the therapy. It is advised to stay away from irritants like soap and to choose instead for emollients to end the cycle of itch.⁵

The gold standard treatment for genital Lichen sclerosus is the administration of strong topical steroids, such as clobetasol propionate, for three months. It has shown an improvement in symptoms and has a low risk of developing adverse side effects.¹  Other treatment options involve UVA1 phototherapy or photodynamic therapy. Though, these should only be considered upon failure of the other options available. For adult LS, there is no surgical first-line therapy, and recommendations are based on medical expertise. In people AMAB, surgery for circumcision is done whereas for people AFAB, a vulvectomy is common. However, both of these procedures have high recurrence rates. Regenerative therapies such as platelet rich plasma can be done to restore the initial skin barrier, if no other first-line treatment is working.¹ Follow-up examinations are essential indefinitely to observe for recurrence and look for complications. 

Complications

Lichen sclerosus is a chronic, recurrent condition that, if left untreated, might lead to:¹  

• Scarring: It may affect the anatomical structure of the genitals by causing a fusion of labia and a narrowing of the vaginal entrance. Other clinical manifestations may occur as a result of scarring such as constipation and urine retention, if scarring is long term.
• Malignancy: the chances of developing cancer such as squamous cell carcinoma, are a lot higher with the presence of lichen sclerosus. It is unclear what causes LS to become malignant. Therefore, it is crucial to identify skin lesions early and to maintain a lifelong follow-up.
• Sensory abnormality: vulvodynia may develop as a result of lichen sclerosus. Typically, the symptoms last for a long time after the condition has disappeared.
• Further infections may occur as a result through complications of prior treatments

FAQ’s

How can I prevent lichen sclerosus?

Lichen sclerosus is difficult to prevent. By altering your lifestyle, you might be able to relieve the symptoms associated with the disease. Symptoms can be relieved by reducing friction and irritation to the affected area by:

• Avoiding long bike rides.
• Wearing loose-fitting underwear and clothing.
• Changing out of wet clothes immediately
• Using unscented soaps and laundry detergent.

How common is lichen sclerosus?

Lichen sclerosus is rare, and the exact incidence is unknown. There may be many misdiagnosed cases or asymptomatic cases may not be reported at all. The estimated prevalence of LS in patients referred to dermatologists ranges from 1 in 300 to 1000 patients and 1 in 30 older adult women are seen in general gynaecology clinics.²

What can I expect if I have lichen sclerosus?

Lichen sclerosus is a chronic disease that will require long-term treatment to manage symptoms. Practising good hygiene might help reduce some of the symptoms of lichen sclerosus. The symptoms frequently return, even after they are treated. Therefore, long-term follow-up care is necessary. LS can increase the risk of developing squamous cell skin cancer and should be regularly followed up by a doctor.

When should I see a doctor?

Consult your GP if you experience vaginal or anal irritation, itching, or pain. In order to receive treatment, you must identify the source of your symptoms. Sexually transmitted infections (STDs), among other illnesses, are known to produce signs and symptoms that resemble those of lichen sclerosus.  

Summary

Lichen sclerosus is a skin condition that causes chronic inflammation and scarring. Its exact cause is unknown. Risk factors for lichen sclerosus include autoimmune diseases, trauma and hormonal state. In people AMAB, circumcision greatly lowers the risk of LS. The lesions may progress to cause functional impairment, anatomical structural destruction, and a chance of developing cancer. A skin cancer called squamous cell carcinoma is more likely to occur in people with lichen sclerosus. The diagnosis of LS based on clinical characteristics is usually sufficient. Skin biopsies should only be carried out in cases of clinical uncertainty, differential diagnosis, or suspected cancer. Although lichen sclerosus is not life-threatening, it can cause discomfort, if left untreated. Strong topical steroid cream is the primary line of treatment, and surgery is necessary if the scarring has caused anatomical changes. A long-term follow-up is essential for early diagnosis and to prevent complications, allowing for appropriate therapy.