Lichen ruber planus

Last Updated: 2020-06-25

Author(s): -

Wilson 1869.

Nodular lichen, lichen ruber, lichen planus.

Pruritic, non-infectious, subacute to chronic, self-limited (but often lasting for years) disease of the skin and/or mucous membranes.

  • Prevalence: 0.2% - 1.0% of the (adult) population.
  • Up to 25% of patients have an isolated lichen planus of the mucosa.
  • Family lichen planus is rare.
  • Occurs most frequently between 30-60 years, rarely in children.
  • Women = Men 
  • No ethnic predisposition.
  • Lichen ruber planus (classical form)
  • Lichen ruber integumentalis
  • Lichen ruber anularis
  • Lichen ruber linearis
  • Blaschkoid lichen planus
  • Zosteriform lichen planus
  • Lichen ruber verrucosus
  • Lichen planus hypertrophicus
  • Lichen ruber atrophicans
  • Lichen planus pigmentosus
  • Lichen ruber vesiculosus et bullosus
  • Lichen ruber pemphigoides
  • Lichen ruber actinicus
  • Lichen planus exanthematicus 
  • Erythrodermic lichen planus
  • Inverse lichen planus
  • Lichen ruber of the mucous membranes
  • Lichen planus mucosae
  • Lichen planus genitalis
  • Lichen ruber of the skin appendages
    • Lichen ruber follicularis (Lichen planopilaris) 
    • Lichen ruber of the nails
    • Lichen ruber of the palms of the hands and soles of the feet
  • Not fully understood yet,  CD8+ T cell mediated apoptosis of keratinocytes
  • Approx. 100 cases of familial occurrence are described.
  • Associations 
    • Autoimmune diseases 
      • Ulcerative colitis 
      • Crohn's disease 
      • Alopecia areata 
      • Vitiligo 
      • Dermatomyositis 
      • Myastenia gravis 
      • Pemphigus vulgaris 
      • Bullous pemphigoid 
      • Lupus erythematosus 
    • Viral infections 
    • Medications 
      • Antiarthritic drugs
        • Gold compounds 
        • D-Penicillamine
      • Antibiotics 
        • Streptomycin 
        • Tetracycline 
      • Anti-Diabetics
        • Sulphonylureas
        • Glimepiride
      • Antileprosy
        • Dapson
      • Antimalarials 
        • Chloroquine 
        • Hydroxychloroquine 
        • Quinine 
      • Antipsychotics 
        • Levomepromazine 
        • Amitriptyline 
        • Carbamazepine 
      • Diuretics 
        • Hydrochlorothiazide 
        • Furosemide 
        • Spironolactone 
      • Tuberculostatics 
        • Paraaminosalicylic acid
        • Isoniazide 
      • Antihypertensives 
        • β-Blocker
        • Calcium antagonists 
        • ACE inhibitors
      • Anti-inflammatory drugs
        • ASS
        • Ibuprofen
        • Naproxen
      • Lipid reducers 
        • Simvastatin
        • Pravastatin
    • Mechanical trauma ( Koebner phenomenon)
    • Chronic Graft-versus-Host Disease (GVHD)
    • Metabolic disorders 
      • Diabetes mellitus
      • Hypercholesterolemia
      • Hyperuricemia 
    • Liver diseases 
      • Chronically active hepatitis
      • Primary biliary cirrhosis
    • Contact allergens 
    • Paraneoplasia
  • Particularly on the flexor sides of the wrists and forearms, on the lateral knuckles of the ankles, sharply defined, erythematous, lichenoid, polygonal lacquer-like shiny, partly confluent papules and plaques can be seen. Often a whitish reticulation, the Wickham's drawing, can be recognized. A characteristic feature is the surface reflection of cutaneous primary fluorescence, which is best recognized when light is entering from the side. Although the skin lesions begin insidiously at first, exanthematic seeding may occur in the course of the disease. Usually the efflorescences flatten out after months. Hyperpigmentation occurs especially in dark-skinned patients.

