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Background

This is the prototypical lichenoid tissue reaction. It is characterized by violaceous, flat-topped papules, which are usually pruritic. Wickham's striae are on the surface. The lesions occur on flexor surfaces of the wrists, trunk, thighs, oral cavity, and genitalia. Lichen planus has numerous presentations and the most prominent are listed below.

Lichen planus has been associated with many conditions. In long standing lesions, especially in erosive and hypertrophic variants, squamous cell carcinoma may occur.

DISEASE ASSOCIATIONS CHARACTERIZATION
GENERAL Immunodeficiency
Internal malignancies
Primary biliary cirrhosis
Chronic hepatitis C infection
Hepatitis B vaccination
Pemphigus
Ulcerative colitis
Lichen sclerosus et atrophicus
HEPATITIS B VACCINATION  

Lichen planus occurring after hepatitis B vaccination: A new case

Sultan Al-Khenaizan, MBBS, FRCPC

Riyadh, Saudi Arabia

J Am Acad Dermatol 2001;45:614-5 Abstract quote

Lichen planus is a pruritic inflammatory dermatosis of unknown origin. An increased prevalence of a wide range of liver disease in lichen planus has been observed by many authors. Most recently, many reports appeared of the occurrence of lichen planus after administration of different types of hepatitis B vaccines.

We report one case and briefly review this intriguing observation.

RADIATION THERAPY SITE  

Lichen planus confined to a radiation therapy site.

Kim JH, Krivda SJ.

Dermatology Service, Department of Medicine, Walter Reed Army Medical Center.

J Am Acad Dermatol 2002 Apr;46(4):604-5 Abstract quote

We report the case of a 58-year-old man with lichen planus localized to a radiation site. To our knowledge, this is the first reported case of radiation-induced lichen planus in the English-language literature.

 

PATHOGENESIS CHARACTERIZATION
Matrix metalloproetinases in oral lichen planus

J Cutan Pathol 2001;28:72-82

T-cell derived MMP-9 is overexpressed and may cause basement membrane disruption and facilitate intra-epithelial T cell migration

 

GROSS APPEARANCE/CLINICAL VARIANTS CHARACTERIZATION
SKIN VARIANTS  
Atrophic
May resemble porokeratosis
Erythema dyschromicum perstans
Also known as Ashy dermatosis and lichen planus pigmentosus
Prevalent in Latin America
Hypertrophic
Usually on the shins
Linear

Clin Exp Dermatol 1993;18:335-337

Rare
0.24-0.62% of all children with lichen planus
More common in children
May follow lines of Blaschko

Lichen planus erythematosus
Non-pruritic red papules occurring on the forearms
Keratosis lichenoides chronica
Lesions in linear and reticulate pattern on the extremities with a seborrheic-dermatitis like facial eruption
May be associated with glomerulonephritis and lymphoma
Lichen planus actinicus
Also known as lichen planus tropicus, lichen planus subtropicus, lichenoid melanodermatitis, and summertime actinic lichenoid eruption. Limited to sun-exposed areas
Lichen planopilaris

Follicular lichen planus associated with scarring alopecia

Graham Little-Piccardi-Lassueur syndrome associated with follicular keratotic lesions and alopecia

Lichen planus follicularis tumidus has plaques with follicular papules in the retroauricular region

Lichen planus pemphigoides

Histopathology 1991;19:147-154
Br J Dermatol 1998;138:972-980
J Invest Dermatol 1999;113:117-121

Overlap with lichen planus and bullous pemphigoid

Blister formation usually follows the appearance of papules and plaques associated with LP. These lesions occur on LP lesions or on unaffected skin-this is in contrast to LP which has blisters confined to LP lesions

Blisters preferential on distal extremities

Younger patients-average age 44 years
Less severe course than bullous pemphigoid

Autoantibodies against:
BP180
BP 230
200kd protein

BP180 NC16A4(MCW-4)-this is an epitope not recognized by antibodies in BP

Lichen planus pemphigoides is a heterogeneous disease: a report of five cases studied by immunoelectron microscopy.

