Lichen planus (LP) is an idiopathic, cell-mediated inflammatory skin disease. It is characterized by pruritic, flat-topped, papular, purple skin lesions commonly found on the flexural surfaces of the extremities. Other areas affected include genitalia, nails, scalp, and mucous membranes. Exact etiology is unknown but has been found to be associated with hepatitis C infection, other diseases, and multiple drugs. Skin biopsy of the most prominent lesion is used for confirmation of the diagnosis. Cutaneous LP usually resolves in 1–2 years. Other forms, however, are chronic and persistent. Topical corticosteroids are the preferred treatment.
Lichen planusLichen planusLichen planus (LP) is an idiopathic, cell-mediated inflammatory skin disease. It is characterized by pruritic, flat-topped, papular, purple skin lesions commonly found on the flexural surfaces of the extremities. Other areas affected include genitalia, nails, scalp, and mucous membranes. Lichen Planus (LP) affects < 1% of the population in the United States.
Can occur at any age, but most commonly found in patientsPatientsIndividuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures.Clinician–Patient Relationship 30–60 years of age
Rare in children
Etiology[1–3,5,14]
Unknown
Associated risk factors:
Genetic predisposition: ↑ frequency of human leukocyte antigenAntigenSubstances that are recognized by the immune system and induce an immune reaction.Vaccination B7 (HLA-B7), HLA-DR1, and HLA-DR10 in those affected
Hepatitis CHepatitis CHepatitis C is an infection of the liver caused by the hepatitis C virus (HCV). The infection can be transmitted through infectious blood or body fluids and may be transmitted during childbirth or through IV drug use or sexual intercourse. Hepatitis C virus can cause both acute and chronic hepatitis, ranging from a mild to a serious, lifelong illness including liver cirrhosis and hepatocellular carcinoma (HCC).Hepatitis C VirusvirusVirusViruses are infectious, obligate intracellular parasites composed of a nucleic acid core surrounded by a protein capsid. Viruses can be either naked (non-enveloped) or enveloped. The classification of viruses is complex and based on many factors, including type and structure of the nucleoid and capsid, the presence of an envelope, the replication cycle, and the host range. Virology infection
Drug exposure:
Angiotensin-converting enzyme (ACE) inhibitors
Beta-blockersBeta-blockersDrugs that bind to but do not activate beta-adrenergic receptors thereby blocking the actions of beta-adrenergic agonists. Adrenergic beta-antagonists are used for treatment of hypertension, cardiac arrhythmias, angina pectoris, glaucoma, migraine headaches, and anxiety.Class 2 Antiarrhythmic Drugs (Beta Blockers)
Methyldopa
QuinidineQuinidineAn optical isomer of quinine, extracted from the bark of the cinchona tree and similar plant species. This alkaloid dampens the excitability of cardiac and skeletal muscles by blocking sodium and potassium currents across cellular membranes. It prolongs cellular action potentials, and decreases automaticity. Quinidine also blocks muscarinic and alpha-adrenergic neurotransmission.Class 1 Antiarrhythmic Drugs (Sodium Channel Blockers)
ChloroquineChloroquineThe prototypical antimalarial agent with a mechanism that is not well understood. It has also been used to treat rheumatoid arthritis, systemic lupus erythematosus, and in the systemic therapy of amebic liver abscesses.Antimalarial Drugs
HydroxychloroquineHydroxychloroquineA chemotherapeutic agent that acts against erythrocytic forms of malarial parasites. Hydroxychloroquine appears to concentrate in food vacuoles of affected protozoa. It inhibits plasmodial heme polymerase.Immunosuppressants
ThiazideThiazideHeterocyclic compounds with sulfur and nitrogen in the ring. This term commonly refers to the benzothiadiazines that inhibit sodium-potassium-chloride symporters and are used as diuretics.HyponatremiadiureticsDiureticsAgents that promote the excretion of urine through their effects on kidney function.Heart Failure and Angina Medication
Gold salts
Primary biliary cirrhosisBiliary cirrhosisFibrosis of the hepatic parenchyma due to obstruction of bile flow (cholestasis) in the intrahepatic or extrahepatic bile ducts. Primary biliary cholangitis involves the destruction of small intrahepatic bile ducts and decreased bile secretion. Secondary biliary cholangitis is produced by prolonged obstruction of large intrahepatic or extrahepatic bile ducts from a variety of causes.Cystic Fibrosis
Human herpesvirus type 7
MercuryMercuryA silver metallic element that exists as a liquid at room temperature. It has the atomic symbol Hg (from hydrargyrum, liquid silver), atomic number 80, and atomic weight 200. 59. Mercury is used in many industrial applications and its salts have been employed therapeutically as purgatives, antisyphilitics, disinfectants, and astringents. It can be absorbed through the skin and mucous membranes which leads to mercury poisoning. Because of its toxicity, the clinical use of mercury and mercurials is diminishing.Renal Tubular Acidosis (in dental amalgam)
Radiotherapy
Psychogenic factors, such as anxietyAnxietyFeelings or emotions of dread, apprehension, and impending disaster but not disabling as with anxiety disorders.Generalized Anxiety Disorder, depression, and stress
Pathophysiology
Proposed mechanism involves cell-mediated immunityCell-mediated immunityManifestations of the immune response which are mediated by antigen-sensitized T-lymphocytes via lymphokines or direct cytotoxicity. This takes place in the absence of circulating antibody or where antibody plays a subordinate role.Squamous Cell Carcinoma (SCC).[1,5,8]
