Lichen Sclerosus or Lichen Sclero-Atrophic (LSA) is a chronic inflammatory disease that affects the skin and mucous membranes. Predominantly it affects the genital and perianal areas, but it can also extend (more rarely) to other areas of the body such as the trunk, chest and forearms. Currently, the causes of Lichen Sclerosus remain unknown, it is believed that it may arise due to an abnormality of the immune system that incorrectly attacks its own tissues (called autoimmune disease), just like in other pathologies, such as psoriasis.
Lichen Sclerosus affects both sexes and can be a disabling disorder as it can lead to significant sexual dysfunction in both women and men. In the female sex it has a higher rate, in particular two peaks of incidence are observed: childhood age (prepuberal) and post-menopause. LSA can cause serious consequences and induce significant alterations in vulval anatomy, such as: vaginal entry narrowing, lip disappearance, clitoral hood and sometimes complete closure of the vulva. In men, however, it can cause a narrowing of the foreskin, making it difficult or impossible to uncover the glans.
What are the symptoms of Lichen Sclerosus?
The symptoms of Lichen Sclerosus can range from mild to severe and may differ from person to person. The most common symptoms include:
- formation of white lesions or light spots on the skin;
- intense itching;
- pain;
- bleeding;
- irritation;
- dyspareunia (pain during and after sexual intercourse).
The importance of early diagnosis
Early diagnosis is essential to limit any complications that may arise over time. Certainly, Lichen Sclerosus requires a multidisciplinary medical approach; in fact, several figures such as general practitioner, urologist, gynecologist/andrologist, and dermatologist must collaborate in order to formulate a diagnostic suspicion and send the patient toward further clinical-diagnostic-instrumental investigations. Before being diagnosed with Lichen, patients go through a real calvary for months or years. The causes of this delay in diagnosis are twofold: the disease is sometimes confused with simple genital infections, and at the beginning the symptoms are fuzzy and nonspecific enough to be confused with other diseases. In recent years physicians and gynecologists are much more attentive and sensitive to this disease.
Available treatments
There is currently no definitive cure for Lichen Sclerosus, but there are several treatments available to relieve symptoms and keep the condition under control. The main goal of treatment is to reduce itching, inflammation, and prevent or correct tissue shrinkage.
TOPIC DRUGS
In the topical drugs used to combat Lichen Sclerosus we find:
- Ultrapotent corticosteroid creams (represent the first-line therapy to combat the disease. They are applied directly to affected areas, reduce inflammation and relieve itching).
- Topical calcineurin inhibitors (anti-inflammatory drugs used in the acute phase secondarily as adjuncts to topical corticosteroids).
- Vitamin E (has an elasticizing, antioxidant and anti-inflammatory effect on the skin).
SYSTEMIC DRUGS
Retinoids, Cyclosporine and Methotrexate are also used in the treatment of Lichen Sclerosus with modest efficacy, these drugs are reserved for the most severe forms and their use is limited by long-term side effects.
PELVIC FLOOR REHABILITATION
In recent years, pelvic floor rehabilitation has assumed a key role in the treatment of Lichen Sclerosus Vulvaris. Through physiotherapy, a variety of strategies (stretching, perineal massage, and specific exercises) can be used to work directly on the vaginal tissue to stretch it and make it elastic again.
PHOTODYNAMIC THERAPY
Photodynamic therapies are being used successfully to treat some skin diseases, and for the past few years, physicians have been evaluating their safety and efficacy in the treatment of Lichen Sclerosus as well (although they are not therapies used in the first instance). It is important to consult a dermatologist or gynecologist who specializes in the management of Lichen Sclerosus to evaluate the best treatment options for each individual case. Energy-based therapies include:
- Photodynamic therapy (PDT), a noninvasive method that works through three factors, application of a photosensitizing gel to the skin, irradiation with red light at 630 nanometers, and oxygen normally found in the air. The photosensitizing gel selectively targets cells that cause inflammation and fibrosis, leaving healthy cells intact.
- High Intensity Focused Ultrasound (HIFU), releases thermal energy into the subcutaneous tissue. It stimulates cell proliferation, protein synthesis and revascularization, thus accelerating the reconstruction of tissues damaged by disease.
- Fractional CO2 laser therapy, the laser beam is delivered to small areas (spots), alternating between treated and untreated parts of the skin.
LIPOFILLING AND PLATELET-RICH PLASMA (PRP)
These two techniques involve fat collection and a small amount of blood that is filtered and centrifuged to separate the plasma (platelet-rich blood component) from the other elements. PRP, in fact, contains six to nine times the concentration of platelets than the blood drawn. The two syringes containing fat and PRP are injected into the lesion area in order to promote tissue repair and regeneration by stimulating collagen secretion and modifying the inflammatory response with antibacterial effect.
SURGERY
Normally surgery is considered for the male gender; circumcision (surgical removal of the foreskin) is the most commonly used therapy in fact for men with Lichen Sclerosus with disease limited to the foreskin and glans. In women, narrowing of the vulvar introitus (vestibular stenosis) can be operated on with vulvo-vagino plastic surgery to be reserved for more severe cases. Surgery is not always definitive, with the exception of circumcision, and there can be recurrences.
In the presence of LSA also it is essential to maintain good intimate hygiene, avoid the use of irritating/aggressive soap or detergents, use cotton undergarments to avoid increased skin irritation.
Regulatory framework: the rights of patients with LSA
This is a disease that is not particularly frequent, in Italy it was included in the list of Rare Diseases by Ministerial Decree No. 279/2001 “Regulation for the establishment of the national network of rare diseases and exemption from participation in the cost of related health services” and recently expanded by the Prime Ministerial Decree of 12.01.2017. It is recognized as a rare disease as it belongs to that group of severe, disabling diseases that affect a small number of people and, due to their rarity, are often difficult to diagnose and lack specific therapies. Patients diagnosed with certified LSA are entitled to exemption from co-payment, and recently, some regions have independently provided for additional levels of care and arranged for the free supply of rare disease drugs or other products, including those not classified as drugs, that would otherwise be at the full expense of the citizen.
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The contents of this page are for informational purposes only and should in no way replace the advice, diagnosis, or treatment prescribed by your physician. Responses to the same treatment may vary from patient to patient. Always consult your doctor regarding any information related to diagnoses and treatments, and meticulously follow their instructions.