Bouloc A, Vignon-Pennamen MD, Caux F, Teillac D, Wechsler J, Heller M, Lebbe C, Flageul B, Morel P, Dubertret L, Prost C.

Department of Dermatology, Hopital Saint Louis, Paris, France.

Br J Dermatol 1998 Jun;138(6):972-80 Abstract quote

Lichen planus pemphigoides (LPP) is a rare and controversial disease. It is characterized by bullae arising on lichen planus papules and on uninvolved skin, subepidermal bullae in histology, and linear deposits of IgG and C3 along the basal membrane zone on immunofluorescence of peribullous skin.

Our goal was to identify the localization of the target antigen in cases of LPP. Five patients diagnosed with LPP on clinical, histological and immunofluorescence criteria were explored by immunoelectron microscopy and immunoblot. Our results show that the target antigen in LPP is not unique. The localization of the immune deposits was consistent with a diagnosis of bullous pemphigoid in two cases, of cicatricial pemphigoid in two cases and of epidermolysis bullosa acquisita in one case.

Our study supports the view that LPP is a heterogeneous condition in which lichen planus may induce different subepidermal acquired bullous dermatoses.

Lupus erythematosus-lichen planus overlap syndrome
Overlap syndrome
Lupus is usually chronic discoid type or systemic type
Ulcerative or erosive
Oral cavity, vulva, penis
Rarely associated with Castleman's disease and lymphoma
EXTRA-CUTANEOUS VARIANTS  
ESOPHAGUS
Am J Surg Pathol 2000;24:1678-1682
Presented with skin and oral lesions
Had dysphagia with esophageal stricture
ORAL
Oral lesions coexist with skin lesions in 30-50% of cases

The clinical features, malignant potential, and systemic associations of oral lichen planus: A study of 723 patients

Drore Eisen, MD, DDS

Cincinnati, Ohio

J Am Acad Dermatol 2002;46:207-14 Abstract quote

Background: Although oral lichen planus (OLP) is a relatively common disorder, reports comprising large numbers of patients with the disease are lacking in the dermatology literature.

Objective and Methods: The purpose of this investigation was to describe the clinical characteristics of 723 patients with biopsy-proven OLP who were followed up from 6 months to 8 years (mean, 4.5 years).

Results: Of the 723 patients, 75% were women and 25% men. The erosive form of the disease was the predominant type in 40% of patients at initial presentation, and symptoms were present in the majority of patients with all forms of the disease. Isolated gingival lichen planus was observed in 8.6% of patients. Precipitating factors that resulted in an exacerbation of the disease were frequently noted and included stress, foods, dental procedures, systemic illness, and poor oral hygiene. In 195 patients prospectively screened, no liver abnormalities or antibodies to hepatitis B or C were detected. Oral squamous cell carcinoma developed in 6 patients (0.8%) at sites previously diagnosed by clinical examination as erosive or erythematous lichen planus.

Conclusions: Patients with OLP usually display lesions with distinctive clinical morphology and characteristic distribution but may also present with a confusing array of forms and patterns mimicking other diseases. Because patients with OLP may be at an increased risk for the development of squamous cell carcinoma, periodic follow-up is mandatory to detect malignant transformation. Routine screening of American patients with OLP for hepatitis C and other liver abnormalities does not appear to be warranted as in Italian and Japanese patients with OLP.

 

HISTOLOGICAL TYPES CHARACTERIZATION
GENERAL

The characteristic histology is a lichenoid interface dermatitis with Civatte bodies and variable melanin incontinence

There is hyperkeratosis with a wedge shaped hypergranulosis

Occasionally Max-Joseph spaces may occur

VARIANTS  
LICHEN PLANOPILARIS  

Lichen planopilaris: a clinicopathologic study.

