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 Pathogenesis Laboratory/Radiologic/Other Diagnostic Testing Gross Appearance and Clinical Variants Histopathological Features and Variants Special Stains/
Differential Diagnosis Prognosis Treatment Commonly Used Terms Internet Links
DISEASE ASSOCIATIONS CHARACTERIZATION GENERAL Immunodeficiency
Primary biliary cirrhosis
Chronic hepatitis C infection
Hepatitis B vaccination
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.
Lichen planus in children: a possible complication of hepatitis B vaccines.
Limas C, Limas CJ.
Department of Dermatopathology, "Andreas Sygros" Hospital, Athens, Greece
Pediatr Dermatol 2002 May-Jun;19(3):204-9 Abstract quote
Lichen planus (LP) has been reported as a complication of hepatitis B vaccination in both adults and children. According to published observations, an autoimmune reaction may be triggered by the viral S epitope. In children, LP is uncommon and, because of its atypical clinical presentation, definitive diagnosis may require biopsy.
We investigated the possible association of recombinant hepatitis B virus (HBV) vaccines with childhood LP or LP-like eruptions seen in our hospital over the last 3 years. Only biopsy-confirmed cases in which the clinical history could be thoroughly scrutinized were included. We report five patients less than 16 years of age in whom such an association could be supported by relevant data. Thirteen similar pediatric and 15 adult cases have been reported from various countries in the last 5 years.
The data indicate that LP is a complication that rarely occurs in children receiving the HBV vaccine. It appears without known predisposing factors and has variable clinical presentations while the histologic findings are consistent and, with minor variations, typical of LP.
Lichen planus associated with hepatitis C virus: No viral transcripts are found in the lichen planus, and effective therapy for hepatitis C virus does not clear lichen planus.
Harden D, Skelton H, Smith KJ.
J Am Acad Dermatol. 2003 Nov;49(5):847-52. Abstract quote
BACKGROUND: Although hepatitis C virus (HCV) was not discovered until 1989, it was recognized for many years that a viral agent was responsible for many cases of posttransfusion or parenterally transmitted hepatitis. Acute HCV is often relatively mild; however, 70% to 80% of patients with HCV go on to develop chronic liver disease during a prolonged period of up to 40 years, and up to 50% of them may remain relatively asymptomatic during that time. A number of associated cutaneous findings have been reported in up to 15% of these patients including lichen planus-like eruptions (LP).
OBJECTIVE: We sought to determine whether viral transcripts were present within the skin of patients with HCV and LP, and if systemic virologic response to interferon alfa and ribavirin correlated with response of the LP.Materials and methods A total of 4 men and 1 woman all presented with cutaneous eruptions of LP, and 1 had oral LP lesions. Cutaneous biopsies were performed on all patients. All patients were found to have chronic HCV. In addition to pathologic examination, immunohistochemical stains for lymphoid markers and reverse-transcriptase polymerase chain reaction for HCV was performed on the biopsy specimens. All patients were treated with interferon alfa and ribavirin.
RESULTS: In LP there were scattered eosinophils seen in biopsy specimens of 4 of the 5 patients. The lymphoid infiltrate contained predominantly CD3(+) T cells and scattered KP-1(+) mononuclear cells, without CD20(+) B cells. Approximately one fourth of the T cells failed to mark with CD4. Although all patients were seropositive for HCV RNA at the time of biopsy, we were unable to detect HCV RNA by reverse-transcription polymerase chain reaction in any of the patients. The patients' LP showed an inconsistent response to therapy.
CONCLUSION: The virus was not found in the LP lesion using reverse-transcription polymerase chain reaction for HCV. Thus, LP appears to be related to the pattern of immune dysregulation induced by HCV, probably in a host with an underlying susceptibility for autoimmune disease. The combination of interferon alfa and ribavirin may be effective in clearing the virus, but viral response did not correlate with clearing of LP.
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 APOPTOSIS Cytotoxic Molecule Expression and Epithelial Cell Apoptosis in Oral and Cutaneous Lichen Planus
Amerigo Santoro, MD, etal.
Am J Clin Patholl 2004;121:758-764 Abstract quote
We evaluated the expression of T cell–restricted intracellular antigen (Tia-1), granzyme B, and perforin by lymphocytes and the degree of epithelial apoptosis in oral and cutaneous lichen planus (LP) in 51 untreated cases, including 27 oral LP (OLP) and 24 cutaneous LP (CLP) cases.
