Lichen Sclerosus: Causes, Symptoms & Treatment

Lichen sclerosus represents a rare disease of the skin with inflammation-related changes in connective tissue, the cause of which is thought to be a dysregulation of the immune system. Women are generally 5 to 10 times more likely to be affected by lichen sclerosus than men.

What is lichen sclerosus?

Lichen sclerosus is the name given to a rare chronic inflammatory connective tissue disease that is thought to be due to a dysregulation of the immune system (autoimmune disease). Lichen sclerosus usually manifests in the genital area, less frequently in extragenital areas such as the hands, arms or back (about 10-15%). While in women mainly labia (labia majora), clitoris, clitoral prepuce as well as the perianal area (region around the anus) are affected, in men lichen sclerosus manifests itself on the prepuce (praeputium), glans penis and only occasionally in the perianal region. Characteristic of lichen sclerosus are porcelain-like, white skin discolorations with reactive thickening of the epidermis (keratinization) caused by sclerosis (inflammation-induced proliferation) of the underlying connective tissue. As a result of sclerotic atrophy (thinning of tissue) of the affected areas of skin, phimosis (narrowing of the foreskin) may develop in males and shrinkage of the labia or clitoris in females, leading to pain during sexual intercourse. Pruritus (itching), urethral strictures with painful urination, and vaginal stenosis are other possible symptoms of lichen sclerosus.

Causes

The etiology of lichen sclerosus is largely unexplained to date. A multifactorial genesis of the disease is suspected in the presence of a genetic predisposition, although no familial clustering has been observed. In all probability, lichen sclerosus is due to a dysregulation of the immune system (autoimmune disease). In several studies a reactivity against ECM1, an extracellular matrix protein, could be detected in the serum of the majority of affected patients. It is not yet known which factors trigger these dysregulated processes. Trauma (including sexual abuse, scratching) are considered possible trigger factors. In addition, lichen sclerosus is associated in nearly 30 percent of cases with other autoimmune diseases such as Hashimoto’s thyroiditis, type 1 diabetes mellitus, asthma, lupus erythematosus, autoimmune gastritis, circular hair loss (alopecia areata), and vitiligo (white spot disease). Similarly, Borrelia (bacteria causing Lyme disease) is discussed as a disease trigger for lichen sclerosus.

Symptoms, complaints, and signs

Lichen sclerosus is usually noticeable in affected adolescents and adults by episodic itching or burning and stinging in the genital area. In addition, skin changes may occur that initially resemble a fungal infection on the outside. Problems during sexual intercourse, especially during penetration, are also characteristic. In some women, a strong feeling of soreness develops in the intimate area following sexual intercourse. This may last for several days and be associated with bladder problems, pain and inflammation. In men, white, mucus-like plaques form in the penile area and have an unpleasant odor. Painful skin lesions may form, first appearing on the foreskin and then spreading to the glans and the entire penis. In children, lichen sclerosus is noticeable by a symmetrical redness of the genitals. Symptoms of the disease usually appear quite suddenly and increase in intensity as the disease progresses. Eventually, severe pain and noticeable skin changes present themselves. If lichen sclerosus is treated early, the symptoms usually subside after a few days to weeks. If adequate therapy is not given, ulcers and scars may develop in the affected area.

Diagnosis and course

An initial suspicion of lichen sclerosus usually results from the characteristic symptoms (especially the porcelain-like, white skin scarring) and a gynecologic examination with a colposcope (microscope for checking the vagina and cervix). The diagnosis is confirmed by a biopsy (sample taken from the affected skin area with a punch cylinder) with subsequent histological (fine tissue) analysis.The histological findings also serve to exclude malignant degeneration and to differentiate lichen sclerosus from partially comparable diseases such as genital mycosis or circumscritic scleroderma (also morphea). As a chronic disease, lichen sclerosus is not considered curable and progresses in relapses, with symptom-free intervals between relapses. If left untreated, lichen sclerosus leads to atrophy, chronic pruritus, and synechiae (adhesions of tissue layers) and even degeneration of the affected skin areas.

Complications

Due to this disease, patients suffer from various symptoms, all of which are very unpleasant and can lead to a reduced quality of life. Those affected suffer from itching and from itchy wounds. Wound healing is also delayed by this disease, so that infections or inflammations can occur more often. Pain during urination is also not uncommon. These are burning and not infrequently lead to psychological discomfort or depression. It is also not uncommon for bleeding to occur on the skin. In men, the disease can also cause discomfort in the genitals and especially in the foreskin. This can also lead to restrictions in sexual intercourse. Treatment of this disease is usually carried out with the help of medication. Complications usually do not occur and the course of the disease is positive. However, a new outbreak of this disease is not reduced by the treatment. The life expectancy of the affected person is not reduced. Likewise, the affected person must refrain from using various care products to stabilize the skin in the affected areas.

When should one go to the doctor?

