Lichen planus (overview) L43.-

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

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Last updated on: 02.03.2022

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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 pruritic, self-limited (duration of disease between 1 month and 10 years), inflammatory disease of the skin and/or mucous membranes of unexplained aetiology, with typical clinical (like polished shiny papules) and histological morphology (destruction of basal keratinocytes by cytotoxic T cells, lichenoid inflammation) and a characteristic, often flexor accentuated distribution pattern. Lichen (ruber) planus is marked by a characteristic "lichenoid tissue reaction", which may also characterize the histological picture in other inflammatory processes of the skin, e.g. in lichenoid drug reactions, in a"graft-versus-host reaction" in initial lichen sclerosus, in erythema dyschromicum perstans or also in actinic keratoses.

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

Classification by distribution pattern:

  1. Lichen planus exanthematicus (generalized lichen planus).
  2. Localized lichen planus
  3. Erythrodermic lichen planus
  4. Lichen planus linearis (Lichen planus striatus).
    • Blaschkoid LP
    • Zosteriform LP
  5. Inverse LP
  6. Lichen planus mucosae
  7. Lichen planus genitalis (Genital lichen planus; see also Lichen planus vulvae)
  8. Lichen planus of the nails

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Classification according to clinical appearance:

  1. Lichen planus classic type
  2. Lichen planus actinicus
  3. Lichen planus anularis
  4. Lichen planus verrucosus
  5. Lichenplanus follicularis
  6. Graham-Little syndrome
  7. Lichen planus hypertrophicus
  8. Lichen planus atrophicans
  9. Lichen planuserosivus (Erosive lichen planus)
  10. Lichen planus pigmentosus
  11. Lichen planus bullosus
  12. Lichen planus pemphigoides
  13. Invisible lichen planus (pruritic without definite clinical signs of LP; ehenmals lichen discretus).
  14. Overlap Syndromes:

Occurrence/Epidemiology
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Prevalence: 0.6%-1.2% of the (adult) population.

Up to 25% of patients have isolated lichen planus of the mucosa (see below Lichen planus mucosae).

Familial lichen planus is rare. About 100 cases are documented.

Etiopathogenesis
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Autoimmune reaction: To date, the etiology and pathogenesis of lichen planus is not fully understood. There are correlations with autoimmune diseases, viral infections, medications (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 critical effectors. Familial lichen planus is known (rare).

The morphologic analogy of 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. Strikingly, antigen-presenting cells (APCs) are clearly noted in early lesions of LP.

It is undisputed that apoptotic destruction of basal keratinocytes is the common final pathway of the LP reaction (this certainly plays a significant role in other autoimmune skin diseases, e.g., LE). Ligand-receptor-dependent dysregulation ( TNF-alpha/TNFR1= TNF-alpha receptor) is discussed as its cause.

Also discussed is the direct "pore formation" by perforin known in apoptosis and the subsequent enzymatic degradation by serine proteases (see Granzyme B below).

Other etiopathogentic associations:

  • Viral antigens appear to play a preferential role in the etiopathogenesis of lichen planus. The prevalence of HCV/HBV infection (hepatitis C/B) is 13.5-fold higher in lichen planus than in controls. In oral lichen planus, HCV RNA and TTV (transfusion-transmitted virus) DNA were detected in a high percentage in lesional mucosa. The occurrence of lichen planus after HBV vaccine has been described. The etiopathogenetic significance to HHV-7/HHV-8 is not clearly provable.
  • Diabetes mellitus: The association of lichen planus and diabetes mellitus is strikingly frequent. One in 2 patients has a disorder of glucose metabolism and one in 4 has manifest diabetes mellitus.
  • Contact allergens: The role ofcontact allergies to a number of metal salts (gold, amalgam, copper) is well known in oral lichen planus. Here, it is discussed that these hapten-type antigens may trigger an LP reaction.
  • Drugs: Drug triggers include beta receptor blockers, interferons, chloroquine, and nonsteroidal anti-inflammatory drugs.
  • Paraneoplasia: Isolated reports of paraneoplastic lichen planus exist. An association with thymoma has been described (see Good syndrome below).

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: 02.03.2022