Lichen planus classic type L43.-

Author: Prof. Dr. med. Peter Altmeyer

Co-Autor: Jeton Luzha

Our authors

Last updated on: 29.10.2020

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Synonym(s)

lichen ruber; lichen ruber planus; Nodular lichen

History
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Wilson, 1869

Definition
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Non-contagious, subacute to chronic, markedly itchy, self-limited (duration of disease between 1 month and 10 years), inflammatory disease of the skin and/or mucous membranes of unclarified aetiology, with typical clinical (such as polished shiny papules) and histological morphology (destruction of basal keratinocytes by cytotoxic T cells) and a characteristic distribution pattern often accentuated on the flexural side. The lichen planus is characterized by a characteristic "lichenoid tissue reaction", which can also occur in other inflammatory processes of the skin (e.g. in lichenoid drug reactions, in a "graft-versus-host reaction" in initial lichen sclerosus et atrophicus, in erythema dyschromicum perstans).

Occurrence/Epidemiology
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Prevalence: 0.2%-1.0% of the (adult) population.

Up to 25% of patients have an isolated lichen planus of the mucosa.

Familial lichen planus is rare (about 100 cases are known). Earlier age of manifestation than non-familial LP

Etiopathogenesis
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Autoimmune reaction: Until today the etiology and pathogenesis of lichen planus is not fully understood. There are correlations to autoimmune diseases, viral infections, drugs and mechanical trigger factors (scratching, rubbing, etc.). LP-like lesions occur in chronic graft-versus-host disease (GVHD), in which alloreactive cytotoxic T cells and antibodies that recognize foreign MHC molecules are key effectors. The morphological analogy of the dermatitic reactions leads to the hypothesis that in lichen planus there is an autoimmune reaction against epitopes of basal keratinocytes modified by viral or drug induction. It is undisputed that the apoptotic destruction of basal keratinocytes is the common final pathway of the lichen planus reaction (this certainly plays an important role in other autoimmune skin diseases, e.g. LE). As its cause ligand-receptor dependent dysregulations ( TNF-alpha/TNFR1= TNF-alpha-receptor) are discussed. Also discussed is the direct "pore formation" by perforin known from apoptosis and the subsequent enzymatic degradation by serine proteases (see Granzyme B below).

In early changes, the marked increase of antigen-presenting cells (APCs) is noticeable. These are possibly induced by the keratinocytes themselves due to a malfunction in the cytokine production.

Viral antigens seem to play a preferential role in the aetiopathogenesis of lichen planus. The prevalence of HCV/HBV infections (hepatitis C/B) is 13.5 times higher in lichen planus than in controls. A high percentage of HCV-RNA and TTV-DNA (transfusion-transmitted-virus) in lesional mucosa could be detected in oral planus lichen. The occurrence of lichen planus after HBV vaccine is described. The etiopathogenetic significance to HHV-7/HHV-8 cannot be clearly proven.

Diabetes mellitus: An association of lichen planus and diabetes mellitus is remarkably frequent. In every 2nd patient there is a disturbance of glucose metabolism and in every 4th a manifest diabetes mellitus.

Contact allergens: The role of contact allergies to a number of metal salts (gold, amalgam, copper) in oral planus lichen is well known. It is discussed that these antigens can trigger an LP reaction in the manner of haptens.

Paraneoplasia: Occasionally there are reports of a paraneoplastic lichen planus.

Familial occurrence: About 100 cases of familial lichen planus have been reported. There is no significant relationship to certain HLA types.

Manifestation
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Preferably occurring in adults in the 3rd to 6th decade of life.

Rarely in children (about 1-4% of cases).

No ethnic predisposition. Women seem to contract the disease slightly more frequently than men.

Localization
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Mainly the flexion sides of the wrists and forearms, lateral ankle area of the ankles

Mucous membrane infestation (30-40%): Penis, oral and genital mucosa

Skin and mucous membrane infestation (20%)

Nails (10%)

Capillitium.

