Lichen sclerosus (overview) L90.4

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 10.05.2021

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Synonym(s)

Balanitis xerotica obliterans; lichen albus; Lichen plan atrophique; lichen sclerosus et atrophicans; lichen sclerosus et atrophicus; vulgar rufous bush; White spot disease

History
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Hallopeau, 1887; Darier, 1892

Definition
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Lichen sclerosus (lichen from Greek leichen = lichen, sclerosus from Greek skleros = hard, dry, brittle) is an acquired, non-infectious, mucocutaneous, chronic inflammatory (autoimmunological) disease with a phasic course.

Classification
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Clinical-topographical the Lichen sclerosus can be classified:

The different clinical processes and prognoses allow a classification into:

  • Prepubertal lichen sclerosus
  • Postpubertal lichen sclerosus

Occurrence/Epidemiology
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Different information depending on the collective.

Prevalences of 0.1% are reported for young girls and up to 3% for adult women.

Cross-sectional analyses of larger non-preselected male US collectives showed prevalences of 1.4 - 2.1/100,000.

Etiopathogenesis
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It is (analogous to Lichen planus) a T-cell mediated disease (Terlou A et al. 2012)

Discussed is mainly an autoimmunological genesis; furthermore hormonal factors, dysregulation of the sex hormones, infectious genesis(Borrelia burgdorferi; EBV-infection ?), formation of autoantibodies against ECM1 (extracellular matrix protein-1 - detected in 60-70% of cases; the occurrence of these autoantibodies seems to be more of a secondary phenomenon), genetic disposition (rare familial occurrence, association with HLA-B40, HLA-B44). Specific autoantibodies are missing.

Detection of associated autoimmune diseases such as (figures from a collective of 190 patients):

  • autoimmunological thyroid diseases (16%)
  • Vitiligo (10%)
  • Alopecia areata (3%)
  • pernicious anaemia (3%)
  • primary biliary cirrhosis

An association with systemic lupus erythematosus was mentioned in several case reports.

Immunohistological similarities with circumscribed scleroderma suggest a possible pathogenetic relationship.

Manifestation
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The genital lichen sclerosus shows 2 age peaks:

  • Prepubertal: Start in children mostly between 5-11 years of age.
  • Postpubertal: Adult women and men are affected in middle age (30-50 years), women preferably in menopause; possibly the first symptoms appear already well before menopause (Kirtschig G 2018).

Women are affected 4-10 times more frequently than men.

The extragenital lichen sclerosus mainly affects adults. About 6-11% of the entire lichen sclerosus collective is affected extragenitally.

Localization
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  • Genito-anal area: vulva, prepuce, glans penis, anal area (often genito-anal pattern in an 8-formation, this is mainly detectable in prepubertal lichen sclerosus).
  • Extragenital (about 10% of cases): Mainly lateral neck, collarbone area, pre-sternal, submammary and mammary, flexor sides of the forearms, shoulders, rarely oral mucosa.

Clinical features
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I. Genital/genito-anal lichen sclerosus:

  • Prepubertal: Anogenital involvement is typical, with sharply demarcated, whitish plaques with a mirror-like surface, often involving the vulva and anus in an 8-formation. The initial foci of lichen sclerosus usually cause no discomfort. Mild pruritus is occasionally reported,
  • In adults, excruciating pruritus, sometimes combined with pain (rhagaden formation), is most prominent, especially during sexual intercourse.
  • In the later course, whitish atrophic, porcelain-like plaques with a marked increase in consistency are found with a tendency to shrink. Frequently bleeding rhagades with a tendency to superinfection. Furthermore, also extensive, long-term persistent lesional hemorrhages, which can dominate the clinical picture and be diagnostically irritating (sclerosis is covered by older or fresh "blood lakes"). In the late stage, different degrees of vulvar atrophy with atrophy of the small labia (small labia may disappear completely) and subsequently of the large labia, in the further late stage findings of Kraurosis vulvae.
  • Men (see below Lichen sclerosus of the penis): Balanopreputial sclerosis with erosions and rhagades. Also planar haemorrhages, and varying severity of phimosis. Synechiae; in the late stage picture of the so-called "Balanitis xerotica obliterans". The concordant development of squamous cell carcinoma is about 5% in larger collectives.

