Morphea
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Lichen sclerosus (Latin: lichen sclerosus) is a chronic inflammatory skin disease of not fully understood etiology, classified among inflammatory dermatoses of immunologic origin. The condition most often affects the skin of the anogenital area, although it may also occur in other locations. A characteristic feature of the disease is the formation of porcelain-white, atrophic skin lesions, which may be accompanied by itching, burning and discomfort. Lichen sclerosus occurs in women, men and children, but it is diagnosed much more frequently in postmenopausal women. Untreated disease may lead to tissue scarring, functional impairment of the genital organs and an increased risk of developing squamous cell carcinoma of the skin within the affected tissue.
Lichen sclerosus – types
Lichen sclerosus can take various clinical forms depending on the location of the lesions and their course. The basic division includes anogenital and extragenital forms, which differ in symptoms and potential complications.
The anogenital form of lichen sclerosus is by far the most common form of the disease. In women it most often involves:
- the vulva,
- the vaginal vestibule,
- the clitoris,
- the perianal area.
In men the lesions are mainly located on the:
- glans penis,
- foreskin (prepuce),
- coronal sulcus.
In this form of the disease there may be progressive atrophy and scarring of the skin, which in extreme cases leads to narrowing of the vaginal introitus in women or phimosis in men.
The extragenital form of lichen sclerosus occurs less frequently and usually affects the skin of the:
- trunk,
- neck,
- shoulders,
- thighs,
- inframammary regions.
Lesions in these locations appear as whitish patches or papules with a tendency to coalesce into larger plaques. The extragenital form usually does not lead to significant functional impairment, but may cause itching and discomfort.
In dermatology it is also recognised as part of the spectrum of autoimmune diseases, because lichen sclerosus often coexists with conditions such as:
- autoimmune thyroid diseases,
- acquired vitiligo,
- type 1 diabetes,
- alopecia areata.
Vulvar lichen sclerosus
Vulvar lichen sclerosus is the most common form of the disease and mainly affects women in the postmenopausal period, although it can also occur in younger women and in girls before puberty. Lesions most often involve the labia, clitoris, vestibule of the vagina and the perianal area, creating a characteristic pattern of changes in the shape of the so-called "figure-eight" involving the vulva and anus.
In the early stage of the disease, porcelain-white patches and small papules are observed, which over time may coalesce into larger atrophic foci. The skin becomes thin, taut and prone to micro-injuries. The most commonly reported symptoms include:
- intense itching of the genital area, especially at night,
- burning and a sensation of dryness,
- pain during intercourse (dyspareunia),
- cracks and erosions of the skin,
- bleeding from minor skin injuries.
In advanced stages of the disease there may be progressive atrophy of the anatomical structures of the vulva, such as the labia minora or the clitoral hood. Chronic inflammation also leads to tissue fibrosis and narrowing of the vaginal introitus.
Regular monitoring of lesions is particularly important, because long-standing vulvar lichen sclerosus is associated with an increased risk of developing vulvar squamous cell carcinoma, which according to epidemiological data is about 3–5% of cases.
Early diagnosis and appropriate treatment can limit disease progression and reduce the risk of structural and neoplastic complications.
Lichen sclerosus in men
Lichen sclerosus in men occurs less frequently than in women, but its course often leads to significant complications in the genital area. The disease most commonly affects the glans penis and the foreskin, where it causes the formation of whitish, atrophic skin lesions.
In the medical literature these changes are also referred to as balanitis xerotica obliterans, that is, chronic inflammation of the glans and foreskin leading to tissue fibrosis.
Typical symptoms of lichen sclerosus in men include:
- white, porcelain-like patches on the glans penis,
- thinning and tightening of the foreskin,
- burning or itching,
- pain during intercourse,
- difficulty retracting the glans.
Progressive inflammatory changes can lead to foreskin narrowing (phimosis), which impairs proper hygiene and causes discomfort during erection. In some cases there is also narrowing of the urethral meatus, which can cause difficulty urinating.
The basis of treatment in the early stages of the disease is topical therapy using potent glucocorticosteroids, which reduce inflammation and inhibit tissue fibrosis. In cases of established phimosis or persistent scarring changes, surgical treatment in the form of circumcision is often necessary to remove the diseased tissue.
As with vulvar lichen sclerosus, long-standing skin changes of the penis require regular dermatologic or urologic follow-up, because there is a small but significant risk of malignant transformation toward squamous cell carcinoma of the penis.
Lichen sclerosus – causes
The exact etiology of lichen sclerosus has not yet been definitively explained. It is currently believed that the disease develops as a result of a complex interaction of immunological, genetic, hormonal, and environmental factors. Numerous studies have demonstrated a significant role of autoimmune mechanisms, in which the immune system begins to react against the skin's own structures.
One of the best-known mechanisms is the presence of autoantibodies against ECM-1 protein (extracellular matrix protein-1). This protein plays an important role in maintaining the normal structure of the dermis and the function of the extracellular matrix. Disruption of its function leads to degeneration of collagen and elastin fibers and the development of characteristic atrophic changes.
