Lichen planus (overview) L43.-

Authors: Prof. Dr. med. Peter Altmeyer, Julian Baur

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Last updated on: 29.10.2020

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Synonym(s)

LIchen ruber; lichen ruber planus

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 may 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) or in actinic keratoses.

Classification
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Depending on the clinical morphology and distribution pattern, the lichen planus can be subdivided as follows:

classification by distribution pattern:

  1. Lichen planus exanthematicus (generalized Lichen planus)
  2. Localized Lichen planus
  3. Erythrodermixer Lichen planus
  4. Lichen planuslinearis (Lichen planus striatus)
    • Blaschkoider LP
    • Zosteriformer LP
  5. Inverse LP
  6. lichen planus mucosae
  7. Lichen planus genitalis (genital lichen planus; see below Lichen planus vulvae)
  8. Lichen planus of the nails

Classification by clinical appearance:

  1. Lichen planus classical type
  2. lichen planus actinicus
  3. varicella planus anularis
  4. lichen planus verrucosus
  5. lichen planus follicularis
  6. Graham-Little Syndrome
  7. lichen planus hypertrophicus
  8. lichen planus atrophicans
  9. Lichen planus erosivus (Erosive Lichen planus)
  10. lichen planus pigmentosus
  11. lichen planus bullosus
  12. lichen planus pemphigoides
  13. Invisible lichen planus (pruritic without clear clinical signs of LP; formerly lichen discretus).
  14. Overlap Syndrome:

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.

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 (see Lichen planus e medicatione) 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. Familial lichen planus is known (rare).

The morphological analogy of dermatitic reactions leads to the hypothesis that lichen planus is an autoimmune reaction against epitopes of basal keratinocytes modified by viral or drug induction. A striking feature is the distinct presence of antigen-presenting cells (APZ) in early lesions of the LP.

It is undisputed that the apoptotic destruction of the basal keratinocytes represents the common final stretch of the LP 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).

Further etiopathogenic associations:

  • 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.
  • Medicines: As drug triggers beta-receptor blockers, interferons, chloroquine and non-steroidal anti-inflammatory drugs are considered.
  • Paraneoplasia: Occasionally there are reports of a paraneoplastic lichen planus.

Manifestation
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Preferably occurring in adults between the 3rd and 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 (Schilling et al. 2018).

Localization
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The lichen planus is found preferentially on the mucous membranes (65%), often on the skin and mucous membranes (20%) and less frequently isolated on the skin (10%).

Involvement of the nails and hair can lead to permanent nail dystrophy or to scarring irreversible alopecia.

The following nail changes have been described in detail (cited in H. Hamm et al. 2018):

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 planus and follow-up: A descriptive study of 20 patients. Ann DermatolVenereol 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. Hamm H et al (2018) Diseases of the nails. In: Braun-Falco`s Dermatology, Venerology Allergology G. Plewig et al. (Hrsg) Springer Verlag S 1400
  10. 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
  11. 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
  12. Force K (2014) Naturally against pruritus. Close to the skin Dermatology 30: 42-43
  13. Kolb-Maurer A et al (2003) Treatment of lichen planus pemphigoides with acitretin and pulsed corticosteroids. dermatologist 54: 268-273
  14. Lehman J et al (2009) Lichen planus. Int J Dermatol 46: 682-694
  15. Pandhi D et al. (2014) Lichen planus in childhood: a series of 316 patients.
    Pediatrist Dermatol 31:59-67
  16. Schilling L et al (2018) Lichen ruber planus. dermatologist 69: 100-108
  17. Wilson E (1869) On lichen planus. J Cutan Med 8: 117
  18. Wolf R et al (2010) Pleomorphism of Lichen ruber - clinical variation, pathogenesis and therapy. Act Dermatol 36: 180-185

Disclaimer

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

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Last updated on: 29.10.2020