II. extragenital lichen sclerosus:

  1. Confetti type: There are usually disseminated or grouped, white or gray-brown, about 0.2-0.5 cm large spots and plaques. Occur mainly on the axillae and flexor sides of the wrists, on the trunk and neck. In this type there is often no mucosal involvement.
  2. Map type: In this type, the initially small foci of lichen sclerosus are confluent into larger,map-like areas of patches and plaques with atrophic parchment-like surfaces (often affecting the female mammae). Red borders around the foci are typical (sign of underlying inflammation). Band-like hyperpigmentation is also found in the margins of lichen sclerosus. Less common is lesional blistering. Almost pathognomonic are lesional hemorrhages, which can be found in different shades of red (light red, deep red or reddish brown) depending on the duration of the condition. Involvement of the oral mucosa is very rare.
  3. Special forms: Bullous or hemorrhagic lichen sclerosus. The hemorrhagic component results from secondary (often trauma-induced) hemorrhage into the area of lichen sclerosus. In this "bradytrophic" zone, blood is broken down only very slowly, so that the hemorrhage is detectable for several weeks.

Histology
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  • Early stages: epithelial atrophy, orthohyperkeratosis, hypergranulosis. Subepithelial band-shaped epidermotropic round cell infiltrate. This infiltrate can assume considerable density, so that a differential diagnosis must be made to distinguish it from epidermotropic cutaneous T-cell lymphomas.
  • Intermediate stage: Elastic-fibre-free oedema zone with widely gaping blood and lymph vessels, which slides like a zipper between the epidermis and the ligamentous inflammatory zone. Bullous transformation is possible.
  • Late stage: Increasing sclerosing tendency and regression of the cellular inflammatory phenomena.
  • Immunohistology: Dermal infiltrate mainly from T-lymphocytes, with high proportion of B-lymphocytes (20-30%).

  • Note: Sufficiently deep punch biopsy (do not squeeze) from lesioned, non-steroidal pretreated skin/mucosa.

Diagnosis
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Typical unmistakable clinical picture with chronic course, histological substrate.

Differential diagnosis
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Clinical:

  • Circumscribed scleroderma: no genital or anal involvement. Plaque-like on trunk and extremities. Surface not rippled like parchment.
  • Lichen planus: uniform livid colour, usually clearly palpable elevations (papules or plaques); itching.
  • Lichen simplex chronicus Vidal: no mucosal involvement, marked pruritus, extragenital cutaneous lesions.
  • Psoriasis anogenitalis: no mucosal involvement; sharp border to healthy skin; frequent extragenital involvement.
  • Vitiligo: sharp, often bizarre demarcation to healthy skin; no itching, no induration of skin, extragenital infestation!
  • Candida infections: macerated, blurred areas, inguinal and anal regions usually also affected. Obesity, immobility, immune disorders, mycological evidence of the yeasts!

Histology:

  • Lichen planus: hyperorthokeratosis, marked hypergranulosis, no formation of a so-called barren zone.
  • Acrodermatitis chronica atrophicans: rather sparse diffuse lymphocytic infiltrate; relative proportion of plasma cells. No subepithelial band-like arrangement.
  • Mycosis fungoides: dense lymphocytic infiltrate, cell and nuclear polymorphism, eosinophilic granulocytes. No barren zone.

Complication(s)
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Phimosis with subsequent complications; atrophy of the external genitals, Kraurosis vulvae (see also Lichen sclerosus of the vulva)

Remember! The lichen sclerosus et atrophicus in the genital area leads to the development of carcinoma in 3-6% of late adults. Women are preferentially affected!

