Lichen sclerosus

A chronic inflammation of the skin that turns it white, thin and fragile. It affects mainly the skin of the genitals and around the anus. It is not catching and has nothing to do with hygiene, but left untreated it leaves scars that alter the shape of the organs and never undo themselves.

It also goes by the name lichen sclerosus et atrophicus, and in women by the older medical word kraurosis vulvae. It is hard to talk about out loud, which is why people put up with the itch for years, blaming it on thrush or on dryness that comes with age. Yet this is one of the few skin conditions in which starting treatment in good time does more than settle the itch: it prevents changes that cannot be reversed.

What happens to the skin

Affected areas turn porcelain white and shiny, with a surface as wrinkled and thin as cigarette paper. The skin loses its strength and tears with ordinary friction, with scratching, with a hard stool; small fissures appear, along with purple patches that are bleeding into the skin with no injury behind it. The leading sensation is itch, usually worst and most exhausting at night, joined by burning, soreness and pain on touch. Beyond that the picture depends on who develops it.

• In women the skin of the labia, the perineum and around the anus turns white; sometimes both areas are involved and the patch takes on a figure-of-eight shape. One important detail: the lining inside the vagina is not affected. That is what separates it from lichen planus, which does involve the vagina, and from menopausal dryness, in which it is precisely the inner lining that changes.
• In men a whitish, firm ring appears at the edge of the foreskin; the foreskin then stops retracting freely, tears with erection and bleeds. If the process reaches the opening of the urethra, that narrows too: the stream becomes thin, sprays, and straining is needed.
• In girls before puberty the skin around the genitals and the anus turns white, itches and bleeds easily with rubbing, and because the fissures hurt the child starts holding back her stool. Stubborn constipation follows and gets treated in its own right without anyone reaching the cause. Whitened skin with bruising and blood often raises the suspicion of abuse; knowing about this condition matters, so that a false trail is not followed.
• In boys it is the commonest reason for a previously normal foreskin suddenly refusing to retract.
• Away from the genitals patches turn up in roughly one person in six or seven: small white shiny marks on the back, chest, neck or wrists. There they usually do not itch, do not scar and do not carry the risk discussed below.

Why it appears

The exact cause is unknown. The most convincing explanation is a fault in the immune system, which begins to inflame the person's own skin with no outside trigger. The company it keeps supports that: women with lichen sclerosus have more thyroid disease, vitiligo, patchy hair loss and vitamin B12 deficiency than average, which is why the doctor often checks the thyroid even when there are no thyroid complaints. A family tendency can be traced as well. What drives the condition on is repeated damage to the skin: the inflammation settles where skin rubs, gets scratched, where a scar has been left or where it stays permanently damp. In men, urine sitting under a foreskin that will not retract is thought to act in the same way.

What it is not matters just as much. It is not an infection and not a sexually transmitted disease. You cannot catch it and cannot pass it on, either through daily contact or through sex; a partner needs no treatment. It is not caused by poor hygiene and does not clear with more of it — on the contrary, diligent washing with soap makes matters worse.

Why waiting costs so much

The inflammation on its own can be endured for years. But while it runs, the skin remodels, and those changes do not come back. In women the inner labia thin down and gradually seem to merge with the surrounding skin, the clitoris ends up buried under a fused hood and the entrance to the vagina narrows, so that sex becomes painful and then impossible. Adhesions can partly cover the outlet for urine: the stream sprays, urine seeps into the folds and burns skin that is fragile already. In men the foreskin tightens into an unyielding ring, erection hurts, tears appear, and narrowing of the urethra gives a thin stream and straining.

Treatment halts the inflammation but does not give back the shape that has gone: the ointment prevents further scarring, it does not release what has already fused. The price of waiting is therefore measured not in months of discomfort but in what will be left for good.

The second reason not to neglect it is cancer. On skin inflamed for years and never treated, some people go on to develop a squamous cell cancer of the vulva or the penis. The lifetime probability is low, of the order of a few per cent, but it is real, and it is lower in those who use the prescribed ointment regularly. None of this applies to patches away from the genitals.

It is worth checking yourself every few months with a mirror in good light, and seeking advice without waiting for the next planned visit if you notice:

• a lump, a nodule or a thickening that was not there before;
• an ulcer or a fissure that has not healed for more than two or three weeks;
• an area that, instead of whitening, goes red, thickens, scales or weeps and does not respond to treatment;
• bleeding with no obvious cause;
• pain in one particular spot, different from the usual itch.

How the diagnosis is made

In most cases a look from a clinician who knows the condition is enough: a dermatologist, a gynaecologist or a urologist. The combination of white colour, wrinkled surface and small haemorrhages is recognised by eye, and no tests are needed to confirm it.