Factors that increase the risk of disease development include:
- genetic predisposition,
- coexistence of autoimmune diseases (e.g., Hashimoto's disease, vitiligo, type 1 diabetes),
- hormonal disorders, particularly estrogen deficiency after menopause,
- chronic microtrauma to the skin and mechanical irritation,
- impaired epidermal barrier function.
In men, lichen sclerosus often develops in the area of the foreskin and glans penis, which can lead to progressive fibrosis of the tissues and the formation of phimosis. In women, the disease most commonly affects the vulva and the perianal area, where chronic inflammation can cause gradual remodeling of anatomical structures.
Lichen sclerosus – symptoms
Symptoms of lichen sclerosus can be varied and depend on the stage of the disease and the location of the lesions. In the initial phase the disease often develops slowly and can be mistaken for other inflammatory dermatoses or skin infections.
Among the most common symptoms of lichen sclerosus are:
- porcelain-white patches or papules on the skin,
- thinning and atrophy of the epidermis,
- smoothing of the skin's natural texture,
- intense itching,
- burning or pain,
- a tendency to fissures and microtrauma of the skin.
In the genital area the lesions can lead to more serious disturbances. In women the following are often observed:
- pain during intercourse (dyspareunia),
- narrowing of the vaginal introitus,
- atrophy or loss of the labia minora,
- urination disturbances.
In men the disease can cause:
- narrowing of the foreskin (phimosis),
- pain during erection,
- difficulty retracting the glans penis.
In advanced cases there is scarring of the skin and tissue remodeling, which can lead to permanent anatomical deformities. Within chronic lesions there is also an increased risk of developing cutaneous squamous cell carcinoma, so patients require regular dermatological follow-up.
Lichen sclerosus – is it contagious?
Lichen sclerosus is not an infectious disease and is not transmitted between people through direct contact, sexual intercourse, or contact with everyday objects.
It is currently believed that the disease develops as a result of the complex interaction of several factors, among which the most commonly mentioned are:
- autoimmune mechanisms,
- genetic predispositions,
- hormonal disorders,
- chronic microtrauma of the skin,
- environmental factors.
Immunological studies have demonstrated the presence of autoantibodies directed against extracellular matrix proteins of the skin, especially ECM-1 (extracellular matrix protein-1), which is responsible for the proper structure of the dermis.
The disease occurs more frequently in people with other autoimmune diseases, which further supports the involvement of immune system disorders in the pathogenesis of lichen sclerosus.
Although the disease is not infectious, its symptoms – particularly in the genital area – can be mistaken for sexually transmitted diseases, so proper dermatological or gynecological diagnosis is crucial for correct identification.
Lichen sclerosus – diagnosis
The diagnosis of lichen sclerosus is primarily based on a clinical examination performed by a dermatologist or gynecologist. The characteristic appearance of skin lesions – porcelain-white atrophic patches, smoothing of the skin, and the presence of fissures or erosions – often allows a preliminary diagnosis to be made during the physical examination.
In cases of diagnostic uncertainty or an atypical clinical picture, a skin biopsy is performed, followed by histopathological examination of the sampled tissue. This examination makes it possible to confirm the diagnosis and exclude other diseases with a similar course.
On microscopy, typical features of lichen sclerosus include:
- epidermal atrophy,
- homogenization of collagen fibers in the upper dermis,
- presence of a lymphocytic inflammatory infiltrate,
- disruption of the extracellular matrix structure.
In the differential diagnosis, other diseases of the genital skin should be considered, such as:
- lichen planus,
- localized scleroderma (morphea),
- chronic vulvitis,
- sexually transmitted infections,
- premalignant and malignant skin lesions.
Regular dermatological follow-up is particularly important for chronic lesions in the genital area, because long-standing lichen sclerosus may increase the risk of developing cutaneous squamous cell carcinoma.
Lichen sclerosus – treatment
Treatment of lichen sclerosus is long-term and aimed at controlling inflammation, alleviating symptoms, and preventing skin scarring. Complete cure of the disease is not always possible, but appropriate therapy can effectively control its course.
The foundation of treatment is high-potency topical corticosteroids, which:
- reduce inflammation,
- alleviate itching,
- inhibit scarring processes.
In some cases the following are also used:
- calcineurin inhibitors (e.g., tacrolimus),
- hormonal treatment,
- anti-inflammatory therapy.
In advanced cases, especially in men with phimosis, surgical treatment may be necessary, most commonly circumcision.
In aesthetic dermatology and regenerative therapy, methods that support improvement of skin quality are increasingly used, such as:
- fractional laser therapy, which stimulates collagen remodeling and skin regeneration,
- platelet-rich plasma (PRP) therapies, which support tissue repair processes,
- regenerative procedures for the skin of the intimate area used in the treatment of atrophic changes.
In clinical practice, daily skin care is also very important and includes:
- use of emollient preparations,
- avoiding irritating factors,
- wearing breathable underwear made of natural materials.
Patients with lichen sclerosus should remain under regular dermatological or gynecological follow-up, because chronic skin changes — particularly in the genital area — require monitoring for potential malignant complications.