Exposure to exogenous agent (drug, virusVirusViruses are infectious, obligate intracellular parasites composed of a nucleic acid core surrounded by a protein capsid. Viruses can be either naked (non-enveloped) or enveloped. The classification of viruses is complex and based on many factors, including type and structure of the nucleoid and capsid, the presence of an envelope, the replication cycle, and the host range. Virology, other risk factors) → alters epidermal self-antigens
CD4+ and CD8+ (more predominant) lymphocytesLymphocytesLymphocytes are heterogeneous WBCs involved in immune response. Lymphocytes develop from the bone marrow, starting from hematopoietic stem cells (HSCs) and progressing to common lymphoid progenitors (CLPs). B and T lymphocytes and natural killer (NK) cells arise from the lineage.Lymphocytes: Histology are recruited to dermal-epidermal junction → lead a T-cell–mediated attack → apoptosisApoptosisA regulated cell death mechanism characterized by distinctive morphologic changes in the nucleus and cytoplasm, including the endonucleolytic cleavage of genomic DNA, at regularly spaced, internucleosomal sites, I.e., DNA fragmentation. It is genetically-programmed and serves as a balance to mitosis in regulating the size of animal tissues and in mediating pathologic processes associated with tumor growth.Ischemic Cell Damage of keratinocytesKeratinocytesEpidermal cells which synthesize keratin and undergo characteristic changes as they move upward from the basal layers of the epidermis to the cornified (horny) layer of the skin. Successive stages of differentiation of the keratinocytes forming the epidermal layers are basal cell, spinous or prickle cell, and the granular cell.Skin: Structure and Functions
Basal layer is injured → release melaninMelaninInsoluble polymers of tyrosine derivatives found in and causing darkness in skin (skin pigmentation), hair, and feathers providing protection against sunburn induced by sunlight. Carotenes contribute yellow and red coloration.Seborrheic Keratosis into dermisDermisA layer of vascularized connective tissue underneath the epidermis. The surface of the dermis contains innervated papillae. Embedded in or beneath the dermis are sweat glands; hair follicles; and sebaceous glands.Skin: Structure and Functions → hyperpigmentationHyperpigmentationExcessive pigmentation of the skin, usually as a result of increased epidermal or dermal melanin pigmentation, hypermelanosis. Hyperpigmentation can be localized or generalized. The condition may arise from exposure to light, chemicals or other substances, or from a primary metabolic imbalance.Malassezia Fungi
Interaction of T lymphocytesT lymphocytesLymphocytes responsible for cell-mediated immunity. Two types have been identified – cytotoxic (t-lymphocytes, cytotoxic) and helper T-lymphocytes (t-lymphocytes, helper-inducer). They are formed when lymphocytes circulate through the thymus gland and differentiate to thymocytes. When exposed to an antigen, they divide rapidly and produce large numbers of new T cells sensitized to that antigen.T cells: Types and Functions and basal keratinocytesKeratinocytesEpidermal cells which synthesize keratin and undergo characteristic changes as they move upward from the basal layers of the epidermis to the cornified (horny) layer of the skin. Successive stages of differentiation of the keratinocytes forming the epidermal layers are basal cell, spinous or prickle cell, and the granular cell.Skin: Structure and Functions enhanced by:
Intercellular adhesionAdhesionThe process whereby platelets adhere to something other than platelets, e.g., collagen; basement membrane; microfibrils; or other ‘foreign’ surfaces.Coagulation Studies molecule-1 (ICAM-1) upregulation by basal keratinocytesKeratinocytesEpidermal cells which synthesize keratin and undergo characteristic changes as they move upward from the basal layers of the epidermis to the cornified (horny) layer of the skin. Successive stages of differentiation of the keratinocytes forming the epidermal layers are basal cell, spinous or prickle cell, and the granular cell.Skin: Structure and Functions
Other cytokinesCytokinesNon-antibody proteins secreted by inflammatory leukocytes and some non-leukocytic cells, that act as intercellular mediators. They differ from classical hormones in that they are produced by a number of tissue or cell types rather than by specialized glands. They generally act locally in a paracrine or autocrine rather than endocrine manner.Adaptive Immune Response: interferon (IFNIFNInterferon (IFN) is a cytokine with antiviral properties (it interferes with viral infections) and various roles in immunoregulation. The different types are type I IFN (IFN-ɑ and IFN-β), type II IFN (IFN-ɣ), and type III IFN (IFN-ƛ).Interferons)-gamma, tumorTumorInflammationnecrosisNecrosisThe death of cells in an organ or tissue due to disease, injury or failure of the blood supply.Ischemic Cell Damage factor (TNFTNFTumor necrosis factor (TNF) is a major cytokine, released primarily by macrophages in response to stimuli. The presence of microbial products and dead cells and injury are among the stimulating factors. This protein belongs to the TNF superfamily, a group of ligands and receptors performing functions in inflammatory response, morphogenesis, and cell proliferation. Tumor Necrosis Factor (TNF))-alpha, interleukin (IL)-1 alpha, IL-6, and IL-8
Development of lesions in previously healthy sites
Due to trauma from scratching
HyperpigmentationHyperpigmentationExcessive pigmentation of the skin, usually as a result of increased epidermal or dermal melanin pigmentation, hypermelanosis. Hyperpigmentation can be localized or generalized. The condition may arise from exposure to light, chemicals or other substances, or from a primary metabolic imbalance.Malassezia Fungi occurs with healed lesions.
