Lichen sclerosus

Lichen sclerosus (LSc) is a chronic, inflammatory skin disease, of disputed cause, which can affect any body part of any person, but has a strong predilection for the genitals (penis, vulva); it has historically been called balanitis xerotica obliterans when it affects the penis. LSc is not contagious. There is a well-documented increase of genital cancer risk in LSc, potentially much reduced with early diagnosis and effective, definitive treatment, especially in men. LSc in adult age women is held to be incurable, although treatment can lessen its effects, and it often gets progressively worse if not treated properly. Most males with mild or intermediate disease, restricted to the foreskin or the glans penis can be cured by either medical or surgical treatment.

Signs and symptoms

LSc can occur without symptoms. White patches on the affected area, itching, pain, dyspareunia (in genital LSc), easier bruising, cracking, tearing and peeling, as well as hyperkeratosis, are common symptoms in both men and women. In women, the condition most commonly occurs on the vulva and around the anus with ivory-white elevations that may be flat and glistening. In men, difficulty retracting the foreskin, tightening of the foreskin and episodes of 'thrush' or cystitis, prostatitis and urethritis may be accompanying features of the history. An acute presentation is paraphimosis, where the foreskin has become fixed in the retracted state and cannot be replaced. In males, the disease may present in several ways. Symptoms and signs may be subtle or florid. Lichenoid inflammation (creating 'waisting' or an 'hourglass' constrictive posthitis) and Zoonoid inflammation can be encountered and co-exist; Zoon's balanitis in the absence of LSc probably does not exist. Progressive tissue damage causes a waxy pallor, etiolated tissue and then frank sclerosis in the form of grey/white patches on the glans and foreskin. It also causes post-inflammatory hyperpigmentation and melanosis. Adhesions, partial or complete, sub coronal and trans coronal occur and the frenulum can become sclerotic and obliterated. Loss of dermal support creates telangiectasia and angiokeratomas. Progressive preputial sclerosis and narrowing (preputial stenosis), forming an 'indurated ring', which can make retraction more difficult or impossible (phimosis -'muzzling'). Meatal stenosis, making it more difficult or even impossible to urinate, may also occur. In contrast to women, anal involvement in men is much less frequent. On the non-genital skin, the disease may manifest as porcelain-white spots with small visible plugs inside the orifices of hair follicles or sweat glands on the surface. Thinning of the skin may also occur.

Psychological effect Distress due to the discomfort and pain of lichen sclerosus is normal, as are concerns with self-esteem and sex. Counseling can help. According to the US National Vulvodynia Association, which also supports women with lichen sclerosus, vulvo-vaginal conditions can cause feelings of isolation, hopelessness, low self-image, and much more. Some women are unable to continue working or have sexual relations, and may be limited in other physical activities. Depression, anxiety, and even anger are all normal responses to the ongoing pain LSc sufferers experience. Moreover, men also experience lowered quality of life, such as an impairment in daily activities and mental disturbances, albeit to a lower degree, since there are treatments that cure the disease more promptly.

Pathophysiology Although it is not completely established what causes LSc, several theories have been postulated. It is unanimously agreed that LSc is not contagious and cannot be caught from another person. Several risk factors have been proposed, including exposure to the irritant effects of urine, autoimmune diseases, infections and genetic predisposition. There is evidence that LSc can be associated with thyroid disease.

Exposure to urine There is a growing body of evidence, especially in men suggesting that prolonged exposure of susceptible tissues to the irritant effects of urine may contribute to the development of LSc. Urine droplets that leak after urination can become trapped in the external genitalia (e.g., beneath the foreskin), creating an occluded environment that creates irritation and inflammation. Several observations support the "urine occlusion hypothesis," including:

Rare incidence in circumcised individuals: LSc is extremely rare in individuals circumcised during childhood. Resolution through circumcision: In affected men, LSc can be effectively treated or cured by circumcision. Recurrence with neo-foreskin formation: In circumcised men who later develop a neo-foreskin over time, the disease can reappear (especially associated with obesity). Pattern of distribution: The typical distribution of lichen sclerosus corresponds to areas exposed to urine. In men, the condition is usually confined beneath the foreskin, while in women, it can involve the perianal region. Lichen sclerosus in urine-diverted sites: When urine is diverted to other areas of the body, the skin in those regions can also develop lichen sclerosus. Association with incontinence: Men with LSc have been shown to "dribble" and similar findings have been published in women. In addition, urological procedures or genital piercings that disrupt the urethra and lead to post-void dribbling may also contribute to microincontinence and the subsequent development of LSc.

Genetic LSc may have a genetic component. A strong correlation with LSc has been reported between twins and between family members.

Autoimmunity Autoimmunity is a process in which the body fails to recognize itself and therefore attacks its own cells and tissue. Specific antibodies have been found in LSc sufferers. Furthermore, there seems to be a higher prevalence of other autoimmune diseases such as diabetes mellitus type 1, vitiligo, alopecia areata, and thyroid disease.

Infection Both bacterial and viral pathogens have been implicated in the etiology of LSc. A disease that is similar to LSc, acrodermatitis chronica atrophicans, is caused by the spirochete Borrelia burgdorferi. Viral involvement of HPV and hepatitis C are also suspected. A link with Lyme disease has been stated because of claims for the presence of Borrelia burgdorferi in LSc biopsy tissue. However this has been challenged by others.

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