Lichen sclerosus: Porcelain between the legs

20. April 2012

Lichen sclerosus, an inflammation of the connective tissue, is considered a rare disease affecting postmenopausal women. Studies show that boys are more often affected than previously thought. Researchers have not yet found the trigger for the inflammatory processes, but there are several suspicious pictures.

Initially often undetected, many such tales of woe begin with unspecific itching and erythema: these symptoms are rather suggestive of a fungal disease and patients tend to deal with it themselves. Lichen Sclerosus (LS) can be concealed behind it.

Incidental finding

If victims make their way to a dermatologist or gynecologist, what sticks out most of all during the examination is white, porcelain-like scarring of the vagina. Left untreated, labia and clitoris shrink over time due to this atrophy event. Orifices of the body then become narrow, pain with bowel movement, urination or intercourse are then inevitable. LS is not as rare as assumed: perhaps up to one percent of all women of all ages suffer; many patients remain free of symptoms at first, so that their condition is discovered incidentally during screening. Again, others seek help in the clinic much too late as a consequence of misplaced shame. Parents also should pay attention to their children for signs of LS.

Children, children …

Recent studies show that the relevance of the ailment has been especially underestimated with boys: LS can lead to phimosis. Therefore, any acquired foreskin tightening should also be looked into from this aspect. Physicians presently usually operate without performing a histological examination. If not enough tissue is removed, it can lead to relapse and an infective attack on the urethra.

With regard to young girls, the situation is more critical in other ways: in its appearance, LS is similar to the physical consequences of abuse. Conversely, sexual violence, via so-called isomorphic effect, can lead to a similar picture after hours or days – for fellow physicians and researchers, that means: act with tact. LS runs, in contrast to other skin irritations, in waves, something which indicates an autoimmune disease, with clues also to be found in the blood count.

Battle in the body

Already early on, evidence accumulated about the involvement of the immune system: Researchers reported on 64 of 86 LS patients having blood reactivity to the extracellular matrix protein (ECM1), in their control group only six of 85 samples were positive. In the search for triggering factors, dermatologists are groping in the dark as earlier, but have been at least able to show evidence of a comorbidity with other autoimmune diseases : in a retrospective analytical study, researchers analysed the medical records of 82 patients who all had LS which had been confirmed via biopsies. Fifteen patients had thyroid disease (most prominently Hashimoto’s thyroiditis), six diabetes mellitus type 1, five asthma, and five were suffering from other autoimmune diseases. These include for instance systemic lupus erythematosus, vitiligo, autoimmune gastritis and circular hair loss. In the skin layers, there were other pathogens found.

The Usual Suspects

Infections involving human papillomavirus (HPV) are the focus of interest. The matter was controversially discussed as to whether LS itself or in rare cases HPV triggers squamous carcinoma. The multidisciplinary guideline for diagnosis and treatment of vulvar cancer evaluates LS as not being an associated risk factor. Several papers estimate the probability of cancer in women as less than five percent of cases, men are affected even more rarely. The next clue: Austrian dermatologists found Borrelia in 38 instances of 60 LS biopsy cases, especially during inflammatory stages of the condition. Should these findings be confirmed, all LS-therapy would demand a rethink: rather than involving antiviral or antibacterial action, control of the inflammation stands today as the exclusive focus.

Smear, smear, smear

At present, inflammatory processes in women can only be held in check, but not cured. Topically applied corticosteroids are considered the gold standard – at first as a kind of ‘shock treatment’ using for instance clobetasol propionate, then switching to milder agents of this type such as hydrocortisone. In addition, water-in-oil creams act as the base element of care by stabilising the skin barrier. Mechanical stress can otherwise lead to cracks and quickly create the ideal entry points for microorganisms. 

Immunosuppressants such as tacrolimus or pimecrolimus are used as a second choice. In a double-blind, randomised, controlled study, researchers have now compared the effect of clobetasol and pimecrolimus. 38 patients with an LS, whose infection was proven via biopsy, participated in the study. After twelve weeks, the first results: clobetasol had a more positive effect on inflammatory processes, however in both groups itching, burning or pain improved quite comparably. From the data the authors concluded that both pimecrolimus and clobetasol are effective and well suited to LS therapy. They advise, however, the use of corticosteroid as the priority, because the long-term effects of topical imunsuppressiva used as maintenance therapy or as prophylaxis is still not able to be judged. Pimecrolimus and tacrolimus could possibly even have an advantage: unlike steroids, they pass through the skin, especially in regions which are no longer intact. With increasing healing process, it is suspected that absorption could decrease as well.

Cut and forget

When men are to be treated, pharmacotherapy essentially is of very little help, and so physicians resort to a scalpel. Their conclusion: via correctly executed circumcisions more than 90 percent of cases of lesions can be resolved. Alternatively, dermatologists report good results with cryosurgery. However, without topical prophylaxis, particularly on the glans or in the urethra, in up to 45 percent of the cases the condition recurs. It’s quite clear: without evidence-based criteria, this will not work in the long term.

Guideline overdue

The differences alone in the treatment of women and men are a challenge for gynecologists, pediatricians and surgeons. Physicians and researchers have been calling for quite some time to put together a multidisciplinary guideline on the diagnosis and therapy of LS – in the UK it is already standard.

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