May be painful depending on location
Distribution and location:
Symmetric and bilateral
Most commonly found on flexural surfaces of extremities (ankles and wrists)
Clinical course:
Most resolve within 1–2 years
May leave permanent hyperpigmented macules after active disease is cleared
Cutaneous variants by morphology[5,6,8]
Hypertrophic:
Lichen planusLichen planusLichen planus (LP) is an idiopathic, cell-mediated inflammatory skin disease. It is characterized by pruritic, flat-topped, papular, purple skin lesions commonly found on the flexural surfaces of the extremities. Other areas affected include genitalia, nails, scalp, and mucous membranes. Lichen Planus verrucosus
Seen in patientsPatientsIndividuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures.Clinician–Patient Relationship of Middle Eastern descent
Plaques are well-demarcated pale or violaceous papules with superficial atrophyAtrophyDecrease in the size of a cell, tissue, organ, or multiple organs, associated with a variety of pathological conditions such as abnormal cellular changes, ischemia, malnutrition, or hormonal changes.Cellular Adaptation.
Note: Additional subtypes related to lesion distribution exist (e.g., blaschkoid LP, zosteriform LP).[5]
Oral LP[5–7,13]
Clinical features:
Can occur independently or with cutaneous LP
Can present as:
Wickham’s striae or reticular form (lacy, web-like white threads)
Atrophic lesions
Bullous lesions
Painful erosionsErosionsCorneal Abrasions, Erosion, and Ulcers or ulcers (erosive mucosal lichen planusLichen planusLichen planus (LP) is an idiopathic, cell-mediated inflammatory skin disease. It is characterized by pruritic, flat-topped, papular, purple skin lesions commonly found on the flexural surfaces of the extremities. Other areas affected include genitalia, nails, scalp, and mucous membranes. Lichen Planus) that can lead to secondary CandidaCandidaCandida is a genus of dimorphic, opportunistic fungi. Candida albicans is part of the normal human flora and is the most common cause of candidiasis. The clinical presentation varies and can include localized mucocutaneous infections (e.g., oropharyngeal, esophageal, intertriginous, and vulvovaginal candidiasis) and invasive disease (e.g., candidemia, intraabdominal abscess, pericarditis, and meningitis). Candida/Candidiasis infection and/or loss of appetite due to painPainAn unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons.Pain: Types and Pathways
Also affects alveolar mucosa and tongueTongueThe tongue, on the other hand, is a complex muscular structure that permits tasting and facilitates the process of mastication and communication. The blood supply of the tongue originates from the external carotid artery, and the innervation is through cranial nerves.Lips and Tongue: Anatomy
Clinical course: a chronic form of LP
Mucosal and paramucosal lesions, especially at oral and vulvar sites (but not cutaneous lesions) have an increased risk of squamous cell carcinomaSquamous cell carcinomaCutaneous squamous cell carcinoma (cSCC) is caused by malignant proliferation of atypical keratinocytes. This condition is the 2nd most common skin malignancy and usually affects sun-exposed areas of fair-skinned patients. The cancer presents as a firm, erythematous, keratotic plaque or papule. Squamous Cell Carcinoma (SCC).
Lichen planus presenting as Wickham’s striae on the oral mucosa
In women, vulvaVulvaThe vulva is the external genitalia of the female and includes the mons pubis, labia majora, labia minora, clitoris, vestibule, vestibular bulb, and greater vestibular glands. Vagina, Vulva, and Pelvic Floor: Anatomy and vaginaVaginaThe vagina is the female genital canal, extending from the vulva externally to the cervix uteri internally. The structures have sexual, reproductive, and urinary functions and a rich blood supply, mainly arising from the internal iliac artery.Vagina, Vulva, and Pelvic Floor: Anatomy are affected.
Associated with pruritusPruritusAn intense itching sensation that produces the urge to rub or scratch the skin to obtain relief.Atopic Dermatitis (Eczema) and dyspareuniaDyspareuniaRecurrent genital pain occurring during, before, or after sexual intercourse in either the male or the female.Primary Ovarian Insufficiency
Increased risk of squamous cell carcinomaSquamous cell carcinomaCutaneous squamous cell carcinoma (cSCC) is caused by malignant proliferation of atypical keratinocytes. This condition is the 2nd most common skin malignancy and usually affects sun-exposed areas of fair-skinned patients. The cancer presents as a firm, erythematous, keratotic plaque or papule. Squamous Cell Carcinoma (SCC)
LP of the scalp that leads to keratotic follicular papules
Affects women more than men
Can progress to scarringScarringInflammationalopeciaAlopeciaAlopecia is the loss of hair in areas anywhere on the body where hair normally grows. Alopecia may be defined as scarring or non-scarring, localized or diffuse, congenital or acquired, reversible or permanent, or confined to the scalp or universal; however, alopecia is usually classified using the 1st 3 factors.Alopecia if left untreated
Esophageal:
Can be asymptomatic or present with dysphagiaDysphagiaDysphagia is the subjective sensation of difficulty swallowing. Symptoms can range from a complete inability to swallow, to the sensation of solids or liquids becoming “stuck.” Dysphagia is classified as either oropharyngeal or esophageal, with esophageal dysphagia having 2 sub-types: functional and mechanical. Dysphagia or odynophagiaOdynophagiaEpiglottitis
Affects external auditory canals and tympanic membranes
Presents with erythemaErythemaRedness of the skin produced by congestion of the capillaries. This condition may result from a variety of disease processes.Chalazion, pruritusPruritusAn intense itching sensation that produces the urge to rub or scratch the skin to obtain relief.Atopic Dermatitis (Eczema), and indurationIndurationDermatologic Examination; but can cause persistent otorrheaOtorrheaOtitis Externa and hearing lossHearing lossHearing loss, also known as hearing impairment, is any degree of impairment in the ability to apprehend sound as determined by audiometry to be below normal hearing thresholds. Clinical presentation may occur at birth or as a gradual loss of hearing with age, including a short-term or sudden loss at any point. Hearing Loss
