Lichen planus mucosae L43.8

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 18.12.2020

Dieser Artikel auf Deutsch

Synonym(s)

Cobweb leukoplakia; Lichen ruber mucosae; Lichen ruber of the mucous membrane; mucosal lichen planus; Oral lichenoid reaction; Oral lichen planus; Oral lichen ruber planus; planus lichen oral; Spider web leukoplakia; Vulvovaginal gingival syndrome; vulvovagino-gingival syndrome

Definition
This section has been translated automatically.

Polyaetiological, multiform, spotty or plaque-like (also reticulated stripy) whitish keratotic or, more rarely, extensive erosive or vesicular/bullous lichen planus of the oral and/or genital mucosa which is limited to the mucosal area or occurs within the scope of an integumentary lichen planus.

Etiopathogenesis
This section has been translated automatically.

Unknown, see below. Lichen planus.

A pathogenetic role of hepatitis C infections is discussed.

An autoimmunological genesis is to be assumed. In about 30% of patients with a "vulvovagino-gingival syndrome" autoimmunological "concomitant diseases" such as diabetes mellitus, Hashimoto's thyroiditis (15%), vitiligo (5%), alopecia areata (4%), celiac disease, pernicious anemia, Sjögren's syndrome, idiopathic thrombocytopenic purpura are found.

It has been discussed, and in individual cases proven, that oral lichen planus is associated with allergic reactions to amalgam or other materials used in dentistry ( e.g. eugenol).

Manifestation
This section has been translated automatically.

w:m = 2:1

The mean age of onset of the disease is between 40-60 years (11-94 years). In a larger study the median age was 59.2 years.

Both the monotopic oral and vulvovaginal LP tend to be chronic with continuous or discontinuous recurrent clinical courses over 3-10 years.

For the vulvovagino-gingival syndrome a mean duration of disease of > 10 years (3-31 years) is reported.

Smoking does not appear to play a role in either conversation or in triggering oral lichen planus.

Localization
This section has been translated automatically.

  • Oral mucosa (usually on both sides of the cheeks), tongue (especially lateral parts of the tongue), gums.
  • About 75% of the lichen planus patients show a discrete or also distinct, oral and/or vulvo-vaginal involvement.
  • The gingiva is affected in about 10% of patients.

Clinical features
This section has been translated automatically.

S.a. Lichen planus.

Oral mucosa:

  • Type I (white papule or plaque type): Mostly streaky, punctate, annular, reticulated, also flat whitish papules or plaques, which may confluence to large opal plaques especially retroangularly and in the area of the entire dental cusp (Köbner phenomenon). Rarer is a small-papular mucosal type (see Fig.). Underlying erythema (of lichen planus inflammation) is not always found visibly (covering phenomenon of overlying orthokeratotic mucosal keratinization). The changes are usually not painful. The red of the lips can be affected as well but also isolated. Occasionally, brownish hyperpigmentation develops. The tongue may be affected both by extensive opal plaques, which are only better recognizable on lateral view. In parallel, coarsening of the surface relief may be observed (reflecting diffuse lichenoid inflammation). Some patients (about 1%) report taste disturbances.
  • Type II (red or erosive type): the second most common manifestation of lichen planus mucosae (see below: lichen planus erosivus mucosae). The symptoms are painful erythema, erosions or ulcers over a large area (especially after ingestion of acidic food or drink). The leading clinical symptom in the erosive type is "pain", which occurs promptly, especially with acidic food or drink. With 0.5-2.0%, erosive lichen planus has a clear risk of malignant transformation.
  • Type I/Type II in different mixed forms.
  • Lichen planus mucosae is described as an "oral lichenoid reaction", which occurs in the immediate vicinity of amalgam fillings on the buccal buccal mucosa, tongue or gingiva. Some authors postulate an independent entity (see notes below).
  • Gingiva: When the gingiva is affected, chronic desquamative gingivitis appears. The "leukoplakic" aspect is often completely absent. Erythema or very painful erosions may be seen, which increase in size during the acute episode and become symptomatic when acidic foods (e.g. orange juice) are consumed.

Genital mucosa:

  • Less frequently affected than the oral mucosa. The clinical symptoms are analogous to those of the oral mucosa. Usually problems during sexual intercourse or burning discomfort during urination lead to the doctor. A severe, chronic and scarring variant is called"vulvovagino-gingival syndrome".

Histology
This section has been translated automatically.

The histological changes are largely identical to those of the LP of the skin. Here, too, compact orthohyperkeratosis is present, but also focal orthoparahyperkeratosis. The inflammatory infiltrate may be mixed with plasma cells "typical for the location".

