Lichen planus (LP) is a chronic inflammatory and

immune-mediated disease An immune disorder is a dysfunction of the immune system. These disorders can be characterized in several different ways: * By the component(s) of the immune system affected * By whether the immune system is overactive or underactive * By whether t ...

that affects the skin, nails, hair, and mucous membranes. It is not an actual

lichen A lichen ( , ) is a composite organism that arises from algae or cyanobacteria living among filaments of multiple fungi species in a mutualistic relationship. It is characterized by polygonal, flat-topped, violaceous papules and plaques with overlying, reticulated, fine white scale ( Wickham's striae), commonly affecting dorsal hands, flexural wrists and forearms, trunk, anterior lower legs and oral mucosa. The hue may be gray-brown in people with darker skin. Although there is a broad clinical range of LP manifestations, the skin and oral cavity remain as the major sites of involvement. The cause is unknown, but it is thought to be the result of an

autoimmune In immunology, autoimmunity is the system of immune responses of an organism against its own healthy cells, tissues and other normal body constituents. Any disease resulting from this type of immune response is termed an "autoimmune disease". P ...

process with an unknown initial trigger. There is no cure, but many different medications and procedures have been used in efforts to control the symptoms. The term lichenoid reaction (lichenoid eruption or lichenoid lesion) refers to a

lesion A lesion is any damage or abnormal change in the tissue of an organism, usually caused by disease or trauma. ''Lesion'' is derived from the Latin "injury". Lesions may occur in plants as well as animals. Types There is no designated classifi ...

of similar or identical

histopathologic Histopathology (compound of three Greek words: ''histos'' "tissue", πάθος ''pathos'' "suffering", and -λογία ''-logia'' "study of") refers to the microscopic examination of tissue in order to study the manifestations of disease. Spec ...

and clinical appearance to lichen planus (i.e., an area which resembles lichen planus, both to the naked eye and under a

microscope A microscope () is a laboratory instrument used to examine objects that are too small to be seen by the naked eye. Microscopy is the science of investigating small objects and structures using a microscope. Microscopic means being invisibl ...

). Sometimes dental materials or certain medications can cause a lichenoid reaction. They can also occur in association with

graft versus host disease Graft-versus-host disease (GvHD) is a syndrome, characterized by inflammation in different organs. GvHD is commonly associated with bone marrow transplants and stem cell transplants. White blood cells of the donor's immune system which remain wit ...

Classification

''Lichen planus'' lesions are so called because of their "

lichen A lichen ( , ) is a composite organism that arises from algae or cyanobacteria living among filaments of multiple fungi species in a mutualistic relationship. and can be classified by the site they involve, or by their morphology.

Site

Lichen planus may be categorized as affecting

mucosal A mucous membrane or mucosa is a membrane that lines various cavities in the body of an organism and covers the surface of internal organs. It consists of one or more layers of epithelial cells overlying a layer of loose connective tissue. It is ...

cutaneous Skin is the layer of usually soft, flexible outer tissue covering the body of a vertebrate animal, with three main functions: protection, regulation, and sensation. Other animal coverings, such as the arthropod exoskeleton, have different de ...

surfaces. * ''Cutaneous'' forms are those affecting the skin, scalp, and nails. * ''Mucosal'' forms are those affecting the lining of the

gastrointestinal tract The gastrointestinal tract (GI tract, digestive tract, alimentary canal) is the tract or passageway of the digestive system that leads from the mouth to the anus. The GI tract contains all the major organ (biology), organs of the digestive syste ...

(mouth, pharynx, esophagus, stomach, anus),

larynx The larynx (), commonly called the voice box, is an organ in the top of the neck involved in breathing, producing sound and protecting the trachea against food aspiration. The opening of larynx into pharynx known as the laryngeal inlet is about ...

, and other mucosal surfaces including the genitals,

peritoneum The peritoneum is the serous membrane forming the lining of the abdominal cavity or coelom in amniotes and some invertebrates, such as annelids. It covers most of the intra-abdominal (or coelomic) organs, and is composed of a layer of mesoth ...

, ears, nose, bladder and

conjunctiva The conjunctiva is a thin mucous membrane that lines the inside of the eyelids and covers the sclera (the white of the eye). It is composed of non-keratinized, stratified squamous epithelium with goblet cells, stratified columnar epithelium ...

of the eyes.

