Última actualización:
September 10, 2018
Años publicados: 1986, 1989, 1990, 1996, 1999, 2007, 2018
NORD gratefully acknowledges Addie Langner, MD Candidate, University of Utah School of Medicine, and Christopher M. Hull, MD, Department of Dermatology University of Utah, for assistance in the preparation of this report.
Lichen planus (LP) is a rare, chronic, inflammatory autoimmune skin and mucous membrane disease. LP most commonly presents as itchy, shiny, reddish-purple spots (lesions) on the skin (cutaneous LP) or as white-gray lesions in the mouth or on the lips (oral LP). Less commonly, LP may also involve the genitals (penile or vulvar LP), scalp (lichen planopilaris), ears (otic LP), nails, eyes, and esophagus. Similar to lichen found growing on trees and rocks in forests, the skin lesions are often flat-topped and can be somewhat scaly, hence the name “lichen” planus.
weeks or months and intermittent recurrences may occur for years. The appearance of the lesions is dependent on their location. In cutaneous LP, the lesions can present anywhere on the skin, usually on the wrists, legs, palms and soles or torso and are 2 to 4 mm in diameter with angular borders, a violet color and a distinct sheen in cross-lighting. These lesions tend to be symmetrically distributed and may also coalesce into rough scaly patches. Rarely, blisters may develop. Moderate to severe itching is common and frequently fails to respond to treatment.
There are several variants of cutaneous LP, which may present differently. The lesions may become large, scaly and warty (hypertrophic LP), particularly on the lower legs. New spots may appear along a site of minor skin injury such as a superficial scratch (Koebner’s phenomenon). Sometimes degeneration (atrophy) of the skin may occur as lesions persist (atrophic LP) and some patients experience an absence of sweating due to degeneration of sweat glands (anhidrosis).
In areas where lesions have healed, an unusual darkening (hyperpigmentation) or lightening (hypopigmentation) of the skin may occur.
Between 50 and 70 percent of patients show symptoms involving “mucous membranes”, the moist pink skin that lines the inside of the mouth, vagina, and esophagus. LP on mucous membranes can present as red, painful sores, or lesions that have a net-like, white pattern. Oral symptoms often occur before skin lesions develop. Oral symptoms, consisting of a dryness and metallic taste or burning in the mouth, may appear first and may be the only evidence of the disease.
Although not common, hair loss may be among the consequences of LP, which is called lichen planopilaris. When and if hair loss does occur, it can involve small patchy areas of the scalp (atrophic cictrical alopecia) or cause a receding hairline (frontal fibrosing alopecia). Due to scarring, this hair loss is permanent if untreated. For more information on lichen planopilaris, visit https://rarediseases.info.nih.gov/diseases/3247/lichen-planopilaris.
Nail LP presents in 10 to 25 percent of LP patients and tends to present as roughness, vertical ridges or cracks, and thinning of the nail. This can eventually lead to scarring of the nail.
Trouble swallowing, or pain with swallowing can indicate esophageal LP. It is important to treat esophageal disease, as it can eventually lead to the esophagus narrowing over time, called esophageal stricture.
Some cases of cutaneous LP will resolve with time, while oral, genital, nail, and esophageal LP are more persistent and may increase in severity over time. Patients with LP are at an increased risk of squamous cell carcinoma, particularly of the oral mucosa, and need to be monitored periodically.
In most affected individuals, the exact cause of LP is unclear. It is suspected that exposure to infections, drugs, allergens, or injury may sensitize the immune system and cause the immune system to attack skin cells. This initial eruption may persist for weeks to months, and recurrences can continue throughout the individual’s lifetime. There have been reports of LP in family members, indicating that there may be a genetic predisposition, but the genetic factors of LP are still being researched and are uncertain.
There is limited data on how many people are affected by LP, but most studies estimate that LP occurs in less than 1 percent of the world’s population. Cutaneous LP occurs at similar frequencies in men and women, but women are somewhat more likely to develop oral LP or lichen planopilaris. There does not appear to be a racial predisposition for the disease. The majority of LP develops between 30 and 60 years of age but can affect older and younger individuals as well. In rare cases, children may be affected.
A diagnosis of LP can often be made from an examination of the skin or mucous membranes and identification of the characteristic clinical features.
Clinical Testing and Work-Up
If a diagnosis is not clear based on clinical findings, it is common for the physician to take a small sample of skin (biopsy) to confirm the diagnosis.
There is some evidence of an association between LP (especially with oral LP) and hepatitis C virus infection and blood testing may be ordered by your physician.
