Lichen sclerosus is a dermatological disease of unclear etiology, in which atrophy of various skin areas is observed, mainly in the genital area, less often in other regions of the body. A symptom of this condition is the formation of light, almost white plaques and papules around the genitals, occasionally on the neck and near the armpits, which eventually transform into atrophic foci. Diagnosis is carried out on the basis of the results of a dermatological examination of the patient, in controversial cases, a biopsy of the skin in the affected area can be performed with subsequent histological examination. Etiotropic treatment of the disease has not been developed, general tonic agents, vitamin therapy and medications are prescribed to improve microcirculation. There are indications of a positive effect from the use of antimalarial drugs.
General information
Lichen sclerosus (white spot disease, teardrop–shaped scleroderma, vulva/penis kraurosis) is an atrophic skin disease of uncertain genesis, which mainly affects the genital area, but can also be located on other areas of the skin. This condition was first described back in 1889 by the French dermatologist Francois Allopo, who mistook the disease for a type of ordinary lichen planus. Only further research allowed us to determine that the pathology described by Allopo is divided into two types – primary lichen sclerosus and the consequences of red flat lichen (atrophy stage). This dermatological disease is several times more common in women, especially extragenital forms – the latter are almost never observed in men. Observations about the age distribution vary somewhat – according to one data, lichen sclerosus mainly affects older people and extremely rarely occurs in girls during adolescence. According to other researchers, cases of the development of this condition in young women are not uncommon – such differences in the description of many experts are explained by confusion, in which the old forms of lichen planus are taken for the disease of white spots.
Causes
The etiology and pathogenesis of lichen sclerosus are currently insufficiently studied, there are only a few hypotheses about the development of this dermatological disease. The pathogenetic theory enjoys the greatest recognition – both hereditary cases of this condition and their similar development in identical twins are described. However, neither defective genes nor other molecular mechanisms of the occurrence of lichen sclerosus have been studied. Another popular theory points to autoimmune factors in the development of this pathology, since its manifestations are similar to other skin lesions of this type (scleroderma, some forms of psoriasis). But even this version does not explain all the manifestations of lichen sclerosus, in addition, the disease reacts very poorly to treatment with immunosuppressive agents.
There are indications of the role of infectious factors, for example, borreliosis (Lyme disease). After an infection of this type, a number of people have typical manifestations of lichen sclerosus. The influence of other viral or bacterial infections is not excluded, and this theory is also combined with autoimmune by some dermatologists due to a possible cross-reaction to antigens of pathogens and human own tissues. It has been practically proven that hormonal imbalance, endocrine system lesions, and frequent psychoemotional stresses play a predisposing role in the development of lichen sclerosus. The study of this disease, attempts to explain the causes of its occurrence and the use of these data in the treatment of pathology are still ongoing.
Symptoms
Most cases of lichen sclerosus are registered in women aged 35-50 years, sometimes the development of the disease is observed in young girls and even adolescents. Rashes can be localized on the neck, chest, near the armpits, on the pubis, thighs and in the genital area – while the so-called extragenital forms of white spot disease (the presence of rashes on the body in the absence of them on the vulva and nearby areas) account for only 20% of all cases. Most often, lichen sclerosus is manifested by the defeat of the genital area and the vulva itself. The onset of the disease is characterized by the appearance of white papules on the surface of the skin, which quickly transform into light or pink plaques. The surface of the plaques is slightly raised above the healthy skin, flakes off, pronounced itching is noted from the subjective symptoms. With the further development of lichen sclerosus, hypopigmented atrophic skin areas are formed in place of plaques, the surface of which resembles tissue paper.
Men suffer from this pathology several times less often, while they almost always have a lesion of the skin in the genital area (in 90% of cases, the foreskin and the surface of the glans penis are affected). Otherwise, the development of skin manifestations of lichen sclerosus has no features, the appearance of plaques or papules with further skin atrophy and the appearance of white spots is noted. Lesion of the foreskin can lead to the development of scar phimosis. In both men and women, genital forms of lichen sclerosus can be complicated by a secondary infection of bacterial or viral genesis. Also, this form of the disease is dangerous because it is a precancerous condition – according to statistics, in about 3-5% of cases, the disease of white spots degenerates into squamous cell carcinoma of the skin or genitals.
Diagnostics
The detection of lichen sclerosus in dermatology is not particularly difficult due to the pronounced manifestations of the disease – white atrophic skin areas localized around the genitals and on the vulva. On examination, relatively fresh foci of pathology may be detected in the form of light pink papules or plaques. However, the differential diagnosis of lichen sclerosus is somewhat difficult, since scleroderma, old forms of lichen planus, and some varieties of psoriasis have similar manifestations. The main feature of the pathology is the predominant localization of rashes around and on the genitals, therefore, with extragenital forms, diagnostic errors most often occur.
Pathohistological changes in lichen sclerosus are not the same at different stages of the process. At the initial stages, pronounced edema of the dermis, moderate lymphohistiocytic infiltration, and dilation of blood vessels are detected. The epidermis is thickened, there is a violation of the processes of keratinization, horny masses can clog hair follicles, ducts of sweat and sebaceous glands. With the further development of lichen sclerosus, the atrophy of the epidermis and the homogeneity of the collagen fibers of the dermis are determined. In the later stages, in some cases, signs of cell metaplasia and initial manifestations of squamous cell carcinoma may be observed.
Treatment
Due to the ambiguity of the causes of lichen sclerosus, etiotropic treatment of this dermatological disease does not exist today. Various symptomatic therapies are used, as well as drugs whose relative effectiveness has been proven by empirical clinical observations. From external agents, immunosuppressive ointments are prescribed (based on corticosteroids or tacrolimus), but they can reduce the severity of symptoms only at the stage of development of plaques and papules. Similarly, antimalarial agents are also used in the treatment of lichen sclerosus – they are not prescribed for developed skin atrophy. Drugs that improve local blood supply and microcirculation processes (for example, based on vitamins) can also significantly reduce the severity of symptoms. Some experts point to the need to treat the underlying disease, which could be provoking for lichen sclerosus – endocrine disorders, diabetes mellitus, etc.
Sometimes surgical techniques are used in the treatment of white spot disease. Operations can be performed both for aesthetic reasons (elimination of excessively large areas of atrophy and further plastic surgery) and for medical reasons. In severe cases of lichen sclerosus, adhesions occur, leading to fusion of the labia minora, narrowing of the urethra, phimosis. All these complications can be eliminated only surgically – plastic surgery of the vulva and urethra, circumcision (circumcision) in men. In addition, surgical treatment is indicated for the transformation of lichen sclerosus into squamous cell skin cancer.
Prognosis and prevention
Most dermatologists tend to attribute lichen sclerosus to diseases with a relatively unfavorable prognosis. This is due to the uncertain genesis of the pathology, as a result of which, despite all therapeutic measures, atrophic skin areas of various sizes remain in patients. There is always a risk of complications such as the development of adhesions, narrowing of the urethra, secondary infection, malignant degeneration. Patients with lichen sclerosus should visit a dermatologist or oncologist every 2-3 months in order to detect the above-mentioned dangerous complications as early as possible.