Diagnosis
Definitive diagnosis is made based on typical morphologic lesions on exam, in combination with associated histopathologic findings at the affected site.[8,9]
History and clinical findings[5,8]
Review of medical history, medications, and risk factors (e.g., hepatitis CHepatitis CHepatitis C is an infection of the liver caused by the hepatitis C virus (HCV). The infection can be transmitted through infectious blood or body fluids and may be transmitted during childbirth or through IV drug use or sexual intercourse. Hepatitis C virus can cause both acute and chronic hepatitis, ranging from a mild to a serious, lifelong illness including liver cirrhosis and hepatocellular carcinoma (HCC).Hepatitis C Virus)
Physical examination of entire cutaneous surface (including the scalp, oral cavity, and external genitalia)
Mucocutaneous biopsyBiopsyRemoval and pathologic examination of specimens from the living body.Ewing Sarcoma[5,6,8]
Civatte bodies (anucleate, necrotic hypereosinophilic keratinocytesKeratinocytesEpidermal cells which synthesize keratin and undergo characteristic changes as they move upward from the basal layers of the epidermis to the cornified (horny) layer of the skin. Successive stages of differentiation of the keratinocytes forming the epidermal layers are basal cell, spinous or prickle cell, and the granular cell.Skin: Structure and Functions) in the lower epidermisEpidermisThe external, nonvascular layer of the skin. It is made up, from within outward, of five layers of epithelium: (1) basal layer (stratum basale epidermidis); (2) spinous layer (stratum spinosum epidermidis); (3) granular layer (stratum granulosum epidermidis); (4) clear layer (stratum lucidum epidermidis); and (5) horny layer (stratum corneum epidermidis).Skin: Structure and Functions
Wedge-shaped hypergranulosis (hyperplasiaHyperplasiaAn increase in the number of cells in a tissue or organ without tumor formation. It differs from hypertrophy, which is an increase in bulk without an increase in the number of cells.Cellular Adaptation of the stratum granulosumStratum granulosumSkin: Structure and Functions)
Vacuolar alteration of basal layer of epidermisEpidermisThe external, nonvascular layer of the skin. It is made up, from within outward, of five layers of epithelium: (1) basal layer (stratum basale epidermidis); (2) spinous layer (stratum spinosum epidermidis); (3) granular layer (stratum granulosum epidermidis); (4) clear layer (stratum lucidum epidermidis); and (5) horny layer (stratum corneum epidermidis).Skin: Structure and Functions
Lichen planus: epidermis shows hyperkeratosis, hypergranulosis, acanthosis, and saw-tooth rete ridges. Dermis shows a band-like lymphocytic infiltrate touching the epidermis (H&E stain, ×40).
Image: “Lichen planus” by Department of Pathology, JJ Hospital, Mumbai, India. License: CC BY 2.0
Histopathology of a skin biopsy of an LP plaque showing dense lichenoid inflammatory infiltrates underneath the lamina propria. Note the inflammation at the dermal–epidermal junction and an angulated zigzag lower contour, which gives it the saw-tooth appearance characteristic of LP. Note also the thickening of the granular cell layer (hypergranulosis) and the stratum corneum (hyperkeratosis), which are more pronounced in the white lines of Wickham’s striae.
Image: “Histopathology of lichen planus” by Shi G, Sohn KC et al. License: CC BY 4.0, edited by Lecturio.
Other tests[5,8]
Direct immunofluorescence:
May show:
Deposits of:
ImmunoglobulinsImmunoglobulinsImmunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution.Immunoglobulins: Types and Functions (especially IgMIgMA class of immunoglobulin bearing mu chains (immunoglobulin mu-chains). Igm can fix complement. The name comes from its high molecular weight and originally being called a macroglobulin.Immunoglobulins: Types and Functions)
Complement
FibrinogenFibrinogenPlasma glycoprotein clotted by thrombin, composed of a dimer of three non-identical pairs of polypeptide chains (alpha, beta, gamma) held together by disulfide bonds. Fibrinogen clotting is a sol-gel change involving complex molecular arrangements: whereas fibrinogen is cleaved by thrombin to form polypeptides a and b, the proteolytic action of other enzymes yields different fibrinogen degradation products.Hemostasis
Apoptotic keratinocytesKeratinocytesEpidermal cells which synthesize keratin and undergo characteristic changes as they move upward from the basal layers of the epidermis to the cornified (horny) layer of the skin. Successive stages of differentiation of the keratinocytes forming the epidermal layers are basal cell, spinous or prickle cell, and the granular cell.Skin: Structure and Functions
Helpful in distinguishing erosive LP from pemphigus vulgarisPemphigus vulgarisBullous pemphigoid and pemphigus vulgaris are two different blistering autoimmune diseases. In pemphigus vulgaris, autoantibodies attack the desmosomal proteins, which connect the keratinocytes to one another. This attack results in a more severe, potentially fatal condition with fragile, flaccid blisters, usually with significant mucosal involvement. Bullous Pemphigoid and Pemphigus Vulgaris
DermoscopyDermoscopyA noninvasive technique that enables direct microscopic examination of the surface and architecture of the skin.Seborrheic Keratosis may show:
White crossing lines (Wickham sign)
Dull, red background
Peripheral vessels
Laboratory tests: screen for hepatitis CHepatitis CHepatitis C is an infection of the liver caused by the hepatitis C virus (HCV). The infection can be transmitted through infectious blood or body fluids and may be transmitted during childbirth or through IV drug use or sexual intercourse. Hepatitis C virus can cause both acute and chronic hepatitis, ranging from a mild to a serious, lifelong illness including liver cirrhosis and hepatocellular carcinoma (HCC).Hepatitis C Virus in patientsPatientsIndividuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures.Clinician–Patient Relationship with risk factors
Management
Management may vary based on location. The following recommendations are based on US, UK, and European literature and guidelines. Specialist consultation (e.g., dermatology) is recommended to help guide therapy.