In cases where an allergy is suspected, a type of allergy test called patch testing may be helpful to identify the cause.
Treatment
In mild cases, symptoms may be minimal or absent and no therapy may be needed.
For patients requiring treatment, first line therapy is usually a topical corticosteroid medication. These are available in many strengths and formulations including cream, ointment, gels, solutions, oral rinses and others. If topical corticosteroids are not effective or cause side effects, a non-steroid topical medication called tacrolimus or pimecrolimus may be prescribed.
Erosive oral lesions and widespread itchy skin lesions often require the use of a systemic corticosteroid (e.g., oral prednisone). Unfortunately, skin lesions may return after systemic prednisone has been discontinued. In this case, continued low dosage of a systemic corticosteroid may be instituted.
Phototherapy may be helpful for widespread skin disease.
In more severe refractory cases, stronger immune suppressing medications may be needed such as mycophenolate mofetil, methotrexate, azathioprine, cyclosporine, and others.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.
For more information about clinical trials being conducted at the National Institutes of Health (NIH) Clinical Center in Bethesda, MD, contact the NIH Patient Recruiting Office:
Tollfree: (800) 411-1222
TTY: (866) 411- 1010
Email: [email protected]
Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/
For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com
For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/
JOURNAL ARTICLES
Heelan, K., McAleer, M. A., Roche, L., McCreary, C., & Murphy, M. Intractable erosive lichen planus treated successfully with rituximab. British Journal of Dermatology. 2015;172(2):538-540.
Davari P, Hsiao HH, & Fazel N. Mucosal lichen planus: an evidence-based treatment update. American Jurnal of Clinical Dermatology. 2014;15(3):181-195.
Gorouhi F, Davari P, & Fazel N. Cutaneous and mucosal lichen planus: a comprehensive review of clinical subtypes, risk factors, diagnosis, and prognosis. The Scientific World Journal. Volume 2014, Article ID 742826.
Manousaridis I, Manousaridis K, Peitsch WK & Schneider S W. Individualizing treatment and choice of medication in lichen planus: a step by step approach. JDDG: Journal der Deutschen Dermatologischen Gesellschaft. 2013;11(10): 981-991.
Wagner G, Rose C, & Sachse MM.Clinical variants of lichen planus. JDDG: Journal der Deutschen Dermatologischen Gesellschaft 2013;11(4): 309-319.
Le Cleach L, & Chosidow O. Lichen Planus. New England Journal of Medicine. 2012; 366(8):723-732.
Wee JS, Shirlaw PJ, Challacombe SJ, & Setterfield JF. Efficacy of mycophenolate mofetil in severe mucocutaneous lichen planus: a retrospective review of 10 patients. British Journal of Dermatology 2012;167(1): 36-43.
Torti D C, Jorizzo J L, & McCarty MA. Oral lichen planus: a case series with emphasis on therapy. Archives of dermatology. 2007;143(4): 511-515.
Verma KK, Mittal R, & Manchanda Y. Azathioprine for the treatment of severe erosive oral and generalized lichen planus. ACTA DERMATOVENEREOLOGICA-STOCKHOLM. 2001; 81(5): 378-378.
Roy K, et al., Hepatitis C virus and oral disease: a critical review. Oral Dis. 1999; 5:270-77.
Lear J. & English JSC. Erosive and generalized lichen planus responsive to azathioprine. Clinical and experimental dermatology. 1996; 21(1): 56-57.
INTERNET
Goldstein BG, Goldstein AO Mostow E. Lichen Planus. Dellavalle, RP, Callen J (Ed.), UpToDate. topic last updated: Jun 27, 2017. https://www.uptodate.com/contents/lichen-planus Accessed September 10, 2018.
Mirowski GW, Schlosser BJ. Oral lichen planus: Pathogenesis, clinical features, and diagnosis. Dellavale RP (Ed.). UpToDate. topic last updated: Jul 11, 2017. https://www.uptodate.com/contents/oral-lichen-planus-pathogenesis-clinical-features-and-diagnosis Accessed June 25, 2018.
Shapiro J. Lichen planopilaris. Ofori AO (Ed.), UpToDate. topic last updated: Nov 27, 2017. https://www.uptodate.com/contents/lichen-planopilaris Accessed June 25, 2018.
Ziemer M. Lichenoid drug eruption (drug-induced lichen planus). Corona, R. (Ed.), UpToDate. topic last updated: Jan 09, 2018. https://www.uptodate.com/contents/lichenoid-drug-eruption-drug-induced-lichen-planus
Accessed September 10, 2018 June 25, 2018.
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