Matta M, Kibbi AG, Khattar J, Salman SM, Zaynoun ST.

Department of Dermatology, American University of Beirut Medical Center, Lebanon.

J Am Acad Dermatol 1990 Apr;22(4):594-8 Abstract quote

Three clinicopathologic variants of lichen planopilaris are described.

The first is characterized clinically by individual keratotic follicular papules and histologically by a lichenoid inflammatory cell infiltrate confined to the follicular epithelium.

The second variant consists of erythematous to violaceous plaques, some of which show follicular prominence; the histologic appearance is that of a lichenoid inflammatory cell infiltrate that affects both follicular and interfollicular areas.

The third variant manifests as follicular papules of the scalp with concomitant or subsequent cicatricial alopecia. In this variant the histologic hallmark is a lichenoid, follicular and interfollicular inflammation, associated with or followed by scarring.

Overlap among the three variants exists, and hence the concept of a disease spectrum ranging from pure follicular involvement without evidence of clinical scarring to cicatricial alopecia of the scalp is advocated.

Lichen planopilaris: clinical and pathologic study of forty-five patients.

Mehregan DA, Van Hale HM, Muller SA.

Department of Dermatology, Mayo Clinic, Rochester, MN 55905.

J Am Acad Dermatol 1992 Dec;27(6 Pt 1):935-42 Abstract quote

BACKGROUND: We review the findings in a large series of patients with lichen planopilaris.

OBJECTIVE: Clinical, histologic, and direct immunofluorescence findings were reviewed in 45 patients.

METHODS: Scalp biopsy specimens for routine histologic examination and direct immunofluorescence were reviewed. Clinical data and follow-up were obtained.

RESULTS: Women were affected more commonly and had patchy hair loss, with perifollicular erythema, perifollicular spines, and scarring. Half had or developed glabrous skin, mucous membrane, or nail changes typical of lichen planus. Follicular involvement was limited to the infundibulum and isthmus and included lichenoid inflammation and cytoid formation, with few or no changes in interfollicular epidermis. Direct immunofluorescence showed cytoid body staining with anti-IgM and anti-IgA and patchy or linear fibrinogen deposition along the basement membrane zone. The various therapeutic options used were usually unsuccessful.

CONCLUSION: To make the correct diagnosis, patients with scarring alopecia should be evaluated histologically and with direct immunofluorescence. They should also be followed up to assess whether lichen planus develops elsewhere.

 

SPECIAL STAINS/IMMUNOPEROXIDASE/OTHER CHARACTERIZATION
Direct immunofluorescence (DIF) Colloid bodies show staining for IgM, complement, and some immunoglobulins.

 

PROGNOSIS AND TREATMENT CHARACTERIZATION
TREATMENT Topical occlusive steroids
LICHEN PLANOPILARIS  

Graham Little-Piccardi-Lassueur syndrome: effective treatment with cyclosporin A.

Bianchi L, Paro Vidolin A, Piemonte P, Carboni I, Chimenti S.

Institute of Dermatology, Tor Vergata University of Rome, Italy.

Clin Exp Dermatol 2001 Sep;26(6):518-20 Abstract quote

Graham Little-Piccardi-Lassueur syndrome (GLPLS) is a rare lichenoid dermatosis defined by scarring alopecia, loss of pubic and axillary hairs and progressive development of horny follicular papules variously located. Topical or systemic corticosteroids, retinoids or PUVA therapy are the treatments usually proposed and these have partial and temporary benefits.

We describe the effectiveness of cyclosporin A in a case of GLPLS at the dosage of 4 mg/kg/day. At the end of treatment, substantial reduction of both perifollicular erythema and follicular hyperkeratotic papules was observed. After 3 months of follow-up, besides the results already obtained, a few areas of hair regrowth in the scarring patches and a more consistent improvement of the follicular papules were detected.