Pattern

Lichen planus lesions can occur in many different forms:

Overlap syndromes

Occasionally, lichen planus is known to occur with other conditions. For example: *

Lupus erythematosus Lupus erythematosus is a collection of autoimmune diseases in which the human immune system becomes hyperactive and attacks healthy tissues. Symptoms of these diseases can affect many different body systems, including joints, skin, kidneys, blo ...

overlap syndrome. Lesions of this syndrome share features of both lupus erythematosus and lichen planus. Lesions are usually large and hypopigmented, atrophic, and with a red to blue colour and minimal scaling.

Telangectasia Telangiectasias, also known as spider veins, are small dilated blood vessels that can occur near the surface of the skin or mucous membranes, measuring between 0.5 and 1 millimeter in diameter. These dilated blood vessels can develop anywhere on ...

may be present. *

Lichen sclerosus Lichen sclerosus (LS) is a chronic, inflammatory skin disease of unknown cause which can affect any body part of any person but has a strong preference for the genitals (penis, vulva) and is also known as balanitis xerotica obliterans (BXO) when ...

overlap syndrome, sharing features of lichen planus and lichen sclerosus.

Signs and symptoms

Although lichen planus can present with a variety of lesions, the most common presentation is as a well defined area of purple-coloured, itchy, flat-topped papules with interspersed lacy white lines (Wickham's striae). This description is known as the characteristic "6 Ps" of lichen planus: planar (flat-topped), purple, polygonal, pruritic, papules, and plaques. This rash, after regressing, is likely to leave an area of hyperpigmentation that slowly fades. That said, a variety of other lesions can also occur.

Skin

Variants of cutaneous lichen planus are distinguished based upon the appearance of the lesions and/or their distribution. Lesions can affect the: * Extremities (face, dorsal hands, arms, and nape of neck). This is more common in Middle Eastern countries in spring and summer, where sunlight appears to have a precipitating effect. * Palms and soles *

Intertriginous In medicine, an intertriginous area is where two skin areas may touch or rub together. Examples of intertriginous areas are the axilla of the arm, the anogenital region, skin folds of the breasts and between digits. Intertriginous areas are known ...

areas of the skin. This is also known as "inverse lichen planus." * Nails characterized by irregular longitudinal grooving and ridging of the nail plate, thinning of the nail plate, pterygium formation, shedding of the nail plate with atrophy of the nail bed, subungual keratosis, longitudinal erthronychia (red streaks), and subungual hyperpigmentation. A sand-papered appearance is present in around 10% of individuals with nail lichen planus. * Hair and scalp. The scalp is rarely affected by a condition known as lichen planopilaris, acuminatus, follicular lichen planus, and peripilaris, characterised by violaceous, adherent follicular scale with progressive scarring alopecia. While lichen planus and lichen planopilaris may occur together, aside from sharing the term 'lichen' and revealing inflammation on skin biopsy, there is neither established data on their co-occurrence nor data to suggest a common etiology. Lichen planopilaris is considered an orphan disease with no definitive prevalence data and no proven effective treatments. Other variants may include: * ''Lichen planus pemphigoides'' characterized by the development of tense blisters atop lesions of lichen planus or the development vesicles de novo on uninvolved skin. * ''Keratosis lichenoides chronica'' (also known as "Nekam's disease") is a rare dermatosis characterized by violaceous papular and nodular lesions, often arranged in a linear or reticulate pattern on the dorsal hands and feet, extremities, and buttock, and some cases manifest by seborrheic dermatitis-like eruption on the scalp and face; also palmo plantar keratosis has been reported. * ''Lichenoid keratoses'' (also known as "benign lichenoid keratosis," and "Solitary lichen planus") is a cutaneous condition characterized by brown to red scaling maculopapules, found on sun-exposed skin of extremities. Restated, this is a cutaneous condition usually characterized by a solitary dusky-red to violaceous papular skin lesion. * ''Lichenoid dermatitis'' represents a wide range of cutaneous disorders characterized by lichen planus-like skin lesions.

Mucous membranes

Lichen planus affecting mucosal surfaces may have one lesion or be multifocal. Examples of lichen planus affecting mucosal surfaces include: * '' Esophageal lichen planus'', affecting the esophageal mucosa. This can present with difficulty or pain when swallowing due to oesophageal inflammation, or as the development of an

esophageal stricture A benign esophageal stricture, or peptic stricture, is a narrowing or tightening of the esophagus that causes swallowing difficulties. Signs and symptoms Symptoms of esophageal strictures include heartburn, bitter or acid taste in the mouth, chok ...