Cutaneous LP
Classical cutaneous LP may resolve spontaneously (6 months to 2 years). However, the following treatments may be used to alleviate symptoms and speed up resolution. There is some variation in 1st– and 2nd–line treatments between European and US literature.[6,8]
Supportive care:[6,8]
Oral antihistaminesAntihistaminesAntihistamines are drugs that target histamine receptors, particularly H1 and H2 receptors. H1 antagonists are competitive and reversible inhibitors of H1 receptors. First-generation antihistamines cross the blood-brain barrier and can cause sedation. Antihistamines
Topical antipruritics, as needed (mentholMentholA monoterpene cyclohexanol produced from mint oils.Pityriasis Rosea, camphor, doxepinDoxepinA dibenzoxepin tricyclic compound. It displays a range of pharmacological actions including maintaining adrenergic innervation. Its mechanism of action is not fully understood, but it appears to block reuptake of monoaminergic neurotransmitters into presynaptic terminals. It also possesses anticholinergic activity and modulates antagonism of histamine h(1)- and h(2)-receptors.Tricyclic Antidepressants topical local anestheticsAnestheticsAgents that are capable of inducing a total or partial loss of sensation, especially tactile sensation and pain. They may act to induce general anesthesia, in which an unconscious state is achieved, or may act locally to induce numbness or lack of sensation at a targeted site.Anesthesiology: History and Basic Concepts)
1st–line treatment:[5,8,10]
Topical steroidsSteroidsA group of polycyclic compounds closely related biochemically to terpenes. They include cholesterol, numerous hormones, precursors of certain vitamins, bile acids, alcohols (sterols), and certain natural drugs and poisons. Steroids have a common nucleus, a fused, reduced 17-carbon atom ring system, cyclopentanoperhydrophenanthrene. Most steroids also have two methyl groups and an aliphatic side-chain attached to the nucleus.Benign Liver Tumors (superpotent and potent):
TriamcinoloneTriamcinoloneA glucocorticoid given, as the free alcohol or in esterified form, orally, intramuscularly, by local injection, by inhalation, or applied topically in the management of various disorders in which corticosteroids are indicated.Glucocorticoids acetonide
ClobetasolClobetasolA derivative of prednisolone with high glucocorticoid activity and low mineralocorticoid activity. Absorbed through the skin faster than fluocinonide, it is used topically in treatment of psoriasis but may cause marked adrenocortical suppression.Glucocorticoids propionate
Fluocinolone acetonide
BetamethasoneBetamethasoneA glucocorticoid given orally, parenterally, by local injection, by inhalation, or applied topically in the management of various disorders in which corticosteroids are indicated. Its lack of mineralocorticoid properties makes betamethasone particularly suitable for treating cerebral edema and congenital adrenal hyperplasia.Glucocorticoids dipropionate
Intralesional injections:
May be helpful for:
Discrete lesions
Hypertrophic lesions
Option: triamcinoloneTriamcinoloneA glucocorticoid given, as the free alcohol or in esterified form, orally, intramuscularly, by local injection, by inhalation, or applied topically in the management of various disorders in which corticosteroids are indicated.Glucocorticoids
2nd–line treatment:[5,6,8,10]
For lesions unresponsive to 1st-line or for severe LP
Oral: prednisonePrednisoneA synthetic anti-inflammatory glucocorticoid derived from cortisone. It is biologically inert and converted to prednisolone in the liver.Immunosuppressants
IM: triamcinoloneTriamcinoloneA glucocorticoid given, as the free alcohol or in esterified form, orally, intramuscularly, by local injection, by inhalation, or applied topically in the management of various disorders in which corticosteroids are indicated.Glucocorticoids
Oral retinoidsRetinoidsRetinol and derivatives of retinol that play an essential role in metabolic functioning of the retina, the growth of and differentiation of epithelial tissue, the growth of bone, reproduction, and the immune response. Dietary vitamin A is derived from a variety of carotenoids found in plants. It is enriched in the liver, egg yolks, and the fat component of dairy products.Fat-soluble Vitamins and their Deficiencies:
UVB is preferred over UVA (potential increased risk of skinSkinThe skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue.Skin: Structure and Functions cancer with long-term use)
Other potential treatments:[5,8,10]
Vitamin derivatives:
Topical calcipotriol (vitamin DVitamin DA vitamin that includes both cholecalciferols and ergocalciferols, which have the common effect of preventing or curing rickets in animals. It can also be viewed as a hormone since it can be formed in skin by action of ultraviolet rays upon the precursors, 7-dehydrocholesterol and ergosterol, and acts on vitamin D receptors to regulate calcium in opposition to parathyroid hormone.Fat-soluble Vitamins and their Deficiencies)
Alitretinoin (vitamin AVitamin ARetinol and derivatives of retinol that play an essential role in metabolic functioning of the retina, the growth of and differentiation of epithelial tissue, the growth of bone, reproduction, and the immune response. Dietary vitamin A is derived from a variety of carotenoids found in plants. It is enriched in the liver, egg yolks, and the fat component of dairy products.Fat-soluble Vitamins and their Deficiencies)
Antibiotics:
MetronidazoleMetronidazoleA nitroimidazole used to treat amebiasis; vaginitis; trichomonas infections; giardiasis; anaerobic bacteria; and treponemal infections.Pyogenic Liver Abscess