Generalized or universal (erythrodermic) occurrence is possible (see Lichen planus exanthematicus).

Clinical features
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Initially about 0.1 cm large, raised, plateau-like, smooth, lacquer-like shiny (as if polished), clearly itchy, red papules bordered by the natural skin furrows. Aggregation of several papules with formation of plaques of varying size. The diagnostically important surface reflection of the lichen-planus papules can best be recognized when light is incident from the side. Also typical are the Wickham's pattern and the linear arrangement of the efflorescences in scratch or rub marks (see Koebner phenomenon [= isomorphic stimulus effect] below). The lesional itching is answered by the patient with rubbing (often shiny nails are detectable); excoriations rarely occur in this case (thus, scratch marks are missing as they are to be expected e.g. in atopic dermatitis ).

On the palms of the hands and soles of the feet including the lateral edges localized, coarse, yellowish, hyperkeratotic plaques can be seen, on the edges with red rim.

Infestation of the oral mucosa(see also Lichen planus mucosae) is observed in > 50% of patients. Typical are symmetrical, reticular or nummular white plaques, also disseminated 0.1 cm white papules of the buccal mucosa and/or tongue and/or gingiva. A special feature is the erosive lichen planus of the oral mucosa.

Genitalia, in particular the glans penis and the vulva, are often affected in the form of annular or circulatory, whitish but also red or erosive plaques (see below Lichen planus mucosae/ Lichen planus vulvae).

Capillitium (see below lichen planus follicularis capillitii).

Nails: Frequently thinned and shortened, often frayed nail plates with longitudinal distortions of the surface and numerous spots. Pterygias possible. Rarely colourless or red longitudinal stripes ( erythronychia). Complete destruction of the nail plate is possible. Shiny nails are not uncommon (consequence of continuous rubbing).

Laboratory
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No pioneering laboratory parameters!

Histology
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Uniform and pathognomic histological pattern of a classical interface dermatitis with irregular, often sawtooth-like acanthosis, compact orthohyperkeratosis with prominent hypergranulose (circumscribed thickening of the keratohyalin-containing cell layers of the stratum granulosum causes the clinical picture of Wickham's drawing). Usually very prominent, dense, band-shaped, lymphoid-cellular, epidermotropic infiltrate. The inflammatory infiltrate consists predominantly of oligoclonal, CD8-positive, cytotoxic T-cells. Focal pigment incontinence. Vacuole degeneration of the basal epithelial cell layers, which can lead to the formation of fissures (Max-Joseph's spaces) or subepithelial blisters (Lichen planus bullosus). Detection of numerous cytoid bodies (see apoptosis below). Epidermal Langerhans cells are increased in active lesions. Plasma cells, neutrophil and eosinophil leukocytes may be present, but are not common.

Direct Immunofluorescence
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Typical, strong, band-shaped, subepithelial fibrin deposits. Clear fluorescence phenomena of the cytoid corpuscles withC3 and IgM antibodies.