We believe that cyclosporin A could be effective mainly in the initial phases of this rare variant of lichen planopilaris, before the development of severe follicle damage, either by interfering with the acute inflammatory processes or by limiting the progression of the disease.

To the best of our knowledge, this is the first report showing a good and persistent therapeutic effect of cyclosporin A in GLPLS.

NAIL  

Nail Lichen Planus in Children Clinical Features, Response to Treatment, and Long-term Follow-up

Antonella Tosti, MD; Bianca Maria Piraccini, MD; Stefano Cambiaghi, MD; Matilde Jorizzo, MD

Arch Dermatol. 2001;137:1027-1032 Abstract quote

Objective To report clinical features, response to treatment, and long-term follow-up of nail lichen planus in children.

Design Retrospective study involving 15 children with nail lichen planus.

Setting Outpatient consultation for nail disorders at the Department of Dermatology of the University of Bologna, Bologna, Italy. Patients or Other Participants We diagnosed nail lichen planus in 15 children younger than 12 years, including 10 children with typical nail matrix lesions, 2 children with 20-nail dystrophy (trachyonychia), and 3 children with idiopathic atrophy of the nails. Only 2 of the 15 children had oral lichen planus; none had cutaneous lesions. A nail biopsy confirmed the diagnosis in all cases. Intervention Intramuscular triamcinolone acetonide, 0.5 to 1 mg/kg per month, was prescribed to children with typical nail lichen planus and prolonged from 3 to 6 months until the proximal half of the nail was normal. No treatment was prescribed to patients with 20-nail dystrophy or idiopathic atrophy of the nails.

Results Treatment with systemic corticosteroids was effective in curing typical nail lichen planus. Two children experienced a recurrence of the disease during the follow-up. Recurrences were always responsive to therapy. The 2 children with 20-nail dystrophy improved without any therapy. Nail lesions caused by idiopathic atrophy of the nails remained unchanged during the follow-up period.

Conclusions Nail lichen planus in children is not rare but probably underestimated. It often presents with atypical clinical features such as 20-nail dystrophy or idiopathic atrophy of the nails.

ORAL  

Topical tacrolimus in the treatment of symptomatic oral lichen planus: A series of 13 patients

Todd W. Rozycki, MD
Roy S. Rogers III, MD
Mark R. Pittelkow, MD
Marian T. McEvoy, MD
Rokea A. el-Azhary, MD, PhD
Alison J. Bruce, MD
Joseph P. Fiore, MD
Mark D. P. Davis, MD

Rochester, Minnesota

J Am Acad Dermatol 2002;46:27-34. Abstract quote

Background: Oral lichen planus (OLP) is a relatively common, chronic inflammatory condition, which frequently presents with symptoms of pain and irritation. OLP is often difficult to manage. Therefore there is a need for more effective and safer therapies for symptomatic OLP.

Objective: Our purpose was to determine the effectiveness of topical tacrolimus as therapy for symptomatic OLP.

Methods: A retrospective review was performed of 13 patients with symptomatic OLP treated with topical tacrolimus between September 1999 and September 2000. Results: Eleven of the 13 patients reported definite symptomatic response to treatment and 2 had no response. Eight patients had a partial response, whereas 3 patients had a complete response with respect to lesion clearance. Seven of the responding patients had no flares with continued treatment. The other 4 patients noted flares soon after stopping the treatment. Side effects were rare and minor.

Conclusions: In this retrospective case series of 13 patients, topical tacrolimus was well tolerated and appeared to be an effective therapy to control symptoms and clear lesions of OLP.

Management of recalcitrant ulcerative oral lichen planus with topical tacrolimus

F. Kaliakatsou, BDS, MSca
T. A. Hodgson, FDS RCS, MRCP(UK)a
J. D. Lewsey, PhD
A. M. Hegarty, MSc, MFDS RCSIa
A. G. Murphy, MSc, B Pharm, MRPharmSc
S. R. Porter, MD, PhD, FDS RCSEa

London, United Kingdom

J Am Acad Dermatol 2002;46:35-41. Abstract quote

Objective: Our purpose was to investigate the efficacy and safety of 0.1% topical tacrolimus in erosive or ulcerative oral lichen planus.