TrimethoprimTrimethoprimThe sulfonamides are a class of antimicrobial drugs inhibiting folic acid synthesize in pathogens. The prototypical drug in the class is sulfamethoxazole. Although not technically sulfonamides, trimethoprim, dapsone, and pyrimethamine are also important antimicrobial agents inhibiting folic acid synthesis. The agents are often combined with sulfonamides, resulting in a synergistic effect. Sulfonamides and Trimethoprim–sulfamethoxazoleSulfamethoxazoleA bacteriostatic antibacterial agent that interferes with folic acid synthesis in susceptible bacteria. Its broad spectrum of activity has been limited by the development of resistance.Sulfonamides and Trimethoprim
Antimalarial: hydroxychloroquineHydroxychloroquineA chemotherapeutic agent that acts against erythrocytic forms of malarial parasites. Hydroxychloroquine appears to concentrate in food vacuoles of affected protozoa. It inhibits plasmodial heme polymerase.Immunosuppressants
Antifungals:
ItraconazoleItraconazoleA triazole antifungal agent that inhibits cytochrome p-450-dependent enzymes required for ergosterol synthesis.Azoles
TerbinafineTerbinafineIn addition to the 3 other major classes of antifungal agents (azoles, polyenes, and echinocandins), several other clinically important antifungal agents are used, including flucytosine, griseofulvin, and terbinafine. Terbinafine acts within the stratum corneum of the skin and are used to treat dermatophyte infections of the skin, hair, and nails.Flucytosine, Griseofulvin, and Terbinafine
GriseofulvinGriseofulvinIn addition to the 3 other major classes of antifungal agents (azoles, polyenes, and echinocandins), several other clinically important antifungal agents are used, including flucytosine, griseofulvin, and terbinafine. Griseofulvin acts within the stratum corneum of the skin and are used to treat dermatophyte infections of the skin, hair, and nails.Flucytosine, Griseofulvin, and Terbinafine
Immunomodulators/immunosuppressantsImmunosuppressantsImmunosuppressants are a class of drugs widely used in the management of autoimmune conditions and organ transplant rejection. The general effect is dampening of the immune response.Immunosuppressants:
AzathioprineAzathioprineAn immunosuppressive agent used in combination with cyclophosphamide and hydroxychloroquine in the treatment of rheumatoid arthritis. According to the fourth annual report on carcinogens, this substance has been listed as a known carcinogen.Immunosuppressants
MethotrexateMethotrexateAn antineoplastic antimetabolite with immunosuppressant properties. It is an inhibitor of tetrahydrofolate dehydrogenase and prevents the formation of tetrahydrofolate, necessary for synthesis of thymidylate, an essential component of DNA.Antimetabolite Chemotherapy
CyclophosphamideCyclophosphamidePrecursor of an alkylating nitrogen mustard antineoplastic and immunosuppressive agent that must be activated in the liver to form the active aldophosphamide. It has been used in the treatment of lymphoma and leukemia. Its side effect, alopecia, has been used for defleecing sheep. Cyclophosphamide may also cause sterility, birth defects, mutations, and cancer.Immunosuppressants
ThalidomideThalidomideA piperidinyl isoindole originally introduced as a non-barbiturate hypnotic, but withdrawn from the market due to teratogenic effects. It has been reintroduced and used for a number of immunological and inflammatory disorders. Thalidomide displays immunosuppressive and anti-angiogenic activity. It inhibits release of tumor necrosis factor-alpha from monocytes, and modulates other cytokine action.Immunosuppressants
AdalimumabAdalimumabA humanized monoclonal antibody that binds specifically to tnf-alpha and blocks its interaction with endogenous tnf receptors to modulate inflammation. It is used in the treatment of rheumatoid arthritis; psoriatic arthritis; Crohn’s disease and ulcerative colitis.Disease-Modifying Antirheumatic Drugs (DMARDs)
UstekinumabUstekinumabA humanized monoclonal antibody that binds to il-12 and il-23 and is used as a dermatologic agent in the treatment of patients with plaque psoriasis who have not responded to other therapies.Immunosuppressants
Intralesional triamcinoloneTriamcinoloneA glucocorticoid given, as the free alcohol or in esterified form, orally, intramuscularly, by local injection, by inhalation, or applied topically in the management of various disorders in which corticosteroids are indicated.Glucocorticoids (10 mg/mL)
PrednisonePrednisoneA synthetic anti-inflammatory glucocorticoid derived from cortisone. It is biologically inert and converted to prednisolone in the liver.Immunosuppressants 30–80 mg daily
RetinoidsRetinoidsRetinol and derivatives of retinol that play an essential role in metabolic functioning of the retina, the growth of and differentiation of epithelial tissue, the growth of bone, reproduction, and the immune response. Dietary vitamin A is derived from a variety of carotenoids found in plants. It is enriched in the liver, egg yolks, and the fat component of dairy products.Fat-soluble Vitamins and their Deficiencies:
Acitretin 25–50 mg/day
Followed by isotretinoin 0.5–1 mg/kg/day
CyclosporineCyclosporineA cyclic undecapeptide from an extract of soil fungi. It is a powerful immunosupressant with a specific action on T-lymphocytes. It is used for the prophylaxis of graft rejection in organ and tissue transplantation.Immunosuppressants 3–10 mg/kg/day
Topical calcineurin inhibitorsCalcineurin InhibitorsCompounds that inhibit or block the phosphatase activity of calcineurin.Immunosuppressants (twice daily for 4–6 weeks):
TacrolimusTacrolimusA macrolide isolated from the culture broth of a strain of streptomyces tsukubaensis that has strong immunosuppressive activity in vivo and prevents the activation of T-lymphocytes in response to antigenic or mitogenic stimulation in vitro.Immunosuppressants
SulfasalazineSulfasalazineA drug that is used in the management of inflammatory bowel diseases. Its activity is generally considered to lie in its metabolic breakdown product, 5-aminosalicylic acid released in the colon.Sulfonamides and Trimethoprim 2.5g/day for 6 weeks