Abnormalities in the genital area are in most cases indications of an existing disease. A visit to the doctor is advisable if existing symptoms persist unabated for several days or weeks. If the symptoms increase in intensity or become more widespread, a visit to the doctor is required immediately. If there is pain or a burning sensation on the skin, a doctor is needed. If itching or open sores occur, consultation with a physician is required. Pathogens and germs can enter the organism through the open wounds and cause further diseases. In severe cases, there is a risk of blood poisoning and thus a life-threatening condition. A visit to the doctor is therefore advisable in the event of the formation of pus, fever or a general feeling of indisposition. If a feeling of soreness develops after sexual intercourse, this should be observed. If there are mucous coatings or an unpleasant body odor in the intimate area, a visit to the doctor is necessary. Redness of the skin, swelling or bleeding should be examined and treated. If women experience irregularities in their menstrual cycle, a doctor should be consulted. If sexual dysfunction develops in men or women, a doctor should be consulted. Likewise, if a man notices changes in the foreskin. If ulcers or scars develop, observations should be discussed with a physician.

Treatment and therapy

Therapeutic measures for lichen sclerosus are aimed primarily at alleviating symptoms. For this purpose, highly potent topically applied corticosteroids (including clobetasol propionate) are generally used as part of a shock therapy with high initial doses. Another therapeutic option is treatment with calcineurin antagonists such as pimecrolimus or tacrolimus for immunosuppression. Although advanced atrophy cannot be reversed by these drugs, the progression of the disease is slowed and in the best case stopped. While the previously assumed efficacy of testosterone has not been proven, topical progesterone treatments for lichen sclerosus are being tested in clinical trials. In addition, fat-containing creams (water-in-oil creams), ointments or oils are recommended for additional care of the affected skin areas as well as stabilization of the skin barrier while avoiding irritating shower lotions, soaps or perfumes. If infectious diseases are also present, these should be treated appropriately with anti-infective therapy (including antifungals or antibiotics).Whereas in women the inflammatory processes can only be controlled and restricted, and today surgical measures such as vulvectomy or skin grafts are not used because of the high recurrence rate, in men with lichen sclerosus and the presence of phimosis circumcision (circumcision) is used in most cases, through which the disease can often be stopped.

Outlook and prognosis

Lichen sclerosus is a chronic disease – especially in girls and women – that cannot be cured in most cases. It is therefore lifelong and only the typical symptoms of the disease can be alleviated with a well-adjusted therapy. The earlier treatment is started, the easier it is to control the disease. If lichen sclerosus is diagnosed late, the therapy proves to be more difficult, because in most cases complications such as internal adhesions have already occurred. Up to the present time, no cure is possible, especially in adult women. In boys as well as men a cure by circumcision is conceivable. Basically, lichen sclerosus is a benign disease. Nevertheless, the risk of developing skin cancer is increased. For this reason alone, those affected should visit their dermatologist regularly in order to have malignant changes in the skin detected and treated at an early stage. The current treatment options do not have the same effect on all patients, which is why the general level of suffering varies greatly from patient to patient. Careful management of the affected skin areas during and also outside the disease episodes is very important for a favorable prognosis.

Prevention

Because the causes and trigger factors for lichen sclerosus have not yet been determined, the disease cannot be prevented. However, certain forms of lichen sclerosus (including the hyperplastic variant) are considered noninfection-associated risk factors for malignant degeneration (squamous cell carcinoma), which is why affected individuals, especially women, should undergo regular medical examinations.

Follow-up

The measures of an aftercare depend with skin diseases as a rule very strongly on the exact illness, so that thereby usually no general prediction can be made. The same applies to the disease lichen sclerosus: the earlier this disease is detected and treated by a doctor, the better the further course, which is why the affected person should see a doctor at the first symptoms and signs. Follow-up care focuses on strengthening the weakened immune system and thus advancing the recovery process. Relaxation exercises can help, as can avoiding stress. A high standard of hygiene can have a positive effect on the course of such diseases. In most cases, treatment is carried out by applying creams or ointments and taking medication. The affected person should pay attention to regular application and also to the correct dosage in order to permanently alleviate the symptoms. In this context, regular checks by a doctor are very important.

What you can do yourself

In the treatment of lichen sclerorus, the focus is on alleviating the symptoms. For this purpose, in addition to various medications, conservative household and natural remedies are used, as well as some self-help measures. The most important measure is to prevent attacks. This is achieved by recognizing and avoiding possible causes and triggers. A change in diet is particularly important. Patients should talk to a specialist about this and also consult other sufferers. In this way, it can be quickly determined which individual triggers are present. People suffering from lichen sclerosus should maintain a healthy lifestyle overall. Regular exercise, sufficient sleep, avoidance of stress and other measures that strengthen the immune system can significantly reduce the symptoms of this rare skin disease. Since certain forms of lichen sclerosus carry an increased risk of cancer, those affected should undergo regular medical examinations. It is advisable to consult a specialist at an early stage and to work out a suitable therapy together with him. Accompanying the above-mentioned measures must be carried out regularly in order to avoid an attack and the associated complaints.