. It has also been hypothesized that it is a precursor to squamous cell carcinoma of the esophagus. * ''Genital lichen planus,'' which may cause lesions on the

glans penis In male human anatomy, the glans penis, commonly referred to as the glans, is the bulbous structure at the distal end of the human penis that is the human male's most sensitive erogenous zone and their primary anatomical source of sexual p ...

or skin of the scrotum in males, and the vulva or vagina in females. Symptoms may include lower urinary tract symptoms associated with

stenosis A stenosis (from Ancient Greek στενός, "narrow") is an abnormal narrowing in a blood vessel or other tubular organ or structure such as foramina and canals. It is also sometimes called a stricture (as in urethral stricture). ''Stricture'' ...

of the

urethra The urethra (from Greek οὐρήθρα – ''ourḗthrā'') is a tube that connects the urinary bladder to the urinary meatus for the removal of urine from the body of both females and males. In human females and other primates, the urethra con ...

, painful sexual intercourse, and itching. In females, ''Vulvovaginal-gingival syndrome,'' is severe and distinct variant affecting the

vulva The vulva (plural: vulvas or vulvae; derived from Latin for wrapper or covering) consists of the external sex organ, female sex organs. The vulva includes the mons pubis (or mons veneris), labia majora, labia minora, clitoris, bulb of vestibu ...

vagina In mammals, the vagina is the elastic, muscular part of the female genital tract. In humans, it extends from the vestibule to the cervix. The outer vaginal opening is normally partly covered by a thin layer of mucosal tissue called the hymen ...

, and gums, with complications including scarring, vaginal stricture formation, or vulva destruction. The corresponding syndrome in males, affecting the glans penis and gums, is the ''peno-gingival syndrome''. It is associated with

HLA-DQB1 Major histocompatibility complex, class II, DQ beta 1, also known as HLA-DQB1, is a human gene and also denotes the genetic locus that contains this gene. The protein encoded by this gene is one of two proteins that are required to form the DQ het ...

Mouth

Oral lichen planus (also termed ''oral mucosal lichen planus''), is a form of mucosal lichen planus, where lichen planus involves the

oral mucosa The oral mucosa is the mucous membrane lining the inside of the mouth. It comprises stratified squamous epithelium, termed "oral epithelium", and an underlying connective tissue termed ''lamina propria''. The oral cavity has sometimes been descri ...

, the lining of the mouth. This may occur in combination with other variants of lichen planus. Six clinical forms of oral lichen planus (OLP) are recognized: These types often coexist in the same individual. Oral lichen planus (OLP) tends to present bilaterally as mostly white lesions on the inner cheek, although any mucosal site in the mouth may be involved. Other sites, in decreasing order of frequency, may include the tongue, lips, gingivae, floor of the mouth, and very rarely, the palate. Generally, oral lichen planus tends not to cause any discomfort or pain, although some people may experience soreness when eating or drinking acidic or spicy foodstuffs or beverages. When symptoms arise, they are most commonly associated with the atrophic and ulcerative subtypes. These symptoms can include a burning sensation to severe pain. They may also experience mucosal bleeding in response to mild trauma, such as toothbrushing. Additionally, the Koebner phenomenon (the development of new lesions at sites of trauma) is not only present in cutaneous lichen planus (CLP) but can also occur in the setting of OLP. Residual postinflammatory hyperpigmentation has been reported in association with OLP, manifesting as brown to black pigmentation on the oral mucosa and may most likely occur in dark-skinned individuals. OLP may occur as a sole manifestation of the disease or in conjunction with other clinical manifestations of LP, including cutaneous LP, genital LP, nail LP, and lichen planopilaris (scalp LP).

Causes

Cutaneous LP is a self-limiting condition. It usually resolves within 6 to 12 months. Oral LP is a non infectious, chronic inflammatory condition that involves the oral mucosa and may be accompanied by skin lesions. The etiology of oral LP are unknown. It is not clear whether the mechanisms causing isolated oral LP are different from those causing oral LP with cutaneous LP. An immune-mediated mechanism where basal keratinocytes are being targeted as foreign antigens by activated T cells, especially

CD8+ A cytotoxic T cell (also known as TC, cytotoxic T lymphocyte, CTL, T-killer cell, cytolytic T cell, CD8+ T-cell or killer T cell) is a T lymphocyte (a type of white blood cell) that kills cancer cells, cells that are infected by intracellular pa ...

T cells, has been proposed. Upregulation of intercellular adhesion molecule-1 (

ICAM-1 ICAM-1 (Intercellular Adhesion Molecule 1) also known as CD54 (Cluster of Differentiation 54) is a protein that in humans is encoded by the ''ICAM1'' gene. This gene encodes a cell surface glycoprotein which is typically expressed on endothelial ...

