Lichen Sclerosus: Symptoms, Types, Causes and Treatment

Lichen sclerosus is a chronic, inflammatory skin disorder that can significantly affect quality of life. While it most commonly targets the genital and anal regions, it can also appear elsewhere on the body. This article provides a comprehensive overview of lichen sclerosus, including its symptoms, types, causes, and treatment options, synthesizing the latest evidence-based research.

Symptoms of Lichen Sclerosus

Living with lichen sclerosus can be challenging, as its symptoms are often both physically uncomfortable and emotionally distressing. Recognizing the signs early is crucial for effective management and to prevent complications. Symptoms may vary depending on age, sex, and the affected area, but certain clinical features are particularly characteristic of the disease.

Symptom	Description	Prevalence/Groups	Source(s)
Itching	Persistent, often severe pruritus	Common in all	1 2 5 11
Pain	Burning, soreness, or stinging sensation	Adults & children	2 3 5
Dyspareunia	Painful intercourse	Women	2 5 10
White Plaques	Porcelain-white or ivory patches/plaques	All; genital > extragenital	4 5 6 7

Table 1: Key Symptoms

Itching and Discomfort

The most frequent symptom of lichen sclerosus is intense itching, which can be relentless and severely affect daily activities and sleep. Many patients also report pain, burning, and general discomfort—particularly in the genital or anal areas 1 2 5. In children, pain may be accompanied by difficulty with urination or defecation due to fissures or ulcers 3.

Skin Changes and Lesions

Lichen sclerosus typically presents as shiny, white (porcelain or ivory-colored) patches or plaques with a glistening or wrinkled “cigarette paper” appearance 4 5 6. These lesions are sharply demarcated from healthy skin and, over time, may become atrophic (thin and fragile), sometimes resulting in bleeding or bruising, especially in women and children 3 4 5. The classic “figure-of-eight” pattern is often seen around the vulva and anus 3 5.

Functional Impairment

Chronic inflammation and scarring can cause anatomical changes, such as shrinking of the genital area, narrowing of the vaginal opening, or, in men, tightening of the foreskin (phimosis) 1 2 5. These changes can lead to painful intercourse (dyspareunia), painful urination (dysuria), and, in severe cases, difficulty passing urine or stool 2 3 5. In children, anal or vulval bleeding can occur 3.

Emotional and Psychosexual Impact

The distress caused by persistent symptoms, scarring, and altered genital appearance may lead to significant emotional and psychosexual challenges, including anxiety, embarrassment, and sexual dysfunction 2 5 11.

Types of Lichen Sclerosus

Lichen sclerosus isn’t a one-size-fits-all condition. Its clinical presentation varies based on location, age, and extent, which can influence both symptoms and management strategies.

Type	Predominant Location	Special Features	Source(s)
Anogenital	Vulva, penis, perianal	Figure-of-eight pattern, symptomatic	1 4 5 7
Extragenital	Trunk, limbs, upper body	Less common, may be asymptomatic	1 4 7 8
Pediatric	Mostly genital	Mimics abuse, possible spontaneous resolution	3 4 10
Generalized	Multiple body sites	Rare, widespread involvement	1 4

Table 2: Lichen Sclerosus Types

Anogenital Lichen Sclerosus

The vast majority of cases involve the anogenital region, affecting the vulva and perianal area in women and the foreskin, glans, and sometimes perianal area in men and boys 1 4 5 7. The “figure-of-eight” configuration around the vulva and anus is classic in females, while in males, the foreskin and glans are most often affected. Genital disease tends to be more symptomatic due to the sensitivity and function of the area 4 5.

Extragenital Lichen Sclerosus

While less common, lichen sclerosus can appear on the trunk, upper arms, axillae, thighs, and buttocks 1 4 7. Extragenital lesions present as similar white, shiny plaques but are often less symptomatic and may go unnoticed. About 6–20% of patients have extragenital involvement, and 18% of women with genital disease report extragenital lesions 1 4.

Pediatric Lichen Sclerosus

Children, especially prepubertal girls, may develop lichen sclerosus predominantly in the genital area. It can mimic signs of sexual abuse—such as fissures, ulcers, and bleeding—leading to misdiagnosis and distress 3 4. Fortunately, spontaneous resolution at puberty is possible for some children, although lesions can persist into adulthood 3 4.

Generalized Lichen Sclerosus

Rarely, the disease can be widespread, affecting multiple body sites. The course can be variable, with either long periods of inactivity or rapid progression 4.

Causes of Lichen Sclerosus

Despite decades of research, the precise cause of lichen sclerosus remains unclear. However, significant advances have been made in understanding the underlying mechanisms and risk factors.

