Lichen planus classic type L43.-

Author: Prof. Dr. med. Peter Altmeyer

Co-Autor: Jeton Luzha

All authors of this article

Last updated on: 31.08.2021

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

No ethnic predisposition. Women seem to be affected slightly more often than men (see also Lichen planus mucosae).

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 in size, bordered by the natural furrows of the skin, raised, plateau-like, smooth, lacquer-like shiny (as if polished), distinctly itchy, red papules. Aggregation of several papules with formation of plaques of different sizes. The diagnostically important surface reflection of the lichen planus papules is best recognised when the light is incident from the side. Wickham's pattern and the linear arrangement of the efflorescences in scratch or rub marks are also typical (see below: Köbner phenomenon [= isomorphic stimulus effect]). The patient responds to the lesional itching by rubbing ( glossy nails are frequently detectable); excoriations rarely occur (thus scratch marks are absent as they are to be expected in atopic dermatitis, for example).

The palms and soles, including the lateral edges, show localized, coarse, yellowish, hyperkeratotic plaques, with red margins at the edges.

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

Genitalia, especially the glans penis and the vulva, are frequently affected in the form of anular or circular, 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 surface distortions and numerous spots. Pterygia possible. More rarely colourless or red longitudinal striations ( erythronychia). Complete destruction of the nail plate is possible. Shiny nails are not uncommon (consequence of constant rubbing).

Laboratory
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No pioneering laboratory parameters!

Histology
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Uniform and pathognomic histological pattern of a classic interface dermatitis with irregular, frequently sawtooth-like acanthosis, compact orthohyperkeratosis with prominent hypergranulosis (circumscribed thickening of the keratohyalin-containing cell layers of the stratum granulosum causes the clinical picture of Wickham's pattern). Usually very prominent, dense, band-like, lymphoid cellular, epidermotropic infiltrate. The inflammatory infiltrate consists predominantly of oligoclonal, CD8-positive, cytotoxic T cells. Focal pigmentary incontinence. Vacuolar degeneration of the basal epithelial cell layers, resulting in clefts (Max-Joseph spaces) and even subepithelial blister formation (Lichen planus bullosus). Detection of numerous cytoid bodies (see apoptosis below). Epidermal Langerhans cells are increased in active lesions. Plasma cells, neutrophils and eosinophils 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 diagnoses:
    • Integument:
      • Psoriasis punctata: The Auspitz phenomenon is always detectable and always absent in lichen planus!
      • Lichenoid medicinal exanthema: History; usually no involvement of the oral mucosa.
      • Pityriasis lichenoides et varioliformis acuta: As in "Heubner's star chart" very polymorphous, pruritic or also burning exanthema, with papules, erosions, ulcers and possibly hemorrhagic vesicles. Lichen planus exanthema is monomorphic.
      • Papular syphilid: Clinically, the lichenoid character of the single-cell lesions is absent; pruritus is mild or absent.
      • Scabies: Multiple erythematous papules on the wrists may complicate the differential diagnosis of scabies and lichen ruber.
    • Oral mucosa:
      • Leukoplakia: In the area of the oral mucosa, leukoplakia or mechanical mucosal irritations have to be differentiated. Histological clarification is necessary. In both cases the typical "anular structures" of the LP are missing.
      • Candidiasis of the oral mucosa: Infestation of the tongue, cheek mucosa and soft and hard palate with white to grey-white plaques that can be easily wiped off (LP cannot be wiped off!).
      • Gingivitis marginalis: similar, therapy-resistant pattern with analogous symptoms. Histological clarification is necessary.
  • Histological differential diagnoses:
    • Lichenoid medicinal exanthema: Largely identical picture, apoptotic keratinocytes are frequent, possibly focal parakeratosis, which is always absent in lichen planus. Marked histoeosinophilia is possible as well as a plasma cell infiltrate component.
    • Fixed drug reaction: Numerous apoptotic keratinocytes, perivascular infiltrate thickening, often marked eosinophilia, prominent pigment incontinence.
    • Lichensclerosus et atrophicus: Initial lichenoid infiltrate without 3-zone phenomenon. Pattern analogous to lichen planus; later typical zonal pattern.
    • Acute graft-versus-host disease: Numerous apoptotic keratinocytes, strong vacuolization of the junctional zone, less dense infiltrate.
    • Papular syphilid: Epidermis with psoriasiform acanthosis and focal spongiosis; admixture of neutrophilic granulocytes and (numerous) plasma cells.

Complication(s)
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In long persisting LP lesions of the skin and mucous membranes there is a certain (unspecified) risk to develop 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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Externally: Phytotherapy: Anti-itching plant 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 applied on intact skin! 2-3x /day can be applied.

Formulation example for a 1% menthol cream:

  • Menthol1,0
  • DAC base cream ad 100,0.
  • S.:Apply Ambiphilic 1% Menthol Cream 2-3 times/day to itchy skin areas. Use-by date: Tube: 6 months

Systemic: Oral bromelain (e.g. Bromelain-POS) or Phlogenzym(Phlogenzym-mono) is further 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: 31.08.2021