) and cytokines associated with T-helper 1 immune response, may also play an important role in the pathogenesis of lichen planus. Stress is thought to play a role in the pathogenesis of oral LP. Patients with anxiety and depression are reported more commonly with oral LP if compared to normal healthy individuals. Some studies have indicated that stressful events can induce LP lesions in otherwise healthy individuals. However, a cause effect relationship between stress and the onset of oral LP has not been demonstrated. Autoimmune response to epithelial

self-antigen Immune tolerance, or immunological tolerance, or immunotolerance, is a state of unresponsiveness of the immune system to substances or tissue that would otherwise have the capacity to elicit an immune response in a given organism. It is induced by ...

s remains a possibility. A single study of cutaneous LP reported evidence in support of autoimmunity by expanding in vitro T cells isolated from the skin lesions of two patients, followed by testing the ability of these T cells to kill autologous keratinocytes (cytotoxicity). Several potential triggers of oral LP have been proposed over the years, mainly #

Hypersensitivity reaction Hypersensitivity (also called hypersensitivity reaction or intolerance) refers to undesirable reactions produced by the normal immune system, including allergies and autoimmunity. They are usually referred to as an over-reaction of the immune s ...

#* Restorative material (e.g.

amalgam Amalgam most commonly refers to: * Amalgam (chemistry), mercury alloy * Amalgam (dentistry), material of silver tooth fillings ** Bonded amalgam, used in dentistry Amalgam may also refer to: * Amalgam Comics, a publisher * Amalgam Digital ...

and composite) or drugs can cause hypersensitivity reaction and lead to oral LP. #* Oral LP usually resolve upon removal of the trigger, as is characteristics of oral LP # Viral infection

Pathogenesis

Oral LP is considered to be a T-cell mediated chronic inflammatory tissue reaction that results in a cytotoxic reaction against epithelial basal cells. The inflammatory infiltrate in oral LP is primarily composed of CD8+ T cells. A potential pathway for

T cell-mediated cytotoxicity in oral LP is described as follows: Antigens presented on MHC 1 molecules activates CD8+ T cells on keratinocytes or by encounters with activated CD4+ helper T cells or cytokines produced by activated CD4+

helper T cells The T helper cells (Th cells), also known as CD4+ cells or CD4-positive cells, are a type of T cell that play an important role in the adaptive immune system. They aid the activity of other immune cells by releasing cytokines. They are considere ...

Activated CD8+ T cells induce keratinocyte

apoptosis Apoptosis (from grc, ἀπόπτωσις, apóptōsis, 'falling off') is a form of programmed cell death that occurs in multicellular organisms. Biochemical events lead to characteristic cell changes (morphology) and death. These changes incl ...

through various mechanisms such as secretion of

tumor necrosis factor Tumor necrosis factor (TNF, cachexin, or cachectin; formerly known as tumor necrosis factor alpha or TNF-α) is an adipokine and a cytokine. TNF is a member of the TNF superfamily, which consists of various transmembrane proteins with a homolog ...

(TNF)-alpha, secretion of granzyme B, or Fas-Fas ligand interactions. Chemokines are produced by activated CD8+ T cells that attract additional inflammatory cells, thereby promoting continued inflammation. Other mechanisms that have been proposed include: * upregulation of

matrix metalloproteinase Matrix metalloproteinases (MMPs), also known as matrix metallopeptidases or matrixins, are metalloproteinases that are calcium-dependent zinc-containing endopeptidases; other family members are adamalysins, serralysins, and astacins. The MMPs b ...

s that disrupt the epithelial

basement membrane The basement membrane is a thin, pliable sheet-like type of extracellular matrix that provides cell and tissue support and acts as a platform for complex signalling. The basement membrane sits between Epithelium, epithelial tissues including mesot ...

zone and allow entry of immune cells into the epidermis, * the release of proinflammatory mediators and proteases by mast cells, and * perturbations in the innate immune response that may involve

toll-like receptor Toll-like receptors (TLRs) are a class of proteins that play a key role in the innate immune system. They are Bitopic protein, single-pass membrane-spanning Receptor (biochemistry), receptors usually expressed on sentinel cells such as macrophage ...

s. Oral LP may also be caused by genetic factor which influence the immune function. A separate study performed in China found an association between a polymorphism in the TNF-alpha gene and risk for oral LP in a subset of patients. An Italian study found a significant increase in a

genetic polymorphism A gene is said to be polymorphic if more than one allele occupies that gene's locus within a population. In addition to having more than one allele at a specific locus, each allele must also occur in the population at a rate of at least 1% to gen ...

of the first intron of the interferon (IFN)-gamma promoter in patients with oral LP compared with controls.

