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Introduction

Lichen planus (LP) is an inflammatory, often chronic disease with no known cause. It appears as pruritic, violaceous papules and plaques most commonly found on the wrists, lower back, and ankles. LP may also involve the mucous membranes, genitalia, nails, or scalp, and several areas can be affected, either concomitantly or sequentially.1-4
 

Pathophysiology

  • LP is an idiopathic disease and both its etiology and pathogenesis are not fully understood; however, it appears to represent a T-cell-mediated autoimmune disease. Evidence to support this theory includes the identification of immunoglobulins at the dermal-epidermal junction in 95% of lesions, the observation that certain drug reactions can mimic LP, and the occurrence of LP-like eruptions in patients experiencing a graft-versus-host reaction.1-3
  • Various agents have been associated with the development of LP, particularly viruses, with the hepatitis C virus receiving the most recognition. Patients with LP are five times as likely to test positive for hepatitis C as the general population, but there is no known explanation for this association.1,2

Related Anatomy

  • Dermis
  • Epidermis
  • Basal keratinocytes
  • T-cells
  • Antigens

Incidence and Related Conditions

  • LP has been found to occur in 0.1-4% of the general population.5Cutaneous LP has a worldwide prevalence of ~0.2-1%, while the prevalence of oral LP is ~1-4%.1
  • LP can appear at any age, but most cases occur between 30-60 years. Children represent <5% of LP patients.1,2
  • Women, especially perimenopausal women, are affected more frequently than men at a ratio of 1.5:1.1,4
  • Ulcerative colitis
  • Alopecia areata
  • Dermatomyositis
  • Hepatitis C
  • Lichen sclerosis
  • Morphea
  • Myasthenia gravis
  • Vitiligo

Differential Diagnosis

  • Eczema 
  • Graft-versus-host disease
  • Guttate psoriasis
  • Lichen nitidus
  • Lichen simplex chronicus
  • Syphilis
  • Pityriasis rosea
  • Prurigo nodularis
  • Psoriasis
ICD-10 Codes
  • SKIN - COMMON HAND RASHES: LICHEN PLANUS

    Diagnostic Guide Name

    SKIN - COMMON HAND RASHES: LICHEN PLANUS

    ICD 10 Diagnosis, Single Code, Left Code, Right Code and Bilateral Code

    DIAGNOSISSINGLE CODE ONLYLEFTRIGHTBILATERAL (If Available)
    LICHEN PLANUSL43.9   

    ICD-10 Reference

    Reproduced from the International statistical classification of diseases and related health problems, 10th revision, Fifth edition, 2016. Geneva, World Health Organization, 2016 https://apps.who.int/iris/handle/10665/246208

Clinical Presentation Photos and Related Diagrams
Lichen Planus of the Hand
  • Lichen planus of the dorsum of the right hand.
    Lichen planus of the dorsum of the right hand.
Symptoms
Pruritic, purple appearing lesion that often appear on the flexor surface of forearm and wrist
Typical History

The typical patient is a 55-year-old woman who has hepatitis C. Over the course of several days, she began noticing plaques and papules appearing on the skin over the palmar side of her right wrist and both ankles. These lesions were purple in color and polygonal in shape, and became itchy soon after erupting. Their surface also featured a fine reticular pattern of white dots and lines, which resulted from scratching. This onset of symptoms led the woman to seek out care from a dermatologist. 

Positive Tests, Exams or Signs
Work-up Options
Treatment Options
Conservative

Conservative1,2,4

  • Treatment choices depend on the location and severity of the lesions and are guided largely by clinical experience, but the primary objective in most cases is to reduce pruritus and the time to resolution.1,4
  • Topical corticosteroids
    • First-line therapy
    • Example: clobetasol propionate 0.05% twice daily for 2-4 weeks
    • Intralesional steroid injections 
    • Oral metronidazole
    • Narrow-band ultraviolet B radiation (NBUVB)
    • Topical calcineurin inhibitors
    • Methotrexate
    • Sulfasalazine
    • Isotretinoin 
    • Acitretin 
    • Griseofulvin
Operative
  • Surgery may be required when adhesions form in erosive anogenital LP, but should be deferred until active lesions are no longer present to avoid complications. Surgery does not appear to be indicated for non-genital cutaneous LP.4
CPT Codes for Treatment Options

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CPT Code References

The American Medical Association (AMA) and Hand Surgery Resource, LLC have entered into a royalty free agreement which allows Hand Surgery Resource to provide our users with 75 commonly used hand surgery related CPT Codes for educational promises. For procedures associated with this Diagnostic Guide the CPT Codes are provided above. Reference materials for these codes is provided below. If the CPT Codes for the for the procedures associated with this Diagnostic Guide are not listed, then Hand Surgery Resource recommends using the references below to identify the proper CPT Codes.

