Lichen sclerosus is a chronic inflammatory skin disease that typically involves the vaginal and anal areas of the body but may also spread to other areas in rare cases. Lichen sclerosus is most common in females—especially post-menopausal females—but males and children can also be affected. Lichen sclerosus is characterized clinically as skin that is white, thin and patchy and may have a wrinkled, fragile or discolored appearance. Affected skin is usually itchy, but can often become ripped or bleed, and it may soon become uncomfortable. The most common presentation for females is vulvar lichen sclerosus, which affects the external vaginal area. In males, it is referred to as balanitis xerotica obliterans when it affects the penis. Lichen sclerosus targets the epidermis (the outer layer of skin) primarily by thinning the outer layer. Inflammatory cells may also migrate into the leathery inner layer of the skin (dermis), resulting in pain, discomfort and chronic itching.

The exact cause of lichen sclerosus is unknown, but a combination of problems with the immune system, hormone problems (especially low estrogen), and hereditary predisposition may lead to the condition. It is important to remember that lichen sclerosus is not an infectious disease and cannot be transmitted from person to person or through sexual intercourse. Lichen sclerosus is a chronic disease and there is no cure; however, it can be managed very well, and complications can be avoided through various treatments, primarily topical corticosteroids.  Early recognition and appropriate medical treatment are critical to minimizing the burden of lichen sclerosus on daily life and long-term health.

If it is not treated, lichen sclerosus can result in scarring that can cause the discomfort of painful sexual intercourse (dyspareunia), urinary problems or bowel problems. In very rare cases, lichen sclerosus may increase the risk of certain skin cancers without treatment, namely vulvar squamous cell carcinoma or penile cancer. Lichen sclerosus has sometimes been called white spot disease (due to the appearance of pale, white patches on the skin).

There are several identified contributing factors to lichen sclerosus, although the actual etiology is unknown. Experts believe that lichen sclerosus may develop because of a combination of environmental, hormonal, genetic and immunological factors. Here are some possible known contributors:

1. The immune system is overactive: Lichen sclerosus is often classified as an autoimmune disease, wherein healthy skin tissue is attacked by the immune system erroneously. There is an association with other autoimmune diseases such as vitiligo, areata alopecia and thyroid disease.
2. Genetic propensity: Some research suggests a genetic component may play a role in lichen sclerosus; however, no specific gene has been identified as the cause.
3. An imbalance in hormones: The disorder often manifests during times of hormonal change, such menopause or childhood, which may indicate a connection to lower estrogen levels.  Hormonal variables are thought to play a role in the development of lichen sclerosus, while the precise relationship between estrogen and the disease is not entirely understood.
4. Irritation or skin trauma: In individuals who have genetic or immunological vulnerability, lichen sclerosus can develop as a result of minor skin damage or chronic irritation. The Koebner reaction is termed to describe the mechanism which occurs when skin damage results in the development of new lesions.
5. Potential infections: While some researchers have studied the infectious trigger concept, there is no definitive or dependable association.

It is important to remember that lichen sclerosus is not an STI (sexually transmitted infection) and does not result from poor hygiene or sexual activity. Circumcision may greatly lessen a man’s risk for developing lichen sclerosus, especially if it is on the foreskin of the penis.

A minor instance of lichen sclerosus may not produce any symptoms at all. However, when symptoms do manifest, the symptoms vary depending on the site of involvement and the severity of the condition. Here are common symptoms and signs:

• Severe pruritus (itchiness): Constant itching is one of the most prevalent and upsetting symptoms, particularly in the vulvar or anal region.
• Shiny White Patches: In addition to being white, thin, smooth, and glossy, the afflicted skin may occasionally have a crinkly or wrinkled appearance.
• Pain and Unease: Pain, soreness, or burning could be experienced, particularly when urinating or having sex.
• Vulnerable Skin: Even minor amounts of stress can lead to fragile skin, which can cause it to rip, bruise or break. 
• Skin rashes or blisters: More serious situations may lead to ulcers, blisters or bleeding, and may increase the risk of secondary infections.
• Shrinkage and Scarring: Skin shrinkage and scarring can result from persistent inflammation.  This can cause phimosis (tightening of the foreskin) in men and limit the vaginal opening in women.
• Skin Thickness: If the illness is not treated, long-lasting lichen sclerosus can create areas of tougher, leathery skin due to continual scratching and inflammation. 

