Lichen ruber planus

Wilson, 1869

Lichen ruber, lichen planus.

Pruritic, non-contagious, subacute to chronic, self-limiting (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 isolated lichen planus of the mucosa
• Familial lichen planus is rare
• Most common between 30-60 yrs, rare in children
• Women = men
• No ethnic predisposition

• Lichen ruber planus (classic form)
• Lichen ruber integumentalis
• Lichen ruber anularis
• Lichen ruber linearis
• Blaschkoider 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 and soles

• Not yet fully understood. CD8+ T-cells cause apoptosis of keratinocytes.
• Approximately 100 cases of familial occurrence have been described

• Associations
  • Autoimmune diseases
    • Ulcerative colitis
    • Crohn's disease
    • Alopezia areata
    • Vitiligo
    • Dermatomyositis
    • Myastenia gravis
    • Pemphigus vulgaris
    • Bullous pemphigoid
    • Lupus erythematosus
  • Viral infections
  • Medications
    • Antiarthritics
      • Gold compounds
      • D-penicillamine
    • Antibiotics
      • Streptomycin
      • Tetracyclines
    • Anti-diabetics
      • Sulfonylureas
      • Glimepiride
    • Antileprosa
      • Dapsone
    • Antimalarials
      • Chloroquine
      • Hydroxychloroquine
      • Chinine
    • Antipsychotics
      • Levomepromazine
      • Amitriptyline
      • Carbamazepine
    • Diuretics
      • Hydrochlorothiazide
      • Furosemide
      • Spironolactone
    • Tuberculostatics
      • Paraaminosalicylic acid
      • Isoniazide
    • Antihypertensives
      • β-blockers
      • Calcium antagonists
      • ACE inhibitors
    • Antiphlogistics
      • ASS
      • Ibuprofen
      • Naproxen
    • Lipid reducer
      • Simvastatin
      • Pravastatin
  • Mechanical trauma (Köbner phenomenon)
  • Chronic graft-versus-host disease (GVHD)
  • Metabolic diseases
    • Diabetes mellitus
    • Hypercholesterolaemia
    • Hyperuricaemia
  • Liver diseases
    • Chronic active hepatitis
    • Primary biliary cirrhosis
  • Contact allergens
  • Paraneoplasia

Particularly on the flexors of the wrists and forearms, on the lateral ankles, sharply demarcated, erythematous, lichenoid, polygonal, lacquer-like shiny, partly confluent papules and plaques appear. Often a whitish reticulation (wickham's pattern) can be seen. A characteristic feature of cutaneous primary efflorescences is the surface reflection, which is best recognised when the light is incident from the side. Exanthematous spreading may occur in the course. As a rule, the efflorescences flatten out again after months. Hyperpigmentation develops especially in dark-skinned patients.

• Wickham's drawing/ Wickham's phenomenon/ Wickham's stiffening: whitish reticulation on the primary efflorescences. This is often particularly visible around the mucous membranes. In patients with dark skin colour, the Wickham's pattern is often absent
• Köbner phenomenon: isomorphic stimulus effect triggered by mechanical, thermal or chemical triggers, which triggers a linear arrangement of primary efflorescences. This arises from manipulating / scratching the patient due to the lesional pruritus.

• Palmoplantar as well as lateral edges: Hyperkeratotic, whitish-yellowish plaques with erythematous margins may be visible.
• Nails:
  • Rarely isolated infestation
  • Dystrophy, atrophy, spotted nails, longitudinal striations (erythronychia), pterygia, subungual keratoses, onychoschisis
  • Partial or complete destruction of the nail possible
• Capillitium:
  • Lichen planus follicularis capillitii
    • Pruritic, chronically stationary rather blurred, sometimes hyperkeratotic erythema. Sometimes tufts of hair are seen. Characteristic is the scarring arising from the erythema.
  • Lichen planopillaris
    • Diffuse disseminated follicular-bound erythema, which changes to hyperkeratotic papules up to 1 cm in diameter. In the course, these lesions may also become atrophic or scarring.
• A keratosis follicularis is partly visible on the upper arms or thighs
• Ulerythema ophryogenes is a follicular hyperkeratosis and vasodilatation of the eyebrows. In the course, follicular atrophy may occur
• Mucosal involvement (lichen planus mucosae): Wickham's stiffening is usually clearly visible here. In addition, whitish, nummular papules and plaques may also be visible
  • In erosive lichen planus mucosae, there is an increased tendency to malignancy
  • Lichen planus vulvae is characterised by an anular or circular form

• Anamnesis
  • Medication intake?
• Clinical picture
• Biopsy
  • Dermatopathology
  • Direct immunofluorescence (usually not necessary in practice, but often characteristic)
• Laboratory
  • AST, ALT, γ-GT, cholesterol, uric acid, haemoccult

• Mucous membranes (oral and genital mucosa)
• Nails
• Capillitium: lichen planopilaris

• Interface dermatitis with sawtooth-like acanthosis, orthohyperkeratosis and hypergranulosis. Lymphoid cellular, epidermotropic infiltrate, predominantly consisting of CD8-positive T cells.

• Direct immunofluorescence
  • Subepithelial, ribbon-like fibrin deposits. Cytoid corpuscles load with IgG, IgM, C3 in epithelium and str. papillare.

• Evolution of squamous cell carcinoma
  • V.a. in erosive or verrucous forms
• Scarring alopecia

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

Topical therapy

• Mometasone fuorate cream / solution / ointment
• Clobetasol cream 1-2 dgl (for 1-3 days)
• Tacrolimus ointment 0.03-0.1% 2x daily for 2 weeks
• Pimecrolimus cream 1% 2x tgl. for 2 weeks
  
• In the area of the genital mucous membranes
  • Tacrolimus ointment 0.03-0.1% 2x daily for 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
  • Outcome trial after 1/2 year at the earliest.
• Prednisolone p.o. 0.5 mg/kg tgl
  • Taper off over 4-6 weeks.
  • Maintenance dose according to clinic 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.