HydroxychloroquineHydroxychloroquineA chemotherapeutic agent that acts against erythrocytic forms of malarial parasites. Hydroxychloroquine appears to concentrate in food vacuoles of affected protozoa. It inhibits plasmodial heme polymerase.Immunosuppressants 200‒400 mg/day for 2 months
Other immunosuppressantsImmunosuppressantsImmunosuppressants are a class of drugs widely used in the management of autoimmune conditions and organ transplant rejection. The general effect is dampening of the immune response.Immunosuppressants:
AzathioprineAzathioprineAn immunosuppressive agent used in combination with cyclophosphamide and hydroxychloroquine in the treatment of rheumatoid arthritis. According to the fourth annual report on carcinogens, this substance has been listed as a known carcinogen.Immunosuppressants
MethotrexateMethotrexateAn antineoplastic antimetabolite with immunosuppressant properties. It is an inhibitor of tetrahydrofolate dehydrogenase and prevents the formation of tetrahydrofolate, necessary for synthesis of thymidylate, an essential component of DNA.Antimetabolite Chemotherapy
Oral prednisonePrednisoneA synthetic anti-inflammatory glucocorticoid derived from cortisone. It is biologically inert and converted to prednisolone in the liver.Immunosuppressants 0.5–0.1 mg/kg for 4–6 weeks
Intralesional triamcinoloneTriamcinoloneA glucocorticoid given, as the free alcohol or in esterified form, orally, intramuscularly, by local injection, by inhalation, or applied topically in the management of various disorders in which corticosteroids are indicated.Glucocorticoids (10–20 mg/mL)
RetinoidsRetinoidsRetinol and derivatives of retinol that play an essential role in metabolic functioning of the retina, the growth of and differentiation of epithelial tissue, the growth of bone, reproduction, and the immune response. Dietary vitamin A is derived from a variety of carotenoids found in plants. It is enriched in the liver, egg yolks, and the fat component of dairy products.Fat-soluble Vitamins and their Deficiencies:
Topical: tretinoin or isotretinoin
Oral: acitretin
HydroxychloroquineHydroxychloroquineA chemotherapeutic agent that acts against erythrocytic forms of malarial parasites. Hydroxychloroquine appears to concentrate in food vacuoles of affected protozoa. It inhibits plasmodial heme polymerase.Immunosuppressants
Other systemic immunosuppressantsImmunosuppressantsImmunosuppressants are a class of drugs widely used in the management of autoimmune conditions and organ transplant rejection. The general effect is dampening of the immune response.Immunosuppressants:
AzathioprineAzathioprineAn immunosuppressive agent used in combination with cyclophosphamide and hydroxychloroquine in the treatment of rheumatoid arthritis. According to the fourth annual report on carcinogens, this substance has been listed as a known carcinogen.Immunosuppressants
MethotrexateMethotrexateAn antineoplastic antimetabolite with immunosuppressant properties. It is an inhibitor of tetrahydrofolate dehydrogenase and prevents the formation of tetrahydrofolate, necessary for synthesis of thymidylate, an essential component of DNA.Antimetabolite Chemotherapy
BetamethasoneBetamethasoneA glucocorticoid given orally, parenterally, by local injection, by inhalation, or applied topically in the management of various disorders in which corticosteroids are indicated. Its lack of mineralocorticoid properties makes betamethasone particularly suitable for treating cerebral edema and congenital adrenal hyperplasia.Glucocorticoids valerate
ClobetasolClobetasolA derivative of prednisolone with high glucocorticoid activity and low mineralocorticoid activity. Absorbed through the skin faster than fluocinonide, it is used topically in treatment of psoriasis but may cause marked adrenocortical suppression.Glucocorticoids propionate 0.05%
TriamcinoloneTriamcinoloneA glucocorticoid given, as the free alcohol or in esterified form, orally, intramuscularly, by local injection, by inhalation, or applied topically in the management of various disorders in which corticosteroids are indicated.Glucocorticoids
FluticasoneFluticasoneA steroid with glucocorticoid receptor activity that is used to manage the symptoms of asthma; allergic rhinitis, and atopic dermatitis.Glucocorticoids
Can be used alone or in combination with topical therapy
Used for severe, widespread, or erosive lesions
Options:
PrednisonePrednisoneA synthetic anti-inflammatory glucocorticoid derived from cortisone. It is biologically inert and converted to prednisolone in the liver.Immunosuppressants
2nd-line treatment (for severe or refractory LP):[5,8,10]
RetinoidsRetinoidsRetinol and derivatives of retinol that play an essential role in metabolic functioning of the retina, the growth of and differentiation of epithelial tissue, the growth of bone, reproduction, and the immune response. Dietary vitamin A is derived from a variety of carotenoids found in plants. It is enriched in the liver, egg yolks, and the fat component of dairy products.Fat-soluble Vitamins and their Deficiencies:
Acitretin
Isotretinoin
HydroxychloroquineHydroxychloroquineA chemotherapeutic agent that acts against erythrocytic forms of malarial parasites. Hydroxychloroquine appears to concentrate in food vacuoles of affected protozoa. It inhibits plasmodial heme polymerase.Immunosuppressants
Not recommended by the US FDA, but European guidelines do support its use
Options:
TacrolimusTacrolimusA macrolide isolated from the culture broth of a strain of streptomyces tsukubaensis that has strong immunosuppressive activity in vivo and prevents the activation of T-lymphocytes in response to antigenic or mitogenic stimulation in vitro.Immunosuppressants
