HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
Non-contagious, subacute to chronic, markedly pruritic, usually self-limited (duration of disease between 1 month and 10 years), inflammatory disease of the skin and/or mucous membranes covered with squamous epithelium (intestinal mucosa and mucous membranes of the urogenital tract remain uninvolved) of unknown etiology, with typical clinical (papules shining on the skin as if polished) and histological morphology (destruction of basal keratinocytes by cytotoxic T cells) and a characteristic distribution pattern often accentuated on the flexor side.
Lichen planus is characterized by a characteristic "lichenoid tissue reaction", which can also occur in other inflammatory processes of the skin (e.g. in lichenoid drug reactions, in a "graft-versus-host reaction" in initial lichen sclerosus et atrophicus, in erythema dyschromicum perstans).
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Occurrence/EpidemiologyThis section has been translated automatically.
Prevalence: 0.2%-1.0% of the (adult) population.
Up to 25% of patients have an isolated lichen planus of the mucosa.
Familial lichen planus is rare (about 100 cases are known). Earlier age of manifestation than non-familial LP
EtiopathogenesisThis section has been translated automatically.
Autoimmune reaction: To date, the etiology and pathogenesis of lichen ruber is not fully understood. There are correlations with autoimmune diseases, viral infections, medications, as well as mechanical trigger factors (scratching, rubbing, etc.). LP-like lesions occur in chronic graft-versus-host disease (GVHD), in which alloreactive cytotoxic T cells and antibodies recognizing foreign MHC molecules are crucial effectors. The morphologic analogy of dermatitic reactions leads to the hypothesis that in lichen planus there is an autoimmune reaction against epitopes of basal keratinocytes modified by viral or drug induction. It is undisputed that apoptotic destruction of basal keratinocytes is the common final pathway of the lichen planus reaction (this certainly plays an important role in other autoimmune diseases of the skin, e.g. LE). Ligand-receptor-dependent dysregulation ( TNF-alpha/TNFR1= TNF-alpha receptor) is discussed as its cause. Also discussed is the direct "pore formation" by perforin known in apoptosis and the subsequent enzymatic degradation by serine proteases (see Granzyme B below).
In early changes, the marked proliferation of antigen-presenting cells (APCs) is striking. These are possibly induced by the keratinocytes themselves through a misregulation of cytokine production.
Viral antigens appear to play a preferential role in the etiopathogenesis of lichen ruber. The prevalence of HCV/HBV infection (hepatitis C/B) is 13.5-fold higher in lichen planus than in controls. In oral lichen planus, HCV-RNA and TTV-DNA (transfusion-transmitted virus) were detected in a high percentage in lesional mucosa. The occurrence of lichen ruber after HBV vaccination has been described. The etiopathogenetic relevance to HHV-7/HHV-8 is not clearly provable. Notable are reports of the occurrence of lichen ruber after SARS-CoV-2 vaccination (Zengarini C et al. 2022).
Drugs: Lichen ruber as a UAW after therapy with monoclonal antibodies against programmed cell death 1 (anti-PD-1 - e.g. nivolumab): These UAWs include both classic lichen ruber and, in rarer cases, pemphigoid lichen ruber. Naproxen is known to be another lichen planus inducer (Güneş AT et al. 2006).
Diabetes mellitus: A strikingly frequent association is that of lichen ruber and diabetes mellitus. One in 2 patients has a disorder of glucose metabolism and one in 4 has manifest diabetes mellitus.
Contact allergens: The role ofcontact allergies to a number of metal salts (gold, amalgam, copper) is well known in oral lichen ruber. Here, it is discussed that these hapten-type antigens may trigger an LP reaction.
Paraneoplasia: Isolated reports of paraneoplastic lichen ruber exist.
Familial occurrence: Approximately 100 cases of familial lichen planus have been reported. There is no significant relationship to specific HLA types.
ManifestationThis section has been translated automatically.
Preferably occurring in adults in the 3rd to 6th decade of life. Rare in children (about 1-4% of cases).
No ethnic predisposition. Women seem to be affected slightly more often than men (see also Lichen planus mucosae).
LocalizationThis section has been translated automatically.
Flexed side of wrists and forearms, lateral ankle area of ankles.
Mucous membrane affection (30-40%): penis, oral and genital mucosa, esophageal mucosa)
Capillitium (see also Graham-Little syndrome).
Generalized or universal (erythrodermic) occurrence is possible (see Lichen planus exanthematicus).
Lichen ruber affecting the large bends of the joints on the flexor side
Clinical featuresThis section has been translated automatically.
Initially about 0.1 cm in size, bordered by the natural furrows of the skin, raised, plateau-like, smooth, varnish-like shiny (surface smooth as if polished), distinctly itchy, red papules. Aggregation of multiple papules with formation of plaques of varying size. Rarely, a small lamellar scaling is visible.
The diagnostically important surface reflection of the lichen planus papules is best recognized when the light is incident from the side. Furthermore, the Wickham's pattern and the linear arrangement of the efflorescences in scratch or rub marks are typical (see below Köbner phenomenon [= isomorphic stimulus effect]). The patient responds to the lesional itching by rubbing ( glossy nails are often detectable); excoriations rarely occur (thus fresh or older scratch erosions are absent, as they are to be expected, for example, in atopic dermatitis ).
The palms and soles, including the lateral edges, show localized, coarse, yellow-reddish, hyperkeratotic plaques, with red margins at the edges.
A varying degree of oral mucosal involvement (see also lichen planus mucosae) is observed in > 50% of patients. Typical are symmetrical, reticular or nummular white plaques, also disseminated, 0.1 cm white papules of the buccal mucosa and/or tongue and/or gingiva. A special feature is the painful and therapy-resistant erosive lichen planus of the oral mucosa.
Genitalia, especially the glans penis and the vulva, are frequently affected in the form of anular or circular, whitish but also red or erosive plaques (see below Lichen ruber mucosae/ Lichen planus vulvae).
Capillitium (see below Lichen ruber follicularis capillitii).
Nails: Frequently thinned and shortened, often frayed nail plates with longitudinal surface distortions and numerous spots. Pterygia possible. More rarely, colorless or red longitudinal striations(erythronychia). Complete destruction of the nail plate is possible. Shiny nails are not uncommon (consequence of continuous rubbing).
LaboratoryThis section has been translated automatically.
No pioneering laboratory parameters!
HistologyThis section has been translated automatically.
Uniform and pathognomic histologic pattern of a classic interface dermatitis with irregular, often sawtooth-like acanthosis, compact orthohyperkeratosis with prominent hypergranulosis (circumscribed thickening of the keratohyalin-containing cell layers of the stratum granulosum causes the clinical picture of Wickham's pattern). Usually very prominent, dense, band-like, lymphoid cellular, epidermotropic infiltrate. The inflammatory infiltrate consists predominantly of oligoclonal, CD8-positive, cytotoxic T cells. Focal pigmentary incontinence. Vacuolar degeneration of the basal epithelial cell layers, resulting in clefts (Max-Joseph spaces) and even subepithelial blistering (lichen planus bullosus). Detection of numerous cytoid bodies (Civatte bodies or cytoid bodies (colloid bodies) see below. Apoptosis). Epidermal Langerhans cells are increased in active lesions. Plasma cells, neutrophils, and eosinophils leukocytes may be present but are not common.
Direct ImmunofluorescenceThis section has been translated automatically.
Differential diagnosisThis section has been translated automatically.
- Clinical differential diagnoses:
- Psoriasis punctata: The Auspitz phenomenon is always detectable and always absent in lichen planus!
- Lichenoid medicinal exanthema: History; usually no involvement of the oral mucosa.
- Pityriasis lichenoides et varioliformis acuta: As in "Heubner's star chart" very polymorphous, pruritic or also burning exanthema, with papules, erosions, ulcers and possibly hemorrhagic vesicles. Lichen planus exanthema is monomorphic.
- Papular syphilid: Clinically, the lichenoid character of the single-cell lesions is absent; pruritus is mild or absent.
- Scabies: Multiple erythematous papules on the wrists may complicate the differential diagnosis of scabies and lichen ruber.
- Oral mucosa:
- Leukoplakia: In the area of the oral mucosa, leukoplakia or mechanical mucosal irritations have to be differentiated. Histological clarification is necessary. In both cases the typical "anular structures" of the LP are missing.
- Candidiasis of the oral mucosa: Infestation of the tongue, cheek mucosa and soft and hard palate with white to grey-white plaques that can be easily wiped off (LP cannot be wiped off!).
- Gingivitis marginalis: similar, therapy-resistant pattern with analogous symptoms. Histological clarification is necessary.
- Histological differential diagnoses:
- Lichenoid medicinal exanthema: Largely identical picture, apoptotic keratinocytes are frequent, possibly focal parakeratosis, which is always absent in lichen planus. Marked histoeosinophilia is possible as well as a plasma cell infiltrate component.
- Fixed drug reaction: Numerous apoptotic keratinocytes, perivascular infiltrate thickening, often marked eosinophilia, prominent pigment incontinence.
- Lichensclerosus et atrophicus: Initial lichenoid infiltrate without 3-zone phenomenon. Pattern analogous to lichen planus; later typical zonal pattern.
- Acute graft-versus-host disease: Numerous apoptotic keratinocytes, strong vacuolization of the junctional zone, less dense infiltrate.
- Papular syphilid: Epidermis with psoriasiform acanthosis and focal spongiosis; admixture of neutrophilic granulocytes and (numerous) plasma cells.
Complication(s)This section has been translated automatically.
In long persisting LP lesions of the skin and mucous membranes there is a certain (unspecified) risk to develop epithelial tumors (spinocellular carcinomas).
General therapyThis section has been translated automatically.
The therapy depends on the clinical aspect and the course. Treatment of itching is in many cases the main focus.
External therapyThis section has been translated automatically.
Glucocorticoids: In the case of described low symptom findings, medium-strength glucocorticoids such as 0.25% prednicarbate (e.g. Dermatop® cream), 0.1% mometasone furoate (e.g. Ecural® fat cream), in persistent cases also strong 0.05% glucocorticoids such as clobetasol (e.g. Dermoxin® cream), if necessary also under occlusion (2 times/day 2-4 hours).
Calcineurin inhibitors: Tacrolimus or Pimecrolimus can be applied topically as off-label use. Both substances are especially effective in case of mucosal infestation. Because of the unknown long-term effects of calcineurin inhibitors and the carcinogenicity of Pimecrolimus which has been proved in animal experiments, the indication for the therapy with calcineurin inhibitors has to be set very strictly!
Radiation therapyThis section has been translated automatically.
Internal therapyThis section has been translated automatically.
Acitretin: In case of extensive infestation, start of therapy with acitretin (neotigason) initially 0.5 mg/kg bw/day, maintenance dose 0.1-0.2 mg/kg bw/day after clinic. Systemic retinoids achieve the highest evidence levels in studies (Schilling L et al. 2018). Attempt to discontinue after 1/2 year at the earliest.
Alternatively, or in the case of a severe form: Acitretin in combination with glucocorticoids such as prednisolone (e.g. Decortin H) initially 0.5 mg/kg bw/day, release over a period of 4-6 weeks. Maintenance dose according to clinic with 5-10 mg/day.
Alternatively, other systemic therapeutics described in several smaller studies can be considered as "third line" therapy for therapy-resistant lichen planus of the external integument. These include: Ciclosporin A, griseofulvin, oral metronidazole, sulfasalazine, mycophenolate mofetil, azathioprine, thalidomide.
Alternatively, experimentally: Apremilast, an oral thalidomide analogue, which has been successfully tested in a smaller monocentric study for the exanthematic lichen planus.
- For the generally therapy-resistant lichen planus erosivus mucosae (see there), stronger local or systemic immunosuppressive measures are necessary.
- Lichen planus genitalis: Therapeutic measures see below Lichen planus erosivus mucosae.
- Lichen planus follicularis capillitii (see there).
- Lichen planus of the nails: I.A. no special therapy, since nail changes usually occur with other LP lesions. Some US authors recommend perilesional injections with glucocorticoids(Cave! painfulness). In individual cases, good therapeutic success has been reported with Ciclosporin A (initial: 2x100mg/day p.o. maintenance dose 100mg/day p.o.). Own experiences exist with azathioprine (initial: 1.0-1.5mg/kgkgkg, maintenance dose at 0.5mg/kgkgkg).
- In the case of drug-induced lichen planus, the initiating drugs must be discontinued. Otherwise therapy as with classical lichen planus.
Progression/forecastThis section has been translated automatically.
NaturopathyThis section has been translated automatically.
Externally: Phytotherapy: Anti-itching plant ingredients with cooling effect such as: camphor, mint or peppermint oil, menthol in DAC cream as a base, capsaicin (0.02-0.075%) can be applied on intact skin! 2-3x /day can be applied.
Formulation example for a 1% menthol cream:
- DAC base cream ad 100,0.
- S.:Apply Ambiphilic 1% Menthol Cream 2-3 times/day to itchy skin areas. Use-by date: Tube: 6 months
Systemic: Oral bromelain (e.g. Bromelain-POS) or Phlogenzym(Phlogenzym-mono) is further recommended.
LiteratureThis section has been translated automatically.
- Brănişteanu DE et al (2014) Cutaneous manifestations associated with thyroid disease. Rev Med Chir Soc Med Nat Iasi 118: 953-958.
- Butch F et al (2014) Successful therapy of lichen planus of the nails nit ciclosporin. JDDG 12: 724-725
- Chiheb S et al (2015) Clinical characteristics of nail lichen planus and follow-up: A descriptive study of 20 patients. Ann Dermatol Venereol 142:21-25
- Deen K et al (2015) Mycophenolate mofetil in erosive genital lichen planus: A case and review of the literature. J Dermatol doi: 10.1111/1346-8138.12763
- De Vries et al (2007) Lichen planus remission is associated with decrease of human herpes virus zype 7 protein expression in plasmacytoid dendritic cells. Arch Dermatol Res 299: 213-219
- Eisman S, Orteu CH (2004) Recalcitrant erosive flexural lichen planus: successful treatment with a combination of thalidomide and 0.1% tacrolimus ointment. Clin Exp Dermatol 29: 268-270
- Frieling U et al (2003) Treatment of severe lichen planus with mycophenolate mofetil. J Am Acad Dermatol 49: 1063-1066.
- Gandolfo S et al (2004) Risk of oral squamous cell carcinoma in 402 patients with oral lichen planus: a follow-up study in an Italian population. Oral Oncol 40: 77-83
Güneş AT et al (2006) Naproxen-induced lichen planus: report of 55 cases. Int J Dermatol 45:709-712.
- Harden D et al (2003) 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. J Am Acad Dermatol 49: 847-852.
- Hodgson TA et al (2003) Long-term efficacy and safety of topical tacrolimus in the management of ulcerative/erosive oral lichen planus. Eur J Dermatol 13: 466-470.
- Kraft K (2014) Naturally against pruritus. Hautnah Dermatology 30: 42-43.
- Kolb-Maurer A et al (2003) Treatment of lichen planus pemphigoides with acitretin and pulsed corticosteroids. Dermatologist 54: 268-273
- Lehman J et al (2009) Lichen planus. Int J Dermatol 46: 682-694.
- Mastorino L et al (2022) Lichen ruber planus arising during dupilumab treatment for atopic dermatitis. Ital J Dermatol Venerol 157:449-450.
- Mueller KA et al (2021) A case of severe nivolumab-induced lichen planus pemphigoides in a child with metastatic spitzoid melanoma. Pediatr Dermatol 40: 154-156.
- Wilson E (1869) On lichen planus. J Cutan Med 8: 117
- Wolf R et al (2010) Pleomorphism of lichen ruber - clinical variation, pathogenesis, and therapy. Act Dermatol 36: 180-185
- Zengarini C et al (2022) Lichen ruber planus occurring after SARS-CoV-2 vaccination. Dermatol Ther 35:e15389.
Incoming links (91)Acanthosis; Acitretin; Acrokeratosis verruciformis; Alterserythrodermia; Anal carcinoma; Anal fissure; Angiohistiocytoma with giant cells; Apoptosis; Apremilast; Autoimmune diseases; ... Show all
Outgoing links (50)Acitretin; Apoptosis; Apremilast; Atopic dermatitis (overview); Azathioprine; Candidiasis of the oral mucosa; Ciclosporin a; Drug exanthema lichenoid; Drug reaction fixe; Erythronychia longitudinalis;; ... Show all
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