Differential diagnosis
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  • Clinical Differential Diagnosis:
    • Integument:
      • Psoriasis punctata: The prying out phenomenon is always detectable and always absent in lichen planus!
      • Lichenoid drug exanthema: Anamnesis; usually no infection of the oral mucosa.
      • Pityriasis lichenoides et varioliformis acuta: As in the "Heubner's star chart" very polymorphic, itchy or even burning exanthema, with papules, erosions, ulcers and possibly hemorrhagic vesicles. The Lichen planus exanthema is monomorphic.
      • Papular syphilide: Clinically the lichenoid character of the single lesions is missing; itching is slight or absent.
      • Scabies: In scabies as well as in lichen ruber multiple erythematous changes of the papules of the wrists may complicate the differential diagnosis.
    • Oral mucosa:
      • Leukoplakia: In the region of the oral mucosa leukoplakia or mechanical irritation of the mucosa must be distinguished. Histological clarification necessary. In both cases the typical "anular structures" of the LP are missing.
      • Candidosis of the oral mucosa: Infestation mainly of the tongue, also of the cheek mucosa and soft and hard palate with white to grey-white, easily strippable (LP not strippable!) plaques.
      • Gingivitis marginalis: similar, therapy-resistant pattern with analogous symptoms. Histological clarification is necessary.
  • Histological Differential Diagnoses:
    • Lichenoid drug exanthema: largely identical picture, apoptotic keratinocytes are frequent, possibly focal parakeratosis, which is always absent in lichen planus. Clear histoeosinophilia is possible as well as a plasmacellular infiltrate component.
    • Fixed drug reaction: Numerous apoptotic keratinocytes, perivascular infiltrate compression, often marked eosinophilia, marked pigment incontinence.
    • Lichen sclerosus et atrophicus: Initial lichenoid infiltrate without 3-zone phenomenon. Pattern analogous to the lichen planus; later typical zonal structure.
    • Acute graft-versus-host disease: Numerous apoptotic keratinocytes, strong vacuolisation of the junctional zone, less dense infiltrate.
    • Papular syphilide: Epidermis with psoriasiform acanthosis and focal spongiosis; admixture of neutrophilic granulocytes and (numerous) plasma cells.

Complication(s)
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In long persistent LP lesions of the skin and mucous membranes there is a certain (not to be specified) risk of developing epithelial tumors (spinocellular carcinomas).

General therapy
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The therapy depends on the clinical aspect and the course. Treatment of itching is in many cases the main focus.

External therapy
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Glucocorticoids: In the case of described low symptom findings, medium-strength glucocorticoids such as 0.25% prednicarbate (e.g. Dermatop® cream), 0.1% mometasone furoate (e.g. Ecural® fat cream), in persistent cases also strong 0.05% glucocorticoids such as clobetasol (e.g. Dermoxin® cream), if necessary also under occlusion (2 times/day 2-4 hours).

If necessary, inject the foci with glucocorticoid crystal suspension such as triamcinolone acetonide (e.g. Volon® A) 10-40 mg with 2-4 ml 1% mepivacaine in a syringe and apply intrafocally.

Calcineurin inhibitors: Tacrolimus or Pimecrolimus can be applied topically as off-label use. Both substances are especially effective in case of mucosal infestation. Because of the unknown long-term effects of calcineurin inhibitors and the carcinogenicity of Pimecrolimus which has been proved in animal experiments, the indication for the therapy with calcineurin inhibitors has to be set very strictly!

Radiation therapy
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PUVA: For extensive, especially disseminated forms, PUVA bath therapy, a re-PUVA therapy (PUVA + Acitretin) or a systemic PUVA therapy are suitable. Success is shown in approx. 80-90% of cases.

Internal therapy
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Acitretin: In case of extensive infestation, start of therapy with acitretin (neotigason) initially 0.5 mg/kg bw/day, maintenance dose 0.1-0.2 mg/kg bw/day after clinic. Systemic retinoids achieve the highest evidence levels in studies (Schilling L et al. 2018). Attempt to discontinue after 1/2 year at the earliest.

Alternatively, or in the case of a severe form: Acitretin in combination with glucocorticoids such as prednisolone (e.g. Decortin H) initially 0.5 mg/kg bw/day, release over a period of 4-6 weeks. Maintenance dose according to clinic with 5-10 mg/day.

Alternatively, other systemic therapeutics described in several smaller studies can be considered as "third line" therapy for therapy-resistant lichen planus of the external integument. These include: Ciclosporin A, griseofulvin, oral metronidazole, sulfasalazine, mycophenolate mofetil, azathioprine, thalidomide.

Alternatively, experimentally: Apremilast, an oral thalidomide analogue, which has been successfully tested in a smaller monocentric study for the exanthematic lichen planus.