    • Wickham's drawing / Wickham's phenomenon / Wickham stiffening: whitish netting on the primary fluorescence. Especially in the area of the mucous membranes this is often clearly visible. In patients with dark skin colour the Wickham's pattern is often missing.
    • Koebner phenomenon: isomorphic stimulation effect triggered by mechanical, thermal or chemical triggers, which triggers a linear arrangement of primary fulorescences. This is caused by the manipulation/scratching by the patient due to the lesional pruritus.
  • Palmoplantar as well as on the side edges: Hyperkeratotic, whitish yellowish plaques with erythematous margins may be visible.
  • Nails:
    • Rarely isolated infestation.
    • Dystrophy, atrophy, spotted nails, longitudinal stripes (erythronychia), pterygias, subunguinal keratoses, onychoschisis.
    • Partial or complete destruction of the nail is possible.
  • Capillitium: 
    • Lichen planus follicularis capillitii
      • Pruritic, chronically stationary rather blurred, partly hyperkeratotic erythema. Sometimes tuft hairs are visible. Scarring caused by the erythema is characteristic.
    • Lichen planopillaris
      • Diffusely disseminated follicular-bound erythema, which changes into hyperkeratotic papules up to 1 cm in diameter. In the course of time these lesions can also become atrophic or scarred.
  • Keratosis follicularis is partially visible on the upper arms or thighs.
  • Ulerythema ophryogenes is a follicular hyperkeratosis and vascular dilatation of the eyebrows. In the course of the disease a follicular atrophy may occur.
  • Mucous membrane infestation (Lichen planus mucosae): Usually the Wickham's stiffening is well visible here. Whitish, nummular papules and plaques may also be visible.
    • With the erosive Lichen planus mucosae there is an increased tendency towards malignancy.
    • Lichen planus vulvae is characterized by an anular or circulatory form.
  • Medical history
    • Taking medication?
  • Clinical picture
  • Biopsy
    • Dermatopathology
    • Direct immunofluorescence (in practice usually not necessary, but often characteristic)
  • Laboratory
    • AST, ALT, γ-GT, cholesterol, uric acid, hemoccult

  • Mucous membranes (mouth and genital mucosa)
  • Nails
  • Capillitium: lichen planopilaris.
  • Interface dermatitis with sawtooth-like acanthosis, orthohyperkeratosis and hypergranulosis Lymphoid-cell, epidermotropic infiltrate, predominantly consisting of CD8-positive T cells.
  • Direct Immunofluorescence
    • Subepithelial, band-shaped fibrin deposits. Cytoid corpuscles are loaded with IgG, IgM, C3 in the epithelium and str. papillary.
  • Development of squamous cell carcinoma 
    • Especially with erosive or verrucous forms 
  • Scarring alopecia

Usually spontaneous healing within 1-2 years, but there are also chronic courses.

Topical Therapy 

  • Mometasone furoate cream / solution / ointment 
  • Clobetasol cream 1-2 days (for 1-3 days)
  • Tacrolimus ointment 0.03-0.1% 2x a day 2 weeks 
  • Pimecrolimus Cream 1% 2x a day for 2 weeks 

 

  • In the area of the genital mucous membranes 
  • Tacrolimus ointment 0.03-0.1% 2x daily 2 weeks 

 

  • In the area of the oral mucosa 
    • Kenacort-A Orabase® Paste
    • Dynexan® Mouth Gel
    • Kamillosan® mouth throat spray 

 

At the capillitium

  • Clobetasol foam for application on the skin

 

Radiation therapy 

  • UVA Therapy 

 

Systemic Therapy 

  • Acitretin p.o.
  • Initial: 0.5 mg/kg bw daily 
  • Maintenance dose: 0.1-0.2 mg/kg bw daily 
  • Removal attempt at the earliest after 1/2 year.

 

  • Prednisolone p.o. 0.5 mg/kg daily 
  • Gradual discontinuation over 4-6 weeks.
  • Maintenance dose according to clinical features between 5-10 mg daily.
  1. Kaposi M. Noch einmal: Lichen ruber acuminatus und Lichen ruber planus. Arch f Dermat 1895;31:1-32. 
  2.  Mehregan AH, Heath LE, Pinkus H. Lichen ruber moniliformis and lichen ruber verrucosus et reticularis of Kaposi. J Cutan Pathol 1984;11:2-11. 
  3.  Behzad M, Michl C, Arweiler N, Pfützner W. Kontaktallergische lichenoide Reaktion auf Eugenol unter dem Bild eines Lichen ruber mucosae. Allergo Journal 2014;23:14-7. 
  4.  Lebwohl, Mark. Treatment of Skin Disease: Comprehensive Therapeutic Strategies. Elsevier, 2014. Print.