Methods: This was an open-label, noncomparative study conducted in an outpatient oral medicine unit in London, United Kingdom. The study covered an 8-week period with a 22-week follow-up after cessation of therapy. Nineteen patients, aged 28 to 87 years with biopsy-proven oral lichen planus refractory to, or dependent on, systemic immunosuppressive agents, were enrolled. Seventeen patients (89%) completed the study. Application of 0.1% tacrolimus was administered to all symptomatic oral mucosal lesions. Clinical review took place 1, 3, 5, 7, and 8 weeks after commencing therapy. Alleviation of symptoms was evaluated by using a visual analogue scale as well as the McGill Pain and Oral Health Impact profile questionnaires. The extent of the oral mucosal erosion or ulceration was directly measured by the same clinician at all visits. Safety assessments included monitoring of adverse events, complete blood cell count, renal and hepatic clinical chemistry, and tacrolimus blood concentrations.

Results: Tacrolimus caused a statistically significant improvement in symptoms within 1 week of commencement of therapy. A mean decrease of 73.3% occurred in the area of ulceration over the 8-week study period. Local irritation (in 6 subjects, 35%) was the most commonly reported adverse effect. Laboratory values showed no significant changes with time. Therapeutic levels of tacrolimus were demonstrated in 8 subjects but were unrelated to the extent of oral mucosal involvement. Thirteen of 17 patients suffered a relapse of oral lichen planus within 2 to 15 weeks of cessation of tacrolimus therapy.

Conclusion: Topical tacrolimus is effective therapy for erosive or ulcerative oral lichen planus.

SKIN  

Low-dose ultraviolet A1 phototherapy for extragenital lichen sclerosus: Results of a preliminary study

Alexander Kreuter, MD
Thilo Gambichler, MD
Annelies Avermaete, MD, etal.

Bochum, Germany

J Am Acad Dermatol 2002;46:251-5 Abstract quote

Background: Lichen sclerosus (LS) is a chronic inflammatory skin disease in which numerous therapies have been used, with only limited success. Because low-dose UVA1 phototherapy has been shown to be an effective treatment option for localized scleroderma, which shares several similar clinical and histologic features with LS, we initiated a clinical trial with this phototherapeutic modality in patients with LS.

Methods: Ten patients suffering from extragenital LS were treated with low-dose UVA1 phototherapy 4 times weekly with single UVA1 doses of 20 J/cm2. Forty treatment sessions were performed within 10 weeks, resulting in a cumulative UVA1 dose of 800 J/cm2.

Results: Low-dose UVA1 phototherapy resulted in a marked reduction of the clinical score and a significant (P < .05) decrease of ultrasonographically measured skin thickness as well as a highly significant (P < .001) increase of dermal density. The patients reported a remarkable softening and repigmentation of the affected skin.

Conclusion: Analogous to the treatment results in localized scleroderma, low-dose UVA1 phototherapy seems to be an effective and well-tolerated treatment option for extragenital LS.

Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.


Commonly Used Terms

Caspary-Joseph spaces (Max-Joseph spaces)-Small clefts which form at the dermoepidermal junction secondary to the lichenoid inflammatory cell infiltrate.

Civatte bodies-Apoptotic keratinocytes.

Colloid bodies-Apoptotic bodies extruded into the papillary dermis.

Graham Little-Piccardi-Lassueur syndrome-Lichen planopilaris ssociated with follicular keratotic lesions and alopecia

Interface dermatitis-Inflammatory cell infiltrate obscures the dermoepidermal junction, sometimes used synonymously as lichenoid dermatitis.

Vacuolar change-Liquefaction degeneration of the basal epithelial cells.

Wickham's striae-Fine white lines seen on the surface of the skin papules


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