SulfasalazineSulfasalazineA drug that is used in the management of inflammatory bowel diseases. Its activity is generally considered to lie in its metabolic breakdown product, 5-aminosalicylic acid released in the colon.Sulfonamides and Trimethoprim
AzathioprineAzathioprineAn immunosuppressive agent used in combination with cyclophosphamide and hydroxychloroquine in the treatment of rheumatoid arthritis. According to the fourth annual report on carcinogens, this substance has been listed as a known carcinogen.Immunosuppressants
CyclosporineCyclosporineA cyclic undecapeptide from an extract of soil fungi. It is a powerful immunosupressant with a specific action on T-lymphocytes. It is used for the prophylaxis of graft rejection in organ and tissue transplantation.Immunosuppressants
MethotrexateMethotrexateAn antineoplastic antimetabolite with immunosuppressant properties. It is an inhibitor of tetrahydrofolate dehydrogenase and prevents the formation of tetrahydrofolate, necessary for synthesis of thymidylate, an essential component of DNA.Antimetabolite Chemotherapy
TNF-a inhibitors (alefacept, adalimumabAdalimumabA humanized monoclonal antibody that binds specifically to tnf-alpha and blocks its interaction with endogenous tnf receptors to modulate inflammation. It is used in the treatment of rheumatoid arthritis; psoriatic arthritis; Crohn’s disease and ulcerative colitis.Disease-Modifying Antirheumatic Drugs (DMARDs), etanerceptEtanerceptA recombinant version of soluble human tnf receptor fused to an IgG Fc fragment that binds specifically to tumor necrosis factor and inhibits its binding with endogenous tnf receptors. It prevents the inflammatory effect of tnf and is used to treat rheumatoid arthritis; psoriatic arthritis and ankylosing spondylitis.Immunosuppressants)
Table: Comparison of 1st– and 2nd–line oral LP treatment recommendations based on source
Intralesional triamcinoloneTriamcinoloneA glucocorticoid given, as the free alcohol or in esterified form, orally, intramuscularly, by local injection, by inhalation, or applied topically in the management of various disorders in which corticosteroids are indicated.Glucocorticoids (10 mg/mL)
PrednisonePrednisoneA synthetic anti-inflammatory glucocorticoid derived from cortisone. It is biologically inert and converted to prednisolone in the liver.Immunosuppressants 30–80 mg daily
RetinoidsRetinoidsRetinol and derivatives of retinol that play an essential role in metabolic functioning of the retina, the growth of and differentiation of epithelial tissue, the growth of bone, reproduction, and the immune response. Dietary vitamin A is derived from a variety of carotenoids found in plants. It is enriched in the liver, egg yolks, and the fat component of dairy products.Fat-soluble Vitamins and their Deficiencies:
Acitretin 25–50 mg/day
Followed by isotretinoin 0.5–1 mg/kg/day
CyclosporineCyclosporineA cyclic undecapeptide from an extract of soil fungi. It is a powerful immunosupressant with a specific action on T-lymphocytes. It is used for the prophylaxis of graft rejection in organ and tissue transplantation.Immunosuppressants 3–10 mg/kg/day
Topical calcineurin inhibitorsCalcineurin InhibitorsCompounds that inhibit or block the phosphatase activity of calcineurin.Immunosuppressants (twice daily for 4–6 weeks):
TacrolimusTacrolimusA macrolide isolated from the culture broth of a strain of streptomyces tsukubaensis that has strong immunosuppressive activity in vivo and prevents the activation of T-lymphocytes in response to antigenic or mitogenic stimulation in vitro.Immunosuppressants
SulfasalazineSulfasalazineA drug that is used in the management of inflammatory bowel diseases. Its activity is generally considered to lie in its metabolic breakdown product, 5-aminosalicylic acid released in the colon.Sulfonamides and Trimethoprim 2.5g/day for 6 weeks
HydroxychloroquineHydroxychloroquineA chemotherapeutic agent that acts against erythrocytic forms of malarial parasites. Hydroxychloroquine appears to concentrate in food vacuoles of affected protozoa. It inhibits plasmodial heme polymerase.Immunosuppressants 200‒400 mg/day for 2 months
Other immunosuppressantsImmunosuppressantsImmunosuppressants are a class of drugs widely used in the management of autoimmune conditions and organ transplant rejection. The general effect is dampening of the immune response.Immunosuppressants:
AzathioprineAzathioprineAn immunosuppressive agent used in combination with cyclophosphamide and hydroxychloroquine in the treatment of rheumatoid arthritis. According to the fourth annual report on carcinogens, this substance has been listed as a known carcinogen.Immunosuppressants
MethotrexateMethotrexateAn antineoplastic antimetabolite with immunosuppressant properties. It is an inhibitor of tetrahydrofolate dehydrogenase and prevents the formation of tetrahydrofolate, necessary for synthesis of thymidylate, an essential component of DNA.Antimetabolite Chemotherapy
Oral prednisonePrednisoneA synthetic anti-inflammatory glucocorticoid derived from cortisone. It is biologically inert and converted to prednisolone in the liver.Immunosuppressants 0.5–0.1 mg/kg for 4–6 weeks
Intralesional triamcinoloneTriamcinoloneA glucocorticoid given, as the free alcohol or in esterified form, orally, intramuscularly, by local injection, by inhalation, or applied topically in the management of various disorders in which corticosteroids are indicated.Glucocorticoids (10–20 mg/mL)
RetinoidsRetinoidsRetinol and derivatives of retinol that play an essential role in metabolic functioning of the retina, the growth of and differentiation of epithelial tissue, the growth of bone, reproduction, and the immune response. Dietary vitamin A is derived from a variety of carotenoids found in plants. It is enriched in the liver, egg yolks, and the fat component of dairy products.Fat-soluble Vitamins and their Deficiencies:
Topical: tretinoin or isