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Lichen Planus

See also article on Oral lichen planus Oral Lichen Planus.

Essentials

  • Lichen planus is a chronic autoimmune disease usually occurring on the skin and/or mucosa.
  • The course of the disease and the symptoms often fluctuate; spontaneous recovery occurs in a large proportion of patients.
  • The treatment is symptomatic.
  • Drug-induced dermatitis may look like lichen planus (lichenoid drug eruption).

General remarks

  • A mainly cell-mediated autoimmune disease (CD8-positive T-cells reacting against keratinocytes)
  • Uncommon; lifetime prevalence less than 1% of the population
  • Peak incidence at the age of 30-60 years
  • Several triggering factors have been suspected.
    • There is most evidence of the role of hepatitis C.
    • Other possible factors include drugs, vaccinations, other infections and psychosocial stress.

Clinical features

Skin

  • Typically, there are bluish-red, flat, glistening, polygonal papules with a pale, fine mesh-like surface (Wickham striae; Images 1 2).
  • The main symptom is itching.
  • Typical sites include the volar folds of the wrist, flexor surfaces of the forearms, ankles (Image 8) and lower back. Single papules are often spread over the trunk.
  • Common sites also include the proximal palms, soles, lower lip (Image 9), in men the glans penis (Image 10) and in women the labia and perineal skin.
  • The Koebner phenomenon is typical for lichen planus: scratches on the skin or scars become affected with papules.
  • There may also be different variants.
    • Some of the papules may have bullae (lichen bullosus).
    • On the legs, in particular, the lesions may become hypertrophic (lichen hypertrophicus; Images 3 4).
    • Ring-like grouping of papules (lichen annularis) can often be seen. On dark skin, in particular, hyperpigmented spots may be seen in areas exposed to UV radiation (actinic lichen planus).
    • After healing, lichen planus may leave dark spots (hyperpigmentation) healing slowly over time. These are particularly common in intertriginous disease (inverse lichen planus).
    • Some patients have lichen planus not only on the trunk and limbs but also on the scalp. Lichen planus may also be restricted to the scalp alone (lichen planopilaris), which may cause scarring, patchy hair loss Hair Loss and Balding.
    • Lichen planus may cause nail lesions Nail Lesions and Disorders. Longitudinal lines and pterygia (wings) growing from the nailfold on top of the nail are typical. The area may be scarred with permanent damage.

Oral and genital mucosa

  • Lichen planus may occur on mucosa in addition to skin, or on mucosa only.
  • The disease is divided into three subtypes based on the clinical picture.
    • On the oral mucosa, a pale mesh-like surface is typical (reticular lichen planus; see article Assessment of Oral Mucosal Changes and Images 5 6 7).
    • Erythematous (atrophic) lichen planus causes erythematous, atrophic spots on the mucosa.
    • The erosive form causes ulceration (in the mouth, for example; in women, vulvovaginitis).
    • Chronic mucosal lichen planus is a risk factor for squamous cell carcinoma.

Other types of lichen planus

  • Lichen planus may very rarely occur in the outer or inner ear (ear pain, hearing loss) or the oesophagus (dysphagia or pain on swallowing).

Diagnosis

  • The clinical presentation is usually sufficient for diagnosis.
  • Remember to examine the mucosa!
  • Examine temporal association with any drugs (lichenoid drug eruption).
  • The eruption may occur long (several months) after starting the medication.

Differential diagnosis

Workup

  • Histological examination of a skin biopsy is diagnostic but usually unnecessary in patients with lichen planus (the clinical picture being sufficient).
  • Biopsy may be needed in unclear cases to exclude other skin disorders.
  • In the case of mucosal lesions, biopsy may be important for differential diagnosis, particularly if the clinical picture is atypical or erosive. In such cases, immunofluorescence testing is usually also necessary.
  • Mucosal biopsy should be taken, as necessary, to exclude malignancy (leukoplakia, erosive mucosal lesions).
    • Usually in specialized care or, in the case of oral lesions, by a dentist
  • Consider exclusion of hepatitis C (may be the triggering factor): ALT, anti-HCV antibodies.
  • Other tests should be done according to clinical suspicion; for example, serum Treponema pallidum antibodies (to exclude syphilis Syphilis), antinuclear antibodies, extractable nuclear antigen antibodies (cutaneous lupus erythematosus Discoid Lupus Erythematosus).

Treatment

  • An alternative to all modes of treatment is to wait for spontaneous recovery. There is no predictable time course for spontaneous healing. Treatment decisions should be made in agreement with the patient.
  • It is important for the patient to understand that the treatment is symptomatic.