General therapy
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  • It is important to determine the therapeutic goal together with the patient. In the foreground is the minimization of the symptoms.
  • The therapeutic option to be chosen depends on the age of the patient, the localization (the vulval LS requires a different therapy than the penile LS) and the severity of the disease (see below lichen sclerosus of the vulva)
  • In case of low infestation (smaller plaques), purely local measures are to be preferred.
  • In the case of more extensive infestation, an invasive procedure (circumcision) is recommended at an early stage in the case of penile lichen sclerosus.

External therapy
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A consequent external treatment is especially necessary in the genital area because of the agonizing itching and the progressive sclerotherapy.

  • Glucocorticoids:
    • Glucocorticoid Externa are the therapeutic agents of first choice (procedure for vulvar lichen sclerosus s. there). An interval-like shock therapy with the use of highly potent glucocorticoid extracts is recommended.
    • Relatively good results can be achieved in genital lesions with severe itching or pain with intralesional glucocorticoid injections, such as triamcinolone acetonide crystal suspension (e.g. Triam 10 Lichtenstein burns less than Volon A) diluted 1:1 with local anesthetic, e.g. Scandicain. The injections can be used in combination with cryosurgery if necessary.
  • Calcineurin inhibitors: Good clinical results with significant improvement of the subjective symptoms and long-term remissions can be achieved under a topical therapy with Tacrolimus (0,1% protopic ointment) or Pimecrolimus (Elidel cream 1%). Cave: often an initial burning after application is indicated. Long-term experiences are still missing at present. In a transitional phase it may be advisable to use the preparations in daily alternation with glucocorticoid extracts.
  • Hormones: In early stages in women, especially in younger women, therapy with preparations containing estrogen and progesterone (e.g. Linoladiol N cream, Cordes Estriol cream, Estriol ointment) over several weeks. Relatively good successes can be seen in this area.
  • In men, less than 2-5% testosterone ointment/gel (e.g. Androderm, R249 ) over several months often leads to good results. This therapy is no longer acceptable for women because of the risk of virilization! Cave! resorption! In girls this therapy is absolutely contraindicated!
  • Nursing Externa: In case of infestation of the large or small labia, careful local care (dexpanthenol ointment R065, oestrogen-containing hydrophilic creams) and meticulous intimate hygiene. Use a bidet after going to the toilet. If this is not possible, first dab with moist toilet paper, then dab the lesions with oil-soaked tissues. Before sports or longer marches, apply a hydrophilic, possibly also hydrophobic ointment, e.g. Vaseline. alb. or as a ready-to-use preparation Deumavan® ointment.
  • In addition, regular sitz baths with astringent agents (e.g. Tannosynt) are recommended for genital and perianal LS.
  • Treatment of a genital fluoride, especially candidosis.
  • Alternative: Comparable results to calcineurin inhibitors have been reported in an alternative approach with a thymus-peptide active ingredient (Sclero Discret Thymusskin Intimate Care Cream). These results require further confirmation.

Radiation therapy
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UVA1 phototherapy has been reported to be successful in small pilot studies for both genital and extragenital lichen sclerosus.

Internal therapy
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  • in case of severe infestation, treatment with methotrexate (15mg/week over a period of 6-12 months; personal experience).
  • Trial with acitretin (neotigason) 0.1-0.2 mg/kg bw/day (personal experiences are rather negative)
  • Alternatively penicillin 10 Mega IU/day for 10 days, 3 treatment cycles at intervals of 4 weeks (therapy successes are not very satisfying!).
  • Alternatively sulfasalazine (e.g. azulfidine) 2-4 g/day.
  • If necessary, therapy with chloroquine (e.g. Resochin) 250 mg/day p.o. (this therapy strategy is also not very successful according to own experience!)