Diagnosis

Skin

Patient history and clinical presentation need to be taken to diagnose lichen planus. Patients with suspected cutaneous lichen planus need to be evaluated clinically through patient interviews and physical examinations. Patients should be questioned about their medication history, any history of pruritus or genital pain and history of dysphagia or odynophagia. Examination of entire cutaneous surface including the scalp, oral cavity and external genitalia need to be included. Wickham's striae often can be seen during microscopic examination of cutaneous lesions of lichen planus. To confirm the diagnosis of cutaneous lichen planus, a skin biopsy can be done. A punch biopsy of sufficient depth to the mid dermis is usually significant. Immunofluorescence studies are not always needed. Direct immunofluorescence (DIF) can be useful in patients with bullous lesions to differentiate the condition from an autoimmune vesiculobullous disease.

Mouth

A diagnosis of oral lichen planus (LP) is confirmed through review of the patient history, physical examination, and histologic findings. The clinical evaluation should include a patient history that assesses the following: * History of LP involving other body sites or other skin disorders that may present with similar findings (e.g., autoimmune blistering diseases) * Presence of associated symptoms (e.g., pain, burning) * Medication the patients are taking within the few weeks to months after drug initiation e.g.

antihypertensives Antihypertensives are a class of drugs that are used to treat hypertension (high blood pressure). Antihypertensive therapy seeks to prevent the complications of high blood pressure, such as stroke and myocardial infarction. Evidence suggests that r ...

antidepressant Antidepressants are a class of medication used to treat major depressive disorder, anxiety disorders, chronic pain conditions, and to help manage addictions. Common side-effects of antidepressants include dry mouth, weight gain, dizziness, hea ...

diuretic A diuretic () is any substance that promotes diuresis, the increased production of urine. This includes forced diuresis. A diuretic tablet is sometimes colloquially called a water tablet. There are several categories of diuretics. All diuretics in ...

antidiabetics Drugs used in diabetes treat diabetes mellitus by altering the glucose level in the blood. With the exceptions of insulin, most GLP receptor agonists (liraglutide, exenatide, and others), and pramlintide, all are administered orally and are th ...

, NSAIDs, etc. to evaluate for the possibility of an oral lichenoid drug eruption * History of dental restorations, use of dental appliances, or oral exposure to substances that may cause oral lichenoid contact eruptions (e.g. dental composites, cobalt chromium based dentures etc.) A full examination that includes the evaluation of the mucosal and cutaneous surfaces, including the vulva, vagina, penis, scalp, and nails should be performed. Thorough examination may lead to the detection of extraoral manifestations of LP that provide additional support for the diagnosis or the identification of clinical findings that suggest another diagnosis. Tissue biopsies of oral LP help to confirm the diagnosis and are particularly of value for erythematous and erosive LP, which share features with multiple other mucosal disorders, including oral malignancy. Biopsies to confirm oral LP are less essential in patients who present with classic reticular LP, particularly in patients in whom a diagnosis of LP has already been confirmed through biopsy of an extraoral manifestation of this disorder.

Differential diagnosis

Skin

# Lichenoid drug eruption #* The cutaneous manifestations resemble idiopathic lichen planus. # Chronic graft-versus-host disease #* The history of preceding hematopoietic cell transplant is helpful for diagnosis #

Psoriasis Psoriasis is a long-lasting, noncontagious autoimmune disease characterized by raised areas of abnormal skin. These areas are red, pink, or purple, dry, itchy, and scaly. Psoriasis varies in severity from small, localized patches to complete ...

# Atopic dermatitis # Cutaneous lupus erythematosus # Discoid lupus erythematosus

Mouth

=Oral lichenoid drug reaction

= Lichenoid drug eruptions may be caused by a variety of systemic medications and share clinical features with oral LP. Histologic findings of a deep mixed infiltrate with

lymphocyte A lymphocyte is a type of white blood cell (leukocyte) in the immune system of most vertebrates. Lymphocytes include natural killer cells (which function in cell-mediated, cytotoxic innate immunity), T cells (for cell-mediated, cytotoxic ad ...

plasma cell Plasma cells, also called plasma B cells or effector B cells, are white blood cells that originate in the lymphoid organs as B lymphocytes and secrete large quantities of proteins called antibodies in response to being presented specific substan ...

s, and

neutrophil Neutrophils (also known as neutrocytes or heterophils) are the most abundant type of granulocytes and make up 40% to 70% of all white blood cells in humans. They form an essential part of the innate immune system, with their functions varying in ...

s (with or without eosinophils) and perivascular inflammation favor this diagnosis.