Differential diagnosis
This section has been translated automatically.

Leukoplakia; Pemphigus vulgaris;

Therapy
This section has been translated automatically.

Depends on the acuteity and extent of the lesions. Satisfactory results are only achieved with systemic therapy in cases of extensive symptoms.

External therapy
This section has been translated automatically.

Few, small foci: In non-erosive, clinically less troublesome foci, only a blank local therapy with mild, astringent stomatologic agents (e.g. Tormentill Adstringens R255 ) or dexpanthenol solution (e.g. Bepanthen Lsg., R066 ) is necessary. In the case of isolated oligo- or asymptomatic mucous membrane infestation, such a procedure would also be preferable.

Large non-erosive foci: In case of large-area leukoplakic (isolated) mucosal infestation a consequent local therapy with vitamin A acid solution or gel R258 can be performed (apply gel or solution to the mucosa by means of a toothbrush and let it act for a few minutes). If necessary, use alternately (or in combination) with a 0.1% betamethasone mouth gel R031 or prednisolone acetate paste (Dontisolon D oral healing paste).

Inflammatory, erosive foci (patients are considerably impaired in their quality of life by these often chronic, painful oral mucosa changes).

  • As a "first step therapy", local therapy with topical glucocorticoids, e.g. with 0.1% betamethasone oral gel R031, is possible.
  • Good experience has also been made with clobetasol cream (e.g. Dermoxin cream applied on a gauze-wrapped mouth spatula and applied locally).
  • Alternatively aqueous prednisolone or hydrocortisone solutions are recommended (e.g. Rp. Nystatin 100KUI/Lidocaine 0.1/Prednisolone 0.1/ aqua purificata ad 100.0/ S: Mild, well-tolerated prednisolone-containing solution should be applied 1-2 times daily; or a combination preparation of hydrocortisone acetate and tetracaine hydrochloride - Rp. Hydrocortisone acetate 1.0/ propylene glycol 37.3/ tetracaine hydrochloride 2.0/ guai azulene 25% waterl. 0.05/ cremophor RH40 0.4/ peppermint oil 0.3/ panthenol solution 5% 40.0/ distilled water water ad 285,0/S: Mildly well-tolerated solution containing hydrocortisone (use 1-4 times daily). I have proven to spray the lesions several times a day with a momethasone-containing nasal spray (Nasonex®-Supension)
  • Good success was described with a paste containing Ciclosporin A(Ciclosporin A-adhesive paste 2,5) and a 0,03% tacrolimus suspension (level of evidence: B) (alternative: 1% Pimecrolimus cream which is better tolerated in the mucosa area). For this purpose, apply these externals with a soft toothbrush or on a spatula wrapped with gauze to the lesion and let it act as long as possible. Carry out treatment 2-3 times a day.
  • Topical preparations containing vitamin A acid are less suitable in this form (they are too irritating!).

Notice! In case of pronounced clinical symptoms and chronicity, the (symptomatic) local treatment described above is not sufficient!

Internal therapy
This section has been translated automatically.

The first choice therapy is the combination of acitretin and glucocorticoids. Acitretin (e.g. Neotigason) initial 0.5 mg/kg bw/day and prednisolone (e.g. Decortin H) initial 1 mg/kg bw/day p.o. Maintenance dose according to clinic. The therapy must be carried out over weeks to months according to the clinical symptoms.

Alternatively: if not sufficient, a combination therapy with azathioprine and systemic glucocorticoids is necessary: Azathioprine (e.g. Imurek) initial 1.0-1.5 mg/kg bw/day and Prednisolone (e.g. Decortin H) initial 1.0-1.5 mg/kg bw/day. Dose reduction according to clinic.

Alternatively in case of resistance to therapy: monotherapy with Ciclosporin A (3.0-5.0 mg/kg bw/day p.o.) or mycophenolate mofetil (e.g. CellCept 2.0 g/day p.o.).

Alternatively: Methotrexate 10-15 mg/week p.o. As with other systemic therapeutics a months/years of therapy must be estimated. Positive effects can be expected after 12 weeks of therapy.

Alternative: Application of biologicals (TNF-alpha-blocker). 4-week laboratory controls are necessary.

In the case of vulvo-vagino-gingival syndrome, the initial weight-adapted use of azathioprine (e.g. Imurek) 1.5-2.0 mg/kg bw/day p.o. is recommended , possibly in combination with a glucocorticoid in an initially medium dosage (e.g. prednisolone 0.5 mg/kg bw/day p.o.). Subsequently, clinically adapted lower dosage (5.0-7.5 mg/day p.o.).

