Lichen sclerosus (LS)
The LS is a rare, chronic inflammatory, progressive disease of connective tissue of the skin and localized mainly anogenital. Extragenital shows the LS in up to 15% of cases. The etiology of the disease is unknown.
For an autoimmune cause of the increased incidence talk with other autoimmune diseases such as diabetes mellitus, thyroiditis, morphea, vitiligo, alopecia areata, pernicious anemia, ulcerative colitis.
It there is evidence of a genetic predisposition that may be in specific HLA-constellations (hereditary system of tissue antigens) noticeable. Other studies show a significant association with HLA class II antigen DQ7 (hautspez. antigen).
Also a familiar occurrence suggests a genetic component (mother, child - sisters - twins).
The LS affects girls before puberty and women increases after menopause. The disease rate among boys and men (30-50 years) are also characterized by two peaks. Changes in hormonal status, such as during puberty, may be associated with a change in disease activity during pregnancy and at menopause. The ratio of estrogen - androgens, but cortisol also appear to play a role. In some of the patients have a reduction of dihydrotestosterone. Stress and trauma can lead to a deterioration of the action.
be discussed:
- Increased activity of a protease, elastase type
- Diminished 5-alpha reductase activity, the enzyme that controls the formation of dihydrotestosterone to testosterone
- pathogenetic significance of androgen and retinoid receptors
- Controversial discussions are there to infections, particularly for Borrelia infection
- IgG autoantibodies to ECM1, a skin-specific protein
- Immune dysregulation
Frequency
The LS has a worldwide distribution, but in the Caucasian population more often described and justified. It affects children, women and men. Clinical and epidemiological studies have suggested that reports on the disease inadequate. Patients do not seek medical attention right away or a clinic, and gynecologists, dermatologists and urologists may not always associate the symptoms. Until the diagnosis of the disease may take 3-6 years. In the literature, the prevalence in dermatological practice with 1: 300-1: given 1000th
Symptoms
- Clinically, the LS shows a pale, porcelain-white macules or papules, later confluent whitish to skin areas, proposed either Atrophie or hyperkeratosis, marked. There are also Purpura, fissures and erosions caused by scratching.
- Of pruritus can be excessive in women. Due to the chronic course there is atrophic, sclerotic (keratinizing) plaques, to the fusion of the labia minora, the shrinkage and stenosis of the urethra and the vaginal orifice. Affected may be the complete external genitals of the woman: labia minora, labia majora, clitoris and anal and perianal areas (eight shape distribution to the vulva and the anus). Symptomatically are pruritus, soreness, dysuria, dyspareunia and painful defecation.
- For men, foreskin and glans are affected, often the perianal region. It is difficult to retract the foreskin (phimosis), the slowing of the urinary flow and inflammation of the prepuce and the glans (balanitis) and thus to painful erections.
- For the location of the LS and mechanical factors seem to play a role. A Koebner phenomenon may lead to new lesions in LS.
- The LS takes a relapsing-remitting course, individual episodes can be interrupted by long periods of rest.
Diagnosis
- The diagnosis is confirmed by a biopsy. Autoantibodies against thyroid and gastric parietal cells should be determined and autoimmune diseases are detected.
- Histopathologically, a tight atrophy of the epidermis showing sinuous with follicular hyperkeratosis, destruction of the elastic fibers, infiltration of the edematous hyalinized collagen fibers, ribbon-like perivascular lymphocytic infiltrate. Massive edema can lead to hemorrhagic blisters.
- Frequently occur as concomitant diseases mycoses and bacterial infections that must be addressed. With long-persistent existence of the LS with hyperplastic districts there is a slightly increased risk of squamous cell carcinoma (SCC). Ongoing monitoring is required.
Therapy
- Corticosteroids (Kl.4) in the form of creams and depot injections in the acute stage
- Corticosteroids (Kl.3) and weaker in connection
- Tacrolimus (Protopic Ointment) - a calcineurin inhibitor - has been proven in a multicenter study in Germany and Austria, as well as in small series of observations to be effective.
- Retinoids, antibiotics and calcipotriol have been tried and have shown sporadic effect.
- thymusextrakthaltige creams cause a nonspecific immune stimulation
- Fat creams in asymptomatic periods
- Male circumcision is recommended.
- About balneo and photodynamic therapy is reported as relating to the application of the pulsed dye laser or CO2 laser and cryosurgery
- Testosteronhaltige topical should not be utilized because no proven efficacy and significant side effects in patients due.
- All topical, systemic, or surgical measures are symptomatic.
- Relaxation techniques (autogenic training, yoga) or concomitant psychotherapy can be helpful in this very stressful disease, as well as a good doctor-patient relationship in order to alleviate the discomfort.