=Oral lichenoid contact reaction (allergic contact mucositis)

= Oral lichenoid contact reactions may be caused by a variety of substances. The clinical and histologic features of oral lichenoid contact reactions are similar to oral LP. Patch testing and recognition of the proximity of an offending substance to the eruption can aid with diagnosis.

=Autoimmune blistering diseases

Mucous membrane pemphigoid Mucous membrane pemphigoid is a rare chronic autoimmune subepithelial blistering disease characterized by erosive lesions of the mucous membranes and skin. It is one of the pemphigoid diseases that can result in scarring. Signs and symptoms Th ...

and other autoimmune blistering diseases may present with oral erosions and

desquamative gingivitis Desquamative gingivitis is an erythematous (red), desquamatous (shedding) and ulcerated appearance of the gums. It is a descriptive term and can be caused by several different disorders. Signs and symptoms Desquamative gingivitis involves lesions ...

similar to that seen in erosive LP. Biopsies for routine histologic examination and direct immunofluorescence are useful for distinguishing these disorders from oral LP.

=Graft-versus-host disease (GVHD)

= Lacy, reticulated plaques or erosions that resemble oral LP may occur in GVHD. The histologic findings of these disorders are also similar. The patient history is useful for differentiating chronic GVHD from oral LP. Oral involvement in acute GVHD is less well characterized than chronic GVHD, but has been associated with erythematous, erosive, ulcerative, or lichenoid oral lesions.

=Leukoplakia

Leukoplakia Oral leukoplakia is a ''potentially malignant disorder'' affecting the oral mucosa. It is defined as "essentially an oral mucosal white lesion that cannot be considered as any other definable lesion." Oral leukoplakia is a white patch or plaque th ...

is a manifestation of squamous epithelial

hyperplasia Hyperplasia (from ancient Greek ὑπέρ ''huper'' 'over' + πλάσις ''plasis'' 'formation'), or hypergenesis, is an enlargement of an organ or tissue caused by an increase in the amount of organic tissue that results from cell proliferati ...

that may be a precursor to oral squamous cell carcinoma. White patches or plaques usually appear on the oral mucosa. To rule out malignancy, a biopsy of leukoplakia is indicated.

=Oral squamous cell carcinoma

= Oral squamous cell carcinoma (SCC) can present as erythematous or white patches, ulcers, or exophytic masses. The highest risk for oral SCC may occur in patients with erythematous or erosive oral LP. A biopsy is indicated.

=Leukoedema

Leukoedema Leukoedema is a blue, grey or white appearance of mucosae, particularly the buccal mucosa (the inside of the cheeks); it may also occur on the mucosa of the larynx or vagina. It is a harmless and very common condition. Because it is so common, it ...

is a common, benign finding in the oral cavity that presents as white-gray, somewhat translucent plaques on the mucosa. The

buccal mucosa The oral mucosa is the mucous membrane lining the inside of the mouth. It comprises stratified squamous epithelium, termed "oral epithelium", and an underlying connective tissue termed ''lamina propria''. The oral cavity has sometimes been describ ...

is the most common site for involvement. Symptoms are absent, and no treatment is necessary.

=Oropharyngeal candidiasis

= Oropharyngeal

candidiasis Candidiasis is a fungal infection due to any type of '' Candida'' (a type of yeast). When it affects the mouth, in some countries it is commonly called thrush. Signs and symptoms include white patches on the tongue or other areas of the mouth ...

(also known as thrush) is a common infection that has a predilection for infants, older adults with dentures, immunosuppressed individuals, and individuals utilizing intraoral

corticosteroid Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are involv ...

therapy. Patients present with white plaques or erythematous patches on the buccal mucosa, palate, tongue, or oropharynx that may be mistaken for reticular LP.

Histopathology

The histologic findings of oral LP can offer strong support for the diagnosis, but are not pathognomonic. Clinical correlation is required. Common histologic findings of oral LP include: *

Parakeratosis Parakeratosis is a mode of keratinization characterized by the retention of nuclei in the stratum corneum. In mucous membranes, parakeratosis is normal.Kumar, Vinay; Fausto, Nelson; Abbas, Abul (2010) ''Robbins & Cotran Pathologic Basis of Disease' ...

and slight acanthosis of the epithelium * Saw-toothed rete ridges * Liquefaction (hydropic) degeneration of the

basal layer The ''stratum basale'' (basal layer, sometimes referred to as ''stratum germinativum'') is the deepest layer of the five layers of the epidermis, the external covering of skin in mammals. The ''stratum basale'' is a single layer of columnar or ...

with apoptotic keratinocytes (referred to as Civatte, colloid, hyaline, or cytoid bodies) * An amorphous band of eosinophilic material at the basement membrane composed of fibrin or fibrinogen. * A lichenoid (band-like) lymphocytic infiltrate immediately subjacent to the epithelium. Nonetheless, interpreting the histopathological features of oral LP has been associated historically with high intra-observer and inter-observer variabilities.

Treatment

There is no cure for lichen planus, and so treatment of cutaneous and oral lichen planus is for symptomatic relief or due to cosmetic concerns. When medical treatment is pursued, first-line treatment typically involves either topical or systemic

s, and removal of any triggers. Without treatment, most lesions will spontaneously resolve within 6–9 months for cutaneous lesions, and longer for mucosal lesions.

Skin

Many different treatments have been reported for cutaneous lichen planus, however there is a general lack of evidence of efficacy for any treatment. Treatments tend to be prolonged, partially effective and disappointing. The mainstay of localized skin lesions is topical steroids. Additional treatments include

retinoid The retinoids are a class of chemical compounds that are vitamers of vitamin A or are chemically related to it. Retinoids have found use in medicine where they regulate epithelial cell growth. Retinoids have many important functions throughout t ...

s, such as

acitretin Acitretin (trade names Soriatane and Neotigason) is a second-generation retinoid. It is taken orally, and is typically used for psoriasis. Acitretin is an oral retinoid used in the treatment of severe resistant psoriasis. Because of the potentia ...

, or

sulfasalazine Sulfasalazine, sold under the brand name Azulfidine among others, is a medication used to treat rheumatoid arthritis, ulcerative colitis, and Crohn's disease. It is considered by some to be a first-line treatment in rheumatoid arthritis. It is ...

. Narrow band UVB phototherapy or systemic

PUVA therapy PUVA (psoralen and UVA) is an ultraviolet light therapy treatment for skin diseases: eczema, psoriasis, graft-versus-host disease, vitiligo, mycosis fungoides, large plaque parapsoriasis, and cutaneous T-cell lymphoma, using the sensitizing effec ...

are known treatment modalities for generalized disease.

Mouth

Reassurance that the condition is benign, elimination of precipitating factors and improving

oral hygiene Oral hygiene is the practice of keeping one's mouth clean and free of disease and other problems (e.g. bad breath) by regular brushing of the teeth (dental hygiene) and cleaning between the teeth. It is important that oral hygiene be carried out ...

are considered initial management for symptomatic OLP, and these measures are reported to be useful. Treatment usually involves

topical A topical medication is a medication that is applied to a particular place on or in the body. Most often topical medication means application to body surfaces such as the skin or mucous membranes to treat ailments via a large range of classes ...

corticosteroids Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are involve ...

(such as

betamethasone Betamethasone is a steroid medication. It is used for a number of diseases including rheumatic disorders such as rheumatoid arthritis and systemic lupus erythematosus, skin diseases such as dermatitis and psoriasis, allergic conditions such ...

clobetasol Clobetasol is a synthetic glucocorticoid corticosteroid. A propionate ester In chemistry, an ester is a compound derived from an oxoacid (organic or inorganic) in which at least one hydroxyl group () is replaced by an alkoxy group (), a ...

dexamethasone Dexamethasone is a glucocorticoid medication used to treat rheumatic problems, a number of skin diseases, severe allergies, asthma, chronic obstructive lung disease, croup, brain swelling, eye pain following eye surgery, superior vena cav ...

, and

triamcinolone Triamcinolone is a glucocorticoid used to treat certain skin diseases, allergies, and rheumatic disorders among others. It is also used to prevent worsening of asthma and COPD. It can be taken in various ways including by mouth, injection into ...

) and

analgesic An analgesic drug, also called simply an analgesic (American English), analgaesic (British English), pain reliever, or painkiller, is any member of the group of drugs used to achieve relief from pain (that is, analgesia or pain management). It ...

s, or if these are ineffective and the condition is severe, then systemic corticosteroids may be used.

Calcineurin inhibitor Immunosuppressive drugs, also known as immunosuppressive agents, immunosuppressants and antirejection medications, are drugs that inhibit or prevent activity of the immune system. Classification Immunosuppressive drugs can be classified int ...

s (such as

pimecrolimus Pimecrolimus is an immunomodulating agent of the calcineurin inhibitor class used in the treatment of atopic dermatitis (eczema). It is available as a topical cream, once marketed by Novartis (however, Galderma has been promoting the compound in ...

tacrolimus Tacrolimus, sold under the brand name Prograf among others, is an immunosuppressive drug. After allogeneic organ transplant, the risk of organ rejection is moderate. To lower the risk of organ rejection, tacrolimus is given. The drug can also ...

cyclosporin Ciclosporin, also spelled cyclosporine and cyclosporin, is a calcineurin inhibitor, used as an immunosuppressant medication. It is a natural product. It is taken orally or intravenously for rheumatoid arthritis, psoriasis, Crohn's disease ...

) are sometimes used. While topical steroids are widely accepted as first line treatment for mucosal lichen planus, there is only weak evidence to support their effectiveness for erosive oral lichen planus.

Prognosis

Cutaneous lichen planus lesions typically resolve within six months to a year. However, some variant such as the hypertrophic variant might persist for years if left untreated or unmonitored. It is found that cutaneous lichen planus does not carry a risk of

skin cancer Skin cancers are cancers that arise from the skin. They are due to the development of abnormal cells that have the ability to invade or spread to other parts of the body. There are three main types of skin cancers: basal-cell skin cancer (BCC) ...

. In contrast to cutaneous LP, which is self limited, lichen planus lesions in the mouth may persist for many years, and tend to be difficult to treat, with relapses being common. Although this condition was first described almost a century ago, it has been reported that its associated oral cancer risk has been exaggerated. Overall, it is found that patients with erythematous or erosive oral lichen planus have a higher risk of oral squamous cell carcinoma compared to patients diagnosed with other variants. Due to the possibility that oral LP may increase risk for

oral cancer Oral cancer, also known as mouth cancer, is cancer of the lining of the lips, mouth, or upper throat. In the mouth, it most commonly starts as a painless white patch, that thickens, develops red patches, an ulcer, and continues to grow. When on ...

, patients with oral lichen planus are encouraged to avoid activities known to increase the risk for oral cancer, such as smoking and alcohol use. Patients with oral lichen planus should be followed-up at least every 6 to 12 months, to assess the disease activity, changes in symptoms or even detect early signs of

malignancy Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous ''benign'' tumor in that a malignancy is not s ...

Epidemiology

The overall estimated

prevalence In epidemiology, prevalence is the proportion of a particular population found to be affected by a medical condition (typically a disease or a risk factor such as smoking or seatbelt use) at a specific time. It is derived by comparing the number o ...

of lichen planus in worldwide population is in the range of 0.2% to 5%. It generally occurs more commonly in females, in a ratio of 3:2, and most cases are diagnosed between the ages of 30 and 60, but it can occur at any age. Lichen planus can occur in patients as diverse cutaneous manifestations alone or in combination with mucosal lichen planus and, or lichen planus of the nails. Study shows that frequency of mucosal involvement of lichen planus patients is 30- 70%. Oral lichen planus is relatively common, It is one of the most common mucosal diseases. The

in the general population is about 1.27–2.0%, and it occurs more commonly in middle aged people. Oral lichen planus in children is rare. About 50% of females with oral lichen planus were reported to have undiagnosed vulvar lichen planus. Some studies suggest that cutaneous lichen planus is more commonly found in men whilst oral lichen planus lesions are more commonly found in women.

History

Lichen planus was first reported in 1869 by

Erasmus Wilson Sir William James Erasmus Wilson FRCS FRS (25 November 18097 August 1884), generally known as Sir Erasmus Wilson, was an English surgeon and dermatologist. Biography Wilson was born in London, studied at Dartford Grammar School before St Ba ...

. The origin of the word is believed to be from the Greek word ''Leichen'', which means tree moss, and also from Latin word ''planus'', which means flat and even surface. Dr Wilson explained the condition as an inflammatory disorder with unknown etiology. Initially, the characteristic surface markings or striae was described by Weyl in 1885. In 1895, Wickham further explained the characteristic of the lesion, now known as Wickham striae. Further on, Darier explained the presence of such characteristic markings by correlating with an increase thickness of the granular cell layer. The coexistence of oral, cervical and stomach lichen planus lesions were described by Guogerot and Burnier in 1937. A similar variant of mucosal lichen planus as the vulvovaginal-gingival syndrome with erosive lesions involving oral and vulvovaginal mucosa were introduced by Pelisse and colleagues in year 1982.

Research

Apremilast Apremilast, sold under the brand name Otezla among others, is a medication for the treatment of certain types of psoriasis and psoriatic arthritis. It may also be useful for other immune system-related inflammatory diseases. The drug acts as a se ...

is undergoing investigation as a potential treatment.

Notes

References

External links

* {{DEFAULTSORT:Lichen Planus Autoimmune diseases Conditions of the mucous membranes Hepatitis C virus-associated diseases Lichenoid eruptions Oral mucosal pathology