Special questions:

  • For the generally therapy-resistant lichen planus erosivus mucosae (see there), stronger local or systemic immunosuppressive measures are necessary.
  • Lichen planus genitalis: Therapeutic measures see below Lichen planus erosivus mucosae.
  • Lichen planus follicularis capillitii (see there).
  • Lichen planus of the nails: I.A. no special therapy, since nail changes usually occur with other LP lesions. Some US authors recommend perilesional injections with glucocorticoids(Cave! painfulness). In individual cases, good therapeutic success has been reported with Ciclosporin A (initial: 2x100mg/day p.o. maintenance dose 100mg/day p.o.). Own experiences exist with azathioprine (initial: 1.0-1.5mg/kgkgkg, maintenance dose at 0.5mg/kgkgkg).
  • In the case of drug-induced lichen planus, the initiating drugs must be discontinued. Otherwise therapy as with classical lichen planus.

Progression/forecast
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Different course: Acute course with healing within one year up to a chronic course lasting for years (decades). Spontaneous remissions are possible. Mucosal changes that have existed for years are to be regarded as facultative precanceroses!

Naturopathy
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External: Phytotherapy: Anti-itching herbal ingredients with cooling effect such as: camphor, mint or peppermint oil, menthol in DAC cream as a base, capsaicin (0.02-0.075%) can be used for intact skin! 2-3x /day can be applied.

Example of a formulation for a 1% menthol cream:

  • menthol1,0
  • DAC base cream ad 100.0.
  • S. Apply Ambiphile 1% menthol cream 2-3 times/day to the itchy skin areas. Usage period: Tube: 6 months

Systemic: Furthermore, oral bromelain (e.g. Bromelain-POS) or Phlogenzym(Phlogenzym-mono) is recommended.

Literature
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  1. Brănişteanu EN et al. (2014) Cutaneous manifestations associated with thyroid disease. Rev Med Chir Soc Med Nat Iasi 118: 953-958
  2. Butch F et al (2014) Successful therapy of a lichen planus of the nails with Ciclosporin. SDDG 12: 724-725
  3. Chiheb S et al (2015) Clinical characteristics of nail lichen planus and follow-up: A descriptive study of 20 patients. Ann Dermatol Venereol 142:21-25
  4. Deen K et al (2015) Mycophenolate mofetil in erosive genital lichen planus: A case and review of the literature. J Dermatol doi: 10.1111/1346-8138.12763
  5. De Vries et al (2007) Lichen planus remission is associated with decrease of human herpes virus zype 7 protein expression in plasmacytoid dendritic cells. Arch Dermatol Res 299: 213-219
  6. Eisman S, Orteu CH (2004) Recalcitrant erosive flexural lichen planus: successful treatment with a combination of thalidomide and 0.1% tacrolimus ointment. Clin Exp Dermatol 29: 268-270
  7. Frieling U et al (2003) Treatment of severe lichen planus with mycophenolate mofetil. J Am Acad Dermatol 49: 1063-1066
  8. Gandolfo S et al (2004) Risk of oral squamous cell carcinoma in 402 patients with oral lichen planus: a follow-up study in an Italian population. Oral Oncol 40: 77-83
  9. Harden D et al (2003) Lichen planus associated with hepatitis C virus: no viral transcripts are found in the lichen planus, and effective therapy for hepatitis C virus does not clear lichen planus. J Am Acad Dermatol 49: 847-852
  10. Hodgson TA et al (2003) Long-term efficacy and safety of topical tacrolimus in the management of ulcerative/erosive oral lichen planus. Eur J Dermatol 13: 466-470
  11. Force K (2014) Naturally against pruritus. Close to the skin Dermatology 30: 42-43
  12. Kolb-Maurer A et al (2003) Treatment of lichen planus pemphigoides with acitretin and pulsed corticosteroids. dermatologist 54: 268-273
  13. Lehman J et al (2009) Lichen planus. Int J Dermatol 46: 682-694
  14. Wilson E (1869) On lichen planus. J Cutan Med 8: 117
  15. Wolf R et al (2010) Pleomorphism of Lichen ruber - clinical variation, pathogenesis and therapy. Act Dermatol 36: 180-185

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Last updated on: 29.10.2020