The number of total dermal-positive lymphocytes in OLP and CLP was similar, indicating similar activity of the inflammatory process. Intraepithelial Tia-1–positive, perforin-positive, and granzyme B–positive lymphoid cells were more numerous in OLP than in CLP (P < .05). The epithelial cell apoptotic index (AI) was increased significantly in OLP (P < .05), particularly in erosive-atrophic variants. A linear correlation between AI and the mean ± SEM number of intraepithelial and dermal perforin+ cells (6.85 ± 2.44 and 27.48 ± 10.19, respectively), per 10 high-power fields for OLP and for CLP (1.17 ± 0.88 and 10.42 ± 5.74, respectively), was found (intraepithelial, r = 0.50; dermal, r = 0.51; P < .01).
These data suggest a pivotal role for perforin in triggering epithelial cell apoptosis. The differences of infiltrating cytotoxic cells and related AI observed in OLP and CLP are in keeping with the clinical behaviors that distinguish these LP variants.
HEAT SHOCK PROTEINS
Heat shock proteins 60 and 70 expression of cutaneous lichen planus: comparison with normal skin and psoriasis vulgaris*.
Bayramgurler D, Ozkara SK, Apaydin R, Ercin C, Bilen N.
Department of Dermatology, Kocaeli University, School of Medicine, Izmit, Turkey.
J Cutan Pathol. 2004 Oct;31(9):586-94. Abstract quote
Background: Heat shock proteins (HSPs) are expressed by most living cells and play fundamental roles in many biological processes. Their synthesis increases by a variety of stresses in order to enable cellular survival. Although it is known that they play an important role in immune and inflammatory responses of the skin, the role of HSPs in the pathogenesis of skin diseases has been studied in only limited skin diseases. Lichen planus (LP) is a relatively common papulosquamous dermatosis, and cell-mediated immunity plays an important role in its pathogenesis. Although an altered expression of certain HSPs was reported in oral LP lesions, the expression of HSPs in cutaneous lesions of LP has not been investigated. In this immunohistochemical study, we aimed at investigating the role of HSPs in the pathogenesis of LP by studying whether there is any difference in HSP expression in cutaneous lesions of LP when compared to normal skin and psoriasis vulgaris (PV).
Methods: Formalin-fixed paraffin-embedded skin biopsy specimen blocks from LP patients (n = 39), patients with psoriasis (n = 20), and normal skin controls (n = 20) were used in the study. Antibodies to HSPs 60 and 70 were applied immunohistochemically by using streptavidin-biotin-horseradish peroxidase complex. An immunoreactivity intensity distribution index (IRIDI) was calculated to express the proportion of the immunoreactive cells as well as the staining intensity in different layers of the epidermis.
Results: The mean IRIDI scores for HSP60 expression in the basal, suprabasal, and superficial epidermal layers of cutaneous LP were moderately higher than those of normal skin, but not different from those of PV skin. These scores for HSP70 in lesions of LP were moderately lower than those for normal skin in the basal layer, but not significantly different from normal in the other two layers. Scores for HSP70 in PV lesions were markedly lower in all three layers. In the cells of the inflammatory infiltrates (mostly lymphocytes), HSP60 scores for LP were moderately higher, compared to those for PV, whereas scores for HSP70 were much lower for LP and very much lower for PV.
Conclusions: Significantly altered levels of HSP proteins were found in cutaneous LP lesions in comparison with normal skin and psoriasis, suggesting the role of HSPs in the pathogenesis of LP.
- J Cutan Pathol. 2006 Oct;33(10):672-8. Abstract quote
Introduction: Lichen planus (LP) is an inflammatory autoimmune skin disease of unknown origin. Evidence has accumulated that autoreactive cytotoxic CD8(+) T lymphocytes cause destruction of keratinocytes. Recent studies suggested that type I interferons (IFNs) play a central role in cytotoxic skin inflammation by increasing the expression of IP10/CXCR10 and recruiting effector cells via CXCR3. Here, we investigated whether type I IFNs are also involved in the pathogenesis of LP. Patients and methods: Skin biopsies of altogether 17 donors (seven LP and 10 healthy controls) were analyzed by immunohistochemistry using monoclonal antibodies against CD3, CD4, CD8, CD20, CD68, CXCR3, granzyme B, IP10/CXCL10, CD123, and the MxA protein, which is specifically induced by type I IFNs.
Results: Our analysis revealed a significant expression of the MxA protein in all LP skin biopsies, indicating involvement of type I IFNs. Expression of MxA was closely associated with the recruitment of CXCR3(+) and granzyme B(+) lymphocytes, indicating a Th1-biased cytotoxic immune response. Strong expression of the CXCR3 ligand, the interferon-inducible protein IP10/CXCL10, links type I IFN expression and recruitment of CXCR3(+) lymphocytes. Plasmacytoid dendritic cells (pDCs) appear to be a major source of type I IFNs in LP.
Discussion: Our observations support the hypothesis that lesional type I IFNs produced by pDCs plays an important role in chronic cytotoxic inflammation of LP by recruiting cytotoxic effector lymphocytes via IP10/CXCR3 interactions.
MATRIX METALLOPROETINASES 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
CHARACTERIZATION SKIN VARIANTS ANNULAR
Annular lichen planus: A case series of 20 patients.
Reich HL, Nguyen JT, James WD.
J Am Acad Dermatol. 2004 Apr;50(4):595-9. Abstract quote
BACKGROUND: Annular lichen planus (ALP) is a long-recognized clinical variant of lichen planus, but is often considered uncommon in occurrence. The typical distribution and presentation of this variant have not been well described.
OBJECTIVE: We sought to better define the sites affected and clinical characteristics of the annular variant of lichen planus, along with the age and race of patients affected with this disorder.
METHODS: We conducted a retrospective review of 20 patients given a diagnosis of ALP during an 18-year period. The diagnosis was confirmed histologically in all but 3 cases of classic ALP that presented on the glans penis.
RESULTS: Patients ranged in age from 24 to 76 years. There were 18 men and 2 women; 15 were Caucasian and 5 were African American. Sites of involvement in order of decreasing frequency included: axilla (35%); penis (25%); extremities (25%); groin (including the inguinal creases and scrotum) (20%); back (15%); buttocks (10%); flanks (5%); neck (5%); and eyelids (5%). None of the patients had oral mucosal, vulval, scalp, or nail lesions. A total of 18 patients had purely annular lesions, whereas 2 of the 20 had a few purple polygonal papules in the vicinity of the annular forms. Some eruptions were macular, whereas the majority had a slightly raised edge with central clearing. In all, 6 patients had solitary lesions whereas only 4 had 10 or greater lesions. None exhibited a linear Koebnerized response or generalized lesions. Most patients were asymptomatic.
CONCLUSIONS: ALP commonly involves the male genitalia but also has a predilection for intertriginous areas such as the axilla and groin folds. Eruptions typically consist of a few lesions localized to one or a few sites. Distal aspects of the extremities, and less commonly the trunk, may also be involved. ALP is a subtype of lichen planus that may be more common than is reflected in the literature.
ATROPHIC May resemble porokeratosis ERYTHEMUM DYSCHRONICUM 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
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
- A case-series of 29 patients with lichen planopilaris: the Cleveland Clinic Foundation experience on evaluation, diagnosis, and treatment.
Department of Dermatology, The Cleveland Clinic Foundation, OH, USA.
- J Am Acad Dermatol. 2007 Jul;57(1):47-53. Epub 2007 Apr 30 Abstract quote
BACKGROUND: Lichen planopilaris results in scaling, atrophy, and permanent alopecia with scarring and is thought to be autoimmune in origin.
OBJECTIVE: To evaluate the clinical findings of patients with LPP so as to aid in the evaluation and diagnosis of the disease and to review the current effective therapies.
METHODS: We reviewed the medical records of 29 patients with LPP that were seen in the Department of Dermatology at The Cleveland Clinic Foundation between 1992 and 2003.
RESULTS: Good responses in the active perimeter were seen with topical steroids, intralesional steroids, and tetracycline and in the inactive end-stage with hair transplants and scalp reductions.
LIMITATIONS: This study was limited by being retrospective in nature.
CONCLUSION: Although topical high-potency and intralesional corticosteroids remain the mainstay for treatment of LPP, the use of tetracycline in this disease may be more helpful than once thought.
LICHEN PLANUS PEMPHIGOIDES
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
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-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
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 HYPERTROPHIC LICHEN PLANUS Hypertrophic Lichen Planus–Like Reactions Combined With Infundibulocystic Hyperplasia
Pathway to Neoplasia
Steven Kossard, FACD; Carol Thompson, PhD; Gary M. Duncan, FRACS
Arch Dermatol. 2004;140:1262-1267. Abstract quote Background Retinoids have the capacity to accelerate the involution of multiple keratoacanthomas, including unusual variants such as keratoacanthoma marginatum centrifugum and keratoacanthoma en plaque that may persist and be associated with progressive growth and provide difficulties in diagnosis and management.
Observations We describe 3 patients who had unusual infiltrated and keratotic plaques affecting the lower legs or nasolabial area that persisted or recurred that may be related to this group of unusual keratoacanthomas. The 3 patients had differing clinical lesions that did not resemble classic keratoacanthomas, but were linked by their biopsy findings of hypertrophic lichen planus–like reaction and pseudoepitheliomatous hyperplasia with a prominent infundibulocystic component that progressed to multiple keratoacanthomas or infundibulocystic squamous cell carcinoma. Polymerase chain reaction analysis of biopsy material from 2 patients failed to detect human papillomavirus. All 3 presentations provided a therapeutic dilemma, but responded rapidly to acitretin treatment at a dosage of 10 to 25 mg daily, which was continued for 15 to 24 months.
Conclusions These cases illustrate an unusual reaction pattern that is hypertrophic lichen planus–like but, instead of evolving to classic lichen planus, progresses to infundibulocystic hyperplasia and the development of multiple keratoacanthomas or infundibulocystic squamous cell carcinomas. Retinoids represent a therapeutic option for this difficult clinical problem and may obviate repeated and extensive surgery.
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.
CHARACTERIZATION Direct immunofluorescence (DIF) Colloid bodies show staining for IgM, complement, and some immunoglobulins.
PROGNOSIS AND TREATMENT CHARACTERIZATION TREATMENT Topical occlusive steroids LICHEN PLANOPILARIS
Short course of oral cyclosporine in lichen planopilaris.
Mirmirani P, Willey A, Price VH.
Department of Dermatology, University of California-San Francisco, CA 94117, USA.
J Am Acad Dermatol. 2003 Oct;49(4):667-71. Abstract quote
Lichen planopilaris is a rare inflammatory disorder of unclear etiology that causes permanent scalp hair loss. Current treatments for lichen planopilaris are limited and do not alter the eventual outcome of the disease. Oral cyclosporine has been successful in treating severe and refractory lichen planus of the skin, and has produced sustained remission in some patients.
We present 3 patients with lichen planopilaris who were treated with a short course of oral cyclosporine and report their clinical features and responses, using a standardized method of patient assessment and 3 specific outcome measures.
In all 3 patients alleviation of symptoms, resolution of clinical activity, and halting progression of hair loss was achieved in 3 to 5 months with sustained effect at 12 months after a short course of cyclosporine therapy.
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 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.
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
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.
An open trial of topical tacrolimus for erosive oral lichen planus.
Morrison L, Kratochvil FJ 3rd, Gorman A.
Department of Dermatology, Oregon Health and Science University, Portland 97201, USA
J Am Acad Dermatol 2002 Oct;47(4):617-20 Abstract quote
Erosive oral lichen planus is a chronic, painful disease that is frequently refractory to treatment.
We describe 6 patients with erosive oral lichen planus, not responsive to topical steroids, who showed substantial improvement with the use of topical tacrolimus ointment 0.1%.
This medication was well tolerated and appears to be effective in controlling erosive oral lichen planus.
SKIN MYCOOPHENOLATE MOFETIL
Treatment of severe lichen planus with mycophenolate mofetil.
Frieling U, Bonsmann G, Schwarz T, Luger TA, Beissert S.
Department of Dermatology, University of Munster, Von-Esmarch-Strasse 58, D-48149 Munster, Germany.
J Am Acad Dermatol. 2003 Dec;49(6):1063-6. Abstract quote
Lichen planus (LP) is an inflammatory skin disorder with a wide range of clinical appearances. The treatment of disseminated and especially erosive forms of LP is often difficult and disappointing.
Activated T cells are important in the pathogenesis of LP as indicated by the dermal lymphocytic infiltrate leading to keratinocyte destruction and lesion formation. Similar histologic findings are present in graft-versus-host disease. Since T cells are key players in the development of both disorders and mycophenolate mofetil has been successfully introduced in the treatment of graft-versus-host disease, we have examined the therapeutic potential of this agent in 3 patients suffering from disseminated and erosive LP.
Mycophenolate mofetil was well tolerated and induced complete remission in 2 patients, and substantial improvement in the third patient.
Low-dose ultraviolet A1 phototherapy for extragenital lichen sclerosus: Results of a preliminary study
Alexander Kreuter, MD
Thilo Gambichler, MD
Annelies Avermaete, MD, etal.
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.
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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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Erosive lichen planus
Lichen planus of the skin
Oral Lichen Planus
Cicatricial Alopecia Research Foundation (CARF)
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