Cause/Factor	Description	Evidence Level	Source(s)
Autoimmunity	Immune system attacks skin	Strong	2 11 13 14
Genetic	Familial clustering, HLA associations	Moderate	1 10 11
Hormonal	Previously suspected, now less likely	Weak	2 13
Trauma/Koebner	Physical injury or chronic irritation	Noted in some cases	2 14
Infections	Possible trigger (Borrelia, HPV?)	Controversial	2 13 14
Occlusion	Moist, occlusive environment as a factor	Possible	2 13

Table 3: Key Causes and Risk Factors

Autoimmune Mechanisms

The most compelling evidence points toward lichen sclerosus being an autoimmune disorder. Many patients have other autoimmune diseases, and specific immunogenetic markers (like HLA DQ7) are more common in those affected 2 11 13 14. The immune system appears to attack skin components, leading to chronic inflammation and tissue changes.

Genetic Predisposition

Lichen sclerosus can run in families, and genetic factors may increase susceptibility. Studies have shown familial clustering and links to certain genes involved in immune regulation 1 10 11. However, it is not directly inherited in a simple pattern.

Hormonal and Environmental Factors

Although hormonal changes were once considered a primary cause—especially given the higher incidence in postmenopausal women and prepubertal children—current research suggests hormones play a minimal role 2 13. Environmental triggers such as trauma, chronic irritation, or an occlusive (moist) environment may precipitate or exacerbate the condition in predisposed individuals 2 13 14.

Infections

There is ongoing debate about whether infections—such as Borrelia (the bacteria that causes Lyme disease) or human papillomavirus (HPV)—might trigger lichen sclerosus. Evidence is inconclusive, and infections are not considered a main cause 2 13 14.

Trauma and Koebner Phenomenon

Some cases arise at sites of previous trauma or irritation, supporting the “isotraumatopic” response, where skin injury leads to disease expression in genetically susceptible individuals 14.

Treatment of Lichen Sclerosus

Managing lichen sclerosus focuses on relieving symptoms, preventing complications, and minimizing long-term risks, such as skin cancer. While there is currently no cure, several effective treatments are available.

Treatment	Application/Indication	Effectiveness/Notes	Source(s)
Topical Corticosteroids	First-line, high-potency ointments	Most effective, safe long-term	1 2 10 12
Calcineurin Inhibitors	Alternative for steroid-resistant cases	Useful but less evidence	2 13
Retinoids	Selected extragenital/genital cases	For refractory cases	2 13
Phototherapy/PDT	Extragenital/genital lesions	Option for resistant cases	2 8 15
Surgery/Circumcision	For anatomical impairment or in men	Circumcision often effective	1 2 13
Long-term Follow-up	Cancer surveillance, symptom control	Essential for all patients	1 5 9 12

Table 4: Treatments and Strategies

Topical Corticosteroids

Ultra-potent topical corticosteroids (such as clobetasol propionate) are the cornerstone of treatment and are recommended as the first-line therapy for all patients. They are applied daily for several weeks, then gradually reduced to the lowest effective dose for maintenance 1 2 10 12. This approach effectively relieves symptoms, heals lesions, and may prevent disease progression and scarring.

Calcineurin Inhibitors

For patients who do not respond to corticosteroids or cannot tolerate them, topical calcineurin inhibitors (like tacrolimus or pimecrolimus) may be tried. These are generally reserved for refractory cases due to limited data on long-term safety 2 13.

Retinoids and Other Medical Options

Topical or systemic retinoids can be considered in selected cases, particularly for extragenital lichen sclerosus or when other treatments fail 2 13. Their use is less common given potential side effects.

Phototherapy and Photodynamic Therapy

Phototherapy (UV light) for extragenital lesions and photodynamic therapy for genital lichen sclerosus have shown benefits in resistant cases. Photodynamic therapy, in particular, has demonstrated high rates of improvement and good cosmetic results 2 8 15.

Surgical Management

Surgery is generally reserved for those with functional impairment due to scarring or anatomical changes. In men, circumcision is often curative for penile lichen sclerosus, but recurrences can occur 1 2 13. Surgery in women is less common and typically reserved for severe cases.

Long-term Monitoring and Cancer Surveillance

Given the increased risk of squamous cell carcinoma—up to 5% for vulvar lichen sclerosus and some risk for penile disease—long-term follow-up is essential. Any suspicious lesions should be biopsied promptly 1 5 9 10 11 12. Regular monitoring also helps manage symptoms and maintain quality of life.

Conclusion

Lichen sclerosus is a chronic, often under-recognized skin disease that can profoundly impact physical and emotional well-being. Early recognition and appropriate treatment can help prevent serious complications and improve quality of life. Here’s a summary of the key points:

• Symptoms: Intense itching, pain, white shiny plaques, scarring, and, in severe cases, functional impairment and emotional distress 1 2 3 5.
• Types: Most commonly affects the anogenital area but can also appear extragenitally; presentation varies in children and adults 1 3 4 5 7.
• Causes: Likely autoimmune with genetic predisposition; trauma and infections may be contributing factors, but hormones are less critical than once believed 2 10 11 13 14.
• Treatment: High-potency topical corticosteroids are the standard of care; alternatives include calcineurin inhibitors, retinoids, phototherapy, and surgery for select cases. Long-term follow-up is essential due to cancer risk 1 2 10 12 13 15.

Understanding lichen sclerosus and seeking early medical advice can make a meaningful difference for those affected by this challenging condition.