Lichen sclerosus et atrophicus: causes, symptoms, diagnosis and treatment of this inflammatory dermatosis

By Cristiano Antonino On May 29, 2023

Lichen sclerosus et atrophicus is an inflammatory dermatosis that predominantly affects the ano-genital area

Most common in females in the pre-pubertal and post-menopausal years, it also affects men and can occur at any age.

Diagnosis of lichen sclerosus et atrophicus is not easy

Often the disease is not identified, or is diagnosed long after its first manifestation, because the papules that appear on the skin are underestimated or confused with other diseases.

However, early diagnosis is crucial: lichen is a chronic inflammatory disease that is potentially pre-cancerous, from which it cannot be cured but which can be kept at bay.

What it is and what causes it

Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory disease that can affect both sexes and occurs on the mucous membranes and skin.

It can affect the mucous membranes of the mouth, tongue, trunk, arms and legs but – much more often – the anal and genital area.

Listed as a Rare Disease since 2001, if neglected it can cause even significant sexual dysfunction.

To date, its causes are unknown.

A multifactorial inflammatory disease with a probable autoimmune etiopathogenesis, it was long thought to be a sexually transmitted disease.

However, the discovery of symptomatology among children has ruled out this hypothesis.

Lichen sclerosus et atrophicus affects women and men who are predisposed to an attack by the immune system on certain tissues

Moreover, it is more prevalent in patients suffering from:

dyslipidaemia (blood lipid abnormality)
chronic infections
vitiligo
diabetes mellitus
lupus erythematosus
pernicious anaemia (anaemic form caused by vitamin B12 deficiency)
spinocellular carcinoma of the penis

Symptoms

The first symptom of lichen sclerosus et atrophicus is the presence – around the genitals and anus – of bruises and blisters, often accompanied by severe itching.

Over time, the skin thins, loses its natural colouring and cracks (some patients present with skin thickening instead).

The symptoms of lichen are in reality different depending on the sex of the patient and the age at which they develop it.

In women

The woman suffering from lichen scleroatrophicus experiences itching, burning and pain during sexual intercourse.

In the most severe stages of the disease, the labia minora may atrophy until they fuse with the labia majora ‘hooding’ the clitoris.

Other typical symptoms in women are:

vaginal and perianal dryness
irritation
appearance of whitish spots
fissures
deformation of the labia minora

It is essential to undergo periodic check-ups, especially in the presence of skin lacerations or thickening.

In a small percentage of cases, lichen sclerotrophicus may pave the way for neoplasms such as vulvar cancer.

In men

Men are more likely to develop the disease in old age.

In their case, we speak of lichen sclerosus balano prepuziale.

The first phase consists of the appearance of itching, burning and small tears on the foreskin; phimosis (shrinkage of the foreskin) occurs and the man has difficulty in sexual intercourse.

The second phase sees the formation of shiny white or reddish patches on the glans, with the foreskin joining it.

Then the external urethral meatus (which loses its colouring and narrows, obstructing the flow of urine), and the urethra (which also narrows) are involved.

Approximately 8% of men with lichen develop penile cancer.

Even for male patients, early diagnosis and appropriate treatment become crucial.

There is often a tendency to self-diagnose, not to talk about genital and sexual disorders out of embarrassment, thus paving the way for very serious complications.

Only a timely consultation with the andrologist specialist can prevent the disease from progressing.

In children

The typical manifestation of sclerosus et atrophicus lichen in childhood is itching and burning in the vulvar and perineal area (in females) and irritation of the glans and foreskin (in males).

The discharge of white spots is also an alarm bell.

In children, spots emerge on the foreskin and urethra.

Over time, tight phimosis of the foreskin or stenosis of the urethra (in more severe cases) may occur.

In girls, the typical skin manifestation is represented by small plaques in the area of the vulva and anus that fuse together to form a sort of hourglass shape.

The diagnosis of lichen sclerosus et atrophicus is based on the observation of skin symptoms and signs

Symptoms consist mainly of itching, dryness of the external genitalia and pain during intercourse.

Among the signs, genital changes may include thinning of the skin, whitish patches, shrinkage of the labia minora, conglutination (the labia minora fuse with the labia majora to the point of hiding the clitoral foreskin), and extension of the spots in the perianal area.

These are all conditions that can be assessed by a gynaecological specialist.

Should a man experience the symptoms, a timely andrological examination is essential.

Treatment for sclerosus et atrophicus lichen

Timely diagnosis and appropriate therapy are essential to avoid degeneration of lichen sclerotrophicus and the onset of complications, as well as to provide relief for the patient.

The lichen sufferer experiences a feeling of discomfort and embarrassment due to itching and the appearance of his or her genitals.

In addition, he has a very difficult sex life.

Therapy is based, in most cases, on the administration of drugs:

corticosteroids for topical use, which are effective but not recommended for prolonged use due to their adverse effects (they are generally used for 15-20 days, so as to reduce the autoimmune component and eliminate the more annoying symptoms such as night-time itching)
vitamin E-based ointments, which have an elasticising and anti-inflammatory action;
systemic corticosteroids, reserved for more severe cases.

Sometimes immunosuppressants and retinoids are also prescribed but, even in this case, the side effects are not minor.

For women, there is a tendency not to perform surgery, as it is a relapsing disease.

And the lesions, once removed, may reappear.

In contrast, surgery is preferred for men, who, depending on the area affected, may undergo circumcision, surgery to repair stenosis of the meatus or urethra, or removal of dysplastic lesions on the glans with subsequent reconstruction of the penis.

Innovative therapies for sclerosus et atrophicus lichen

Finally, there are some ‘alternative’ therapies.

In some countries, silk fibroin is used, a silk deprived of sericin (and thus of its potentially allergic component). Treated with a permanent antimicrobial based on quaternary ammonium, which binds to the silk and is not transferred to the skin, the resulting fabric has an emollient action on dry genital skin, prevents the colonisation of fungi and bacteria, and improves dryness, itching and burning. In some European countries, it is classified as a class A medical device and used to produce ‘therapeutic’ underwear.
Regenerative therapy with stem cells and platelet growth factors (PRP) repairs the scarring and ulcerative outcomes of lichen sclerotrophicus. The procedure is performed on an outpatient basis and lasts approximately one hour. Small amounts of blood (for PRP) and adipose tissue (for stem cells) are taken from the patient, then injected into the mucous membranes and areas of the skin affected by the disease. Results are visible in about two months, but the procedure may have to be repeated several times.

However, a cure-all therapy for lichen scleroatrophicus does not exist to date.

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