Lichen sclerosus: The need for extensive research
Lichen sclerosus is a skin disease that causes great amount of discomfort, and globally many people are afflicted. Though the cause of acquiring lichen sclerosus is unknown, we shed light on the symptoms, causes and treatments available today.
Lichen sclerosus (LS) is an uncommon condition affecting the skin around genitalia that results in patchy, white skin that is thinner than normal. It may affect the skin on any part of the body, but most often involves the skin of the vulva, foreskin of the penis or skin around the anus. On the non-genital skin, the disease may manifest as porcelain-white spots with small visible plugs in the hair follicles or sweat glands that may evolve into blotchy, wrinkled patches. The disease often goes undiagnosed for several years, as it is sometimes not recognised and not correctly diagnosed until the patient is referred to a specialist when the problem persists. “Today, people of all age groups are getting affected with LS, however, the incidences are more in females, especially post-menopausal females, as well as children. Occasionally, spontaneous cure may ensue, particularly in young girls. Male genital LS is seen almost exclusively in uncircumcised or incompletely circumcised men and boys,” informs Dr Nitin S Walia, Consultant – Dermatology, Max Hospital.
The age of LS ranges from 3 to 65 years and most cases are seen in people ageing 51-60 years. According to a research conducted by Department of Dermatology and STD, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), in 2007, LS was found in 69.2 per cent women who were post-menopausal, followed by 19.2 per cent in reproductive age group, and 11.5 per cent girls who had not attained menarche.
The cause is unknown, although an overactive immune system may play a role. Some people may have a genetic tendency toward the disease, and many studies suggest that abnormal hormone levels may also play a role. Some scientists believe that an infectious bacterium, called a spirochete, may cause the changes in the immune system that lead to LS. But the fact that most women that get LS are of menopausal age and also have other auto-immune issues seems to indicate there is a hormonal and auto-immune connection. The disease is incurable, and can only be symptomatically managed. “Chronic inflammation/ low grade infection predisposes to this condition. There is a higher prevalence of other autoimmune diseases such as diabetes mellitus type 1, vitiligo and thyroid disease in these patients. There may also be a genetic component as several familial cases and a pair of identical affected twins has been described. LS is primarily found in women with a low estrogen state though hormonal influences have been postulated with little conclusion. Both bacterial as well as viral pathogens have been implicated in the aetiology. A link with Lyme disease is shown by the presence of Borrelia Burgdorferi in the affected biopsy tissue. Viral involvement of HPV and Hepatitis C are also suspected. Some findings suggest that LS can rarely be initiated through scarring or radiation,” says Dr Walia. Further elaborating on the cause of LS, Dr Vanita Mathew, Consultant Dermatologist & Aesthetic Surgeon, Apollo Hospital, Bengaluru, says, “Stress also plays a very important role in getting LS; one might get cured with medication, but if the stress levels are high, the person is likely to develop LS again, hence it is extremely important for a person to keep their stress levels under control.”
Symptoms of the disease
Symptoms of LS vary depending on the area affected. A number of patients experience different degrees of discomfort. When LS occurs on parts of the body other than the genital area, most often there are no symptoms, other than itching. If the disease is severe, bleeding, tearing, and blistering caused by rubbing or bumping the skin can cause pain.
Very mild LS of the genital area may cause itching; however, it often causes no symptoms at all (in early stages). Rarely, LS of the vulva may cause extreme itching that interferes with sleep and daily activities. Also, when LS develops around the anus, the discomfort can lead to constipation. This is particularly common in children. Most men with genital LS sometimes experience difficulty pulling back the foreskin and have decreased sensation at the tip of the penis. Occasionally, erections are painful, and the urethra can become narrow or obstructed.
Available treatment options
The most commonly prescribed treatment for LS is corticosteroid ointments or creams. “However, asymptomatic extra genital LS usually require no treatment as control of pursuits rather than resolution of the lesion, which is a more realistic goal of therapy. In fact, many cases disappear without any treatment,” informs Dr Mathew.
The calcineurin inhibitors (tacrolimus and pimecrolimus) – is often used for maintenance therapy with varied results. “Genital LS may respond to potent topical corticosteroids, although the patient is warned that the clinical appearance does not always reverse, even if symptoms are relieved. It is widely reported that prepubertal LS in girls may resolve spontaneously although some of these patients may suffer from vulvodynia in adulthood,” says Dr Walia.
“Ultraviolet light therapy is also a good option for extra genital lesions as it is safe,” says Dr Walia. Talking about the importance of surgery in LS, he says, “Surgery is advised only for severe, recalcitrant cases in form of circumcision, vulvectomy etc. For men with LS on the foreskin, removal of the foreskin (circumcision) is a common treatment in cases resistant to other therapies or more advanced cases. An operation to widen the opening of the vagina is occasionally needed in women with severe LS.”
“LS has affected many people today; this problem is more common in women than in men, and the ratio varies form 1:5 to 1:6 especially in post- menopausal women and they are 15 per cent more likely to develop LS. Looking at the rate at which people are suffering from LS it is important that we find new drugs and better solutions to treat this disease, but what is most important is to find the cause of LS. Only once the cause is known will the doctors be able to provide better medication and relief to its patients,” concludes Dr Walia. Skin that has been scarred as a result of LS is likely to develop skin cancer. Women with LS may develop vulvar carcinoma. Periodic consultation is thus necessary. The future however looks bright with development of better more effective immunomodulators and safer drugs.