Operative therapie
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Surgical procedures should be used if the clinical symptoms are extensive:

  • Cryosurgery: Good results can be achieved with the open procedure, in the case of circumscribed lesions with closed systems (stamps). Among these, there is a continuous improvement of itching and sclerosis (especially in the genital area). This therapy can also be used in children.
  • Successes with ablative lasers have been reported. The long-term results remain to be seen.
  • Circumcision is recommended in the case of prepuce and/or glans penis infections. Circumcision should be attempted at an early stage, as this therapy approach achieves the best long-term results.
  • In case of lichen sclerosus of the vulva, surgical therapies are indicated if synechia and fusion of the labia have occurred. Surgical correction is also necessary in cases of constriction of the orifice externum of the urethra.

Progression/forecast
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Quoad vitam good, quoad sanationem at first manifestation in adulthood doubtful. Chronic, possibly decades-long, intermittent course.

Irreversible atrophy of the genitals with phimosis and atrophy of the glans penis in men and so-called Kraurosis vulvae in women.

In the case of infantile lichen sclerosus, the chance of healing by puberty is > 80% (own observations!). However, in a small part of cases the changes persist (mostly in a lighter form) even in adulthood.

Note(s)
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There is a clear association between genital lichen sclerosus and circumscribed scleroderma.

Patients with genital lichen sclerosus often suffer from the "no man's land phenomenon". They are on the borderline between dermatology and gynaecology and do not feel well cared for.

Literature
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  1. Böhm M et al (2003) Successful treatment of anogenital Lichen sclerosus with topical Tacrolimus. Arch Dermatol 139: 922-924
  2. Cooper AM et al (2008) The association of lichen sclerosus and erosive lichen planus of the vulva with autoimmune disease. Arch Dermatol 144: 1432-1435
  3. Eisendle K et al (2008) Possible role of Borrelia burgdorferi sensu lato infection in lichen sclerosus. Arch Dermatol 144: 591-598
  4. Haefner HK et al (2014) The impact of vulvar lichen sclerosus on sexual dysfunction. J Womens Health (Larchmt) 23:765-770

  5. Hallopeau FH (1887) Du lichen plan, et particulièrement de sa forme atrophique. Union médicale (Paris) 43: 729-733
  6. Kirchig G (2018) Lichen sclerosus. dermatologist 69: 127-133
  7. Kreuter A et al (2013) Association of autoimmune diseases with lichen sclerosus in 532 male and female patients. Acta Derm Venereol 93:238-241
  8. Lacarrubba F et al (2015) Extragenital lichen sclerosus: clinical, dermoscopic, confocal microscopy and histologiccorrelations
    . J Am Acad Dermatol 72 (1 Suppl): S50-52

  9. Lee H et al (2014) Segmental vitiligo and extragenital lichen sclerosus et atrophicus simultaneously occurring on the opposite sides of the abdomen. Ann Dermatol 26:764-765

  10. Liu Y et al (2013) Oral lichen sclerosus et atrophicus - literature review and two clinical cases. Chin J Dent Res 16:157-160

  11. Lutz V et al (2011) High frequency of general Lichen sclerosus in a prospective series of 76 patients with morphea. Arch Dermatol 2011: 305
  12. Paolino G et al (2013) Lichen sclerosus and the risk of malignant progression: a case series of 159 patients. G Ital Dermatol Venereol 148:673-678
  13. Patel B et al(2015) Extra genital lichen sclerosus et atrophicus with cutaneous distribution and morphology simulating lichen planus. Indian J Dermatol 60:105

  14. Philippou P et al (2013) Genital lichen sclerosus/balanitis xerotica obliterans in men with penile carcinoma: a critical analysis. BJU Int 111:970-976

  15. Pope E et al (2013) Diagnosis and management of morphea and lichen sclerosus and atrophicus in children. Pediatrics Clin North Am 61:309-319

  16. Schürch HG (2018) Lichen sclerosus-underdiagnosed and undertreated. skin-near dermatology 34: 50-56

  17. Terlou A et al (2012) An autoimmune phenotype in vulvar lichen sclerosus and lichen planus: a Th1response
    and high levels of microRNA-155 J Invest Dermatol 132:658-666 .

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 10.05.2021