A biopsy — taking a small piece of skin under local anaesthetic — is arranged in three situations: the picture is not quite typical, treatment has produced nothing over a reasonable period, or there is an area suspicious of a tumour. In girls a biopsy is usually unnecessary and the diagnosis rests on examination. It is also worth ruling out a fungal infection along the way: thrush and lichen sclerosus often run together, the itch is similar, and treating only one of the two leaves the complaints unchanged.

How it is treated

The mainstay is an ointment containing a very strong corticosteroid. An ointment rather than a cream: the greasy base stays put longer and stings less on fissures. The logic of the regimen is nearly always the same, though the timings are set by the doctor: daily at first, usually at night, for several weeks running, then a gradual drop in frequency to a maintenance schedule, as a rule once or twice a week but continued long term, often for years. It goes on in a thin layer over the affected areas only, without rubbing it in until it disappears; if a small tube is gone in a week, it is being applied wrongly or in the wrong place.

The commonest mistake is stopping as soon as the itching settles. Itch is the first thing to go, while the inflammation carries on silently and carries on turning skin into scar. That is exactly why maintenance treatment is prescribed at the point when "nothing is bothering me any more": it reduces both the scarring and the risk of malignant change.

The fear of steroid ointments is upside down here. It is the disease that thins the skin, and correctly used ointment that stops it doing so. Genital skin is genuinely sensitive to corticosteroids, and that is precisely why the regimen is chosen by the doctor rather than by the patient, and why treatment is reviewed at follow-up.

If the ointment is not enough or is poorly tolerated, a specialist may prescribe drugs from another group, the calcineurin inhibitors; for unbearable night-time itch something is added to help avoid scratching in sleep. Emollients are used continuously, but as the basis of skin care rather than instead of treatment.

Some things have fallen out of use: testosterone and progesterone ointments, popular decades ago, are now regarded as ineffective. Local oestrogens treat menopausal dryness and are sometimes given alongside, but they have no effect on lichen sclerosus itself.

Surgery does not cure the condition and is not done as a precaution — it corrects the consequences of scarring. In men with a persistently tight foreskin, circumcision often settles the problem for good. In women, adhesions are divided or the entrance widened, but only once the inflammation is under control with ointment; otherwise everything fuses again.

In girls the treatment is the same and just as necessary. Puberty eases things for some of them, but growing up is no guarantee of cure, so follow-up continues into adult life.

Everyday care

Care does not replace treatment, but without it the ointment works less well and the skin is injured afresh every day.

• Wash once a day with warm water and use an emollient suitable as a soap substitute instead of soap. Gels, foams, perfumed and antiseptic "intimate hygiene" products, douches and wipes are a direct source of irritation. Pat dry afterwards rather than rubbing.
• Put on a greasy protective layer — a plain emollient or petroleum jelly — before passing urine and before swimming in a pool or the sea: it keeps urine and chlorinated water from burning the skin.
• Wear loose cotton underwear, sleep without it, give up thongs and panty liners; wash underwear without fragrance or softener and rinse it well.
• Use a lubricant for sex. Sex is not forbidden, but with fresh fissures it is better to wait until they heal.
• Do not let constipation take hold: hard stools tear the skin at the anus, particularly in children.
• Cut down friction: put cycling and horse riding aside during a flare, and keep nails short so that scratching does not break the skin.
• Stop smoking: it does not drive the inflammation, but it raises the risk of vulval cancer, which is already higher than usual here.

When not to wait

Do not put off an appointment if pain on passing urine or during sex is getting worse despite treatment, or if six to eight weeks of regular ointment have brought no improvement at all: the diagnosis may need reviewing.

Two situations need urgent care. The first is being unable to pass urine at all with a full and painful bladder. The second is a retracted foreskin in a man that will not go back while the head of the penis swells and darkens: this is a constriction that has to be relieved within hours. Both call for emergency assessment and, if needed, an ambulance — in much of Europe on the single number 112.

Online consultation

A remote appointment fits here for a simple reason: people reach a doctor late with these complaints out of embarrassment, and a conversation without an examination removes that first barrier. The doctor will go through the description and, if you are willing, a photograph, say whether it looks like lichen sclerosus, thrush or menopausal dryness, and explain which specialist to see and how urgently.

Remote advice also suits everything that comes up once treatment is under way: how to apply the ointment and how much is needed, why it must not be abandoned when the itch goes, how to move on to maintenance, what to do about stinging when passing urine, what to use instead of soap, which lubricant to choose. The doctor can help make sense of a report you already have and suggest what to ask at the face-to-face visit.

The first diagnosis, a biopsy and any assessment of scarring, however, are possible only in person: the warning signs listed above are examined face to face and without delay.

This material is for information only and does not replace medical advice.