Cutaneous lichen planus

  • Courses of topical treatment with (moderately potent), potent or very potent glucocorticoid ointments (i.e. groups (II), III, IV). A potent (group III) ointment is usually applied first in courses of 2-4 weeks, for instance.
  • Some patients need more long-term maintenance treatment, such as application twice a week in courses of 1-2 months. The response to treatment should be checked.
  • Topical calcineurin inhibitors may also be helpful, such as a tacrolimus 0.1% ointment in courses of 1-2 months.
  • Patients may benefit from taking non-sedating antihistamines. The dose can be increased 2- to 4-fold depending on the response, e.g. one to two 10-mg cetirizine tablets once or twice daily (write the prescription in the locally appropriate manner to verify the higher than usual dosage).
    • Hydroxyzine is a sedating antihistamine that can be used in doses of 25-50 mg before going to bed to alleviate itching. Doses of 25 mg 3-4 times daily can be used, as necessary (unless they cause excessive fatigue).
    • Do not treat pigmented remnants of lichen papules. They return to normal colour spontaneously within several months.
    • The disease recurs in some form in at least 50% of patients.
    • In treatment-resistant or severe cases, phototherapy (such as narrow-band UVB) or various systemic treatments (such as glucocorticoids, acitretin, methotrexate, dapsone, hydroxychloroquine) can be considered in specialized care.

Mucosal lichen planus

  • Often chronic and refractory
  • The treatment of oral mucosal lichen planus is described in a separate article Oral Lichen Planus.
  • The primary treatment of disease of the genital mucosa is (moderately potent), potent or very potent (group (II), III, IV) topical glucocorticoid ointment in courses of 2-4 weeks, for instance. A fatty ointment (unguent) is usually better as it causes less irritation.
  • Calcineurin inhibitor ointments in courses of 2-4 weeks, for instance, are an alternative.
  • Patients often benefit from topical or systemic anticandidal medication (such as oral fluconazole in doses of 150 mg once a week or as defined by weight, in courses of 4-12 weeks, for example).
  • In acute exacerbations, patients usually benefit from short courses of oral glucocorticoids such as prednisolone, firstly in doses of 0.5 mg/kg every morning and gradually decreasing the dose by, for instance, 10-5 mg every 3-5 days, in courses of 2-4 weeks, in total. The risks and adverse effects of glucocorticoids must be considered Pharmacological Glucocorticoid Treatment.
  • In severe cases, various systemic treatments can be given in specialized care.
  • Genital lichen planus in female patients involves a risk of stricture. These are treated in specialized care by dilation, as necessary.

Specialist consultation

  • A dermatologist should be consulted for severe or refractory clinical pictures.
  • A gynaecologist should be consulted in the case of genital mucosal lichen planus, as necessary.
  • Consultation and follow-up by a dentist in association with annual oral and dental checkups, for instance, is recommended for chronic and severe oral mucosal lichen planus.

    References

    • Ioannides D, Vakirlis E, Kemeny L, et al. European S1 guidelines on the management of lichen planus: a cooperation of the European Dermatology Forum with the European Academy of Dermatology and Venereology. J Eur Acad Dermatol Venereol 2020;34(7):1403-1414 [PubMed]
    • Lodi G, Manfredi M, Mercadante V, et al. Interventions for treating oral lichen planus: corticosteroid therapies. Cochrane Database Syst Rev 2020;2(2):CD001168 [PubMed]
    • van der Meijden WI, Boffa MJ, Ter Harmsel WA, et al. 2016 European guideline for the management of vulval conditions. J Eur Acad Dermatol Venereol 2017;31(6):925-941 [PubMed]
    • Atzmony L, Reiter O, Hodak E ym. Treatments for Cutaneous Lichen Planus: A Systematic Review and Meta-Analysis. Am J Clin Dermatol 2016;17(1):11-22. [PubMed]
    • Fazel N. Cutaneous lichen planus: A systematic review of treatments. J Dermatolog Treat 2015;26(3):280-3. [PubMed]
    • Gamil H, Nassar A, Saadawi A et al. Narrow-band ultraviolet B phototherapy in lichen planus. J Eur Acad Dermatol Venereol 2009;23(5):589-90. [PubMed]
    • Pavlotsky F, Nathansohn N, Kriger G et al. Ultraviolet-B treatment for cutaneous lichen planus: our experience with 50 patients. Photodermatol Photoimmunol Photomed 2008;24(2):83-6. [PubMed]