The disease is only identified in a standard physical exam, and some individuals may not be experiencing any symptoms. 

Importantly, lichen sclerosus does not affect internal reproductive organs such as the ovaries, uterus or vagina.

Lichen sclerosus is not contagious.  It cannot be passed from one person to another through sexual intercourse or other forms of skin-to-skin contact.  It is neither transmissible from one person to another, nor caused by infection or hygiene.

To identify lichen sclerosus, medical professionals often conduct a thorough medical history and clinical examination of the affected areas.  The medical historian and clinician will be seeking typical signs including fragility, scarring, white patches, and thinning skin.

If there is any uncertainty about the diagnosis or concern for possible malignancy, a skin biopsy may be performed. This procedure is done under local anesthetic and involves removing a small section of skin to look at it under a microscope. Typical results from a biopsy include:

• Thinning of the epidermis
• Loss of rete ridges
• Inflammation in the deeper dermis

Timely and appropriate diagnosis is essential in securing effective treatment and avoiding complications—including progression to squamous cell carcinoma in rare circumstances.

While there is not yet a cure for lichen sclerosus, there are many effective treatments that can help to manage symptoms, increase comfort and prevent complications. Long-term care is often required.

• Corticosteroids used topically: The primary course of treatment is potent steroid creams, such as clobetasol propionate 0.05%. These are effective in reducing pain, itching and inflammation. Daily treatment is then tapered to a schedule to prevent recurrence.
• Topical Inhibitors of Calcineurin: Patients who are unable to tolerate long-term steroid therapy may be prescribed medications such as tacrolimus or pimecrolimus. These medications alter the immune system without thinning the skin.
• Male Circumcision: Circumcision can result in total resolution for males with foreskin involvement, especially in cases where topical treatments have failed.
• Barrier Creams and Moisturizers: Using moisturizers without fragrance allows the skin to remain non-irritated and moisturized. Barrier creams can protect the skin from irritants and friction, such as tight clothing or urine.
• Topical Estrogen (in some circumstances): Estrogen creams are used specifically in post-menopausal women to increase the flexibility and quality of tissue, but they are not typically considered first-line treatment.
• Surgical Procedure: In cases of significant scarring or anatomical changes, corrective surgery may be necessary. After surgery, however, topical therapies must be utilized to prevent recurrence.
• Phototherapy: In cases of lichen sclerosus that affect non-genital areas, ultraviolet light therapy—particularly narrowband UVB—may reduce inflammation and pain.  It is not commonly used for genital involvement.
• Supportive Care: Supportive measures play an essential role and include:
  • Applying emollients like petroleum jelly
  • Washing gently with mild, unscented soaps
  • Avoiding irritants such as tight clothing and scented hygiene products
• Topical and Oral Retinoids: Retinoids, which are derivatives of vitamin A, are only sometimes needed in difficult to treat cases. They modify skin cell turnover which improves such symptoms as itchiness and is usually limited due to most patients responding to other treatment modalities.

Follow-Up Care:

There is no substitute for regular follow-up with a healthcare provider to monitor side effects, monitor treatment response, and screen for other issues such as skin cancer. Regular medical care, especially in the vaginal area, also has some impact on reducing the chance of anatomical deformation and scarring.

Lichen sclerosus is a chronic inflammatory skin condition that usually affects the anal and vaginal areas. While we are not clear on the exact cause, there are possible contributors, including autoimmune dysfunction, genetic disposition, hormonal effects and environmental triggers. Symptoms can include very itchy, white patches and skin changes, all of which can greatly impact mental health and quality of life. While there is no cure, the disease is manageable. Strong topical corticosteroids are the mainstay of treatment and are usually effective in curtailing symptoms and preventing worse effects over the long term such as risk of cancer, and scarring. Along with medical treatment, lifestyle changes and psychological support is important for improving quality of life with lichen. If you think you might have lichen sclerosus, it is essential to talk to your healthcare provider, in order to get the proper diagnosis and treatment. With early intervention and effective management, it is quite possible to live well with lichen sclerosus.

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