Notice! All therapies presented here are "off-label-use" applications. The scientific evidence of superiority of systemic therapy over local therapy has not been demonstrated for oral (or vulvo-vaginal) LP.

Progression/forecast
This section has been translated automatically.

There is an increased risk (1-3%) with oral LP for the development of oral squamous cell carcinoma. The WHO classifies the oral lichen planus as "premalignant condition" (van der Meij EH et al. 2003).

An increased risk of squamous cell carcinoma was also found for the vulval lichen planus. The rate of inguinal metastasis and recurrence is high, as is the disease-related mortality rate.

Naturopathy
This section has been translated automatically.

Camomile extracts: In terms of naturopathy, camomile rinses(Matricariae flos) applied several times a day can be used.

Alternative: Aloe vera: In smaller clinical studies (several times) good effects have been described for both oral and genital lichen planus with locally applicable aloe vera preparations (e.g. aloe vera gel).

Alternative: Local therapy with a mixture of eucalyptus oil, fennel oil, levomenthol, clove oil, peppermint oil, sage oil, star anise oil, thymol, cinnamon oil as offered in Salviathymol® N.

Alternative: Clove oil: Brushing with clove oil(Caryophylli floris aetheroleum)

Diet/life habits
This section has been translated automatically.

Dietary measures: In the case of erosive Lichen planus mucosae, it is recommended to avoid pungent spices, fruit acids (vinegar, citrus fruits, wine and other spirits) of highly salted foods, as they cause a burning of the oral mucosa. Pay attention to sufficient nutrition (vitamins, minerals)!

When cleaning teeth, it is recommended to use a soft toothbrush and a mild toothpaste.

Note(s)
This section has been translated automatically.

The formation of a compact hyperkeratosis is responsible for the leukoplakic aspect of the mucosal LP. The water-retaining horny layer swells, resulting in a milk glass effect. The surface becomes intransparent white.

Some authors delineate oral lichenoid reaction (OLR) as an immunological hypersensitivity reaction clinically and histologically "similar" to lichen planus mucosae, to which a specific trigger can be assigned. These include drugs (NSAIDs, antihypertensives, retroviral drugs), contact allergens (dental materials, amalgam, copper, gold, eugenol), which by definition are found up to 1.0 cm from the OLR.

The syndromal occurrence of oral lichen planus, arterial hypertension, and diabetes mellitus is referred to as"Grinspan syndrome". The occurrence of squamous cell carcinoma in lesional oral mucosa has been reported (Kökten N et al. 2018).... The occurrence of squamous cell carcinoma in lesional oral mucosa has been reported (Kökten N et al. 2018).

Literature
This section has been translated automatically.

  1. Boyce AE et al (2009) Erosive mucosal lichen planus and secondary epiphora responding to systemic cyclosporine A treatment. Australas J Dermatol 50:190-193
  2. Corrocher G et al (2008) Comparative effect of tacrolimus 0.1% ointment and clobetasol 0.05% ointment in patients with oral lichen planus. J Clin Periodontol 35:244-249
  3. Kökten N et al (2018) Grinspan's syndrome: A rare case with malignant transformation. Case Rep Otolaryngol doi: 10.1155/2018/9427650.
  4. Lauritano D et al. (2016) Oral lichen planus clinical characteristics in Italian patients: a retrospective analysis. Head Face Med 12:18.
  5. Mehlika B et al (2014) Contact allergic lichenoid reaction to eugenol presenting as lichen planus mucosae. Allergo J Int 23: 14-17
  6. Mansourian A et al. (2011) Comparison of aloe vera mouthwash with triamcinolone acetonide 0.1% on oral
  7. lichen planus: a randomized double-blinded clinical trial.Am J Med Sci 342:447-451.
  8. Ohno S et al (2011) Enhanced expression of Toll-like receptor 2 in lesional tissues and peripheral blood monocytes of patients with oral lichen planus. J Dermatol 38:335-344
  9. Poomsawat S et al (2011) Overexpression of cdk4 and p16 in oral lichen planus supports the concept of premalignancy. J Oral Pathol Med 40:294-249
  10. Rajar UD et al (2008) Efficacy of aloe vera gel in the treatment of vulval lichen planus. J Coll Physicians Surg Pak 18:612-614.
  11. Simark-Mattsson C et al (2013) Reduced immune responses to purified protein derivative and Candida albicans in oral lichen planus. J Oral Pathol Med 42:691-697
  12. van der Meij EH et al.(2003) The possible premalignant character of oral lichen planus and oral lichenoid
  13. lesions: a prospective study.Oral Surg Oral Med Oral Pathol Oral Radiol Endod 96:164-171.

Disclaimer

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

Authors

Last updated on: 18.12.2020