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CPT 2021 Professional Edition: Spiralbound

Complications
  • Post-inflammatory hyperpigmentation
  • Nail loss
  • Candidiasis
Outcomes
  • Cutaneous LP usually resolves spontaneously within 1-2 years, but many patients experience residual hyperpigmentation.1
  • A systematic review and meta-analysis found several interventions to be effective for treating cutaneous LP, including systemic treatments with acitretin, sulfasalazine, and griseofulvin, NBUVB, prednisolone, methotrexate, and oral PUVA photochemotherapy.6
Key Educational Points
  • The LP rash is primarily distinguished by the “six Ps,” which often appear on the flexor surface of the forearm and wrist:
    • 1. Purple
    • 2. Palmar wrist
    • 3. Pruritus
    • 4. Polygonal
    • 5. Papules
    • 6. Plaques 
  • Lichen planus demonstrates the Koebner phenomenon, where trauma can induce the formation of new lesions
  • LP can also cause painful erosions and wickham striae.
  • The clinician should evaluate the entire skin closely and look for the “six Ps.” Although lesions are most common on the skin of the wrists, lower back, and ankles, any area may be affected, including the palms, soles, and genitalia.2,3
  • Biopsy of the lesion may be needed for atypical cases or if a clear diagnosis has not been reached. 
    • The histopathologic features that distinguish LP are the presence of irregular acanthosis and colloid bodies in the epidermis with destruction of the basal layer.2,3
  • Immunofluorescence study of the lesion will reveal globular deposits of immunoglobulin M and complement mixed with apoptotic keratinocytes.
  • If the nail is involved in LP, it should be considered an emergency that requires rapid and aggressive treatment to prevent permanent nail loss, which can be catastrophic for patients.7
References

New and Cited Articles

  1. Arnold DL, Krishnamurthy K. Lichen Planus. In: StatPearls.Treasure Island (FL) 2019. PMID: 30252382
  2. Usatine RP, Tinitigan M. Diagnosis and treatment of lichen planus. Am Fam Physician 2011;84(1):53-60. PMID: 21766756
  3. Marks JG, Miller JJ. Lookingbill and Marks’ Principles of Dermatology. Fifth Ed. London, New York: Saunders Elsevier; 2013.
  4. Le Cleach L, Chosidow O. Clinical practice. Lichen planus. N Engl J Med 2012;366(8):723-732.PMID: 22356325
  5. Zakrzewska JM, Chan ES, Thornhill MH. A systematic review of placebo-controlled randomized clinical trials of treatments used in oral lichen planus. Br J Dermatol 2005;153(2):336-341. PMID: 16086745
  6. Atzmony L, Reiter O, Hodak E, Gdalevich M, Mimouni D. Treatments for Cutaneous Lichen Planus: A Systematic Review and Meta-Analysis. Am J Clin Dermatol 2016;17(1):11-22.PMID: 26507510
  7. Lipner SR. Nail lichen planus: A true nail emergency. J Am Acad Dermato 2019. [Epub]. PMID: 30682392
  8. James WD, Berger TG, Elston DM. Andrews’ Diseases of the Skin.12thEd. Philadelphia, PA. Elsevier, 2016.

Reviews

  1. Atzmony L, Reiter O, Hodak E, Gdalevich M, Mimouni D. Treatments for Cutaneous Lichen Planus: A Systematic Review and Meta-Analysis. Am J Clin Dermatol 2016;17(1):11-22.PMID: 26507510
  2. Fazel N. Cutaneous lichen planus: A systematic review of treatments. J Dermatolog Treat2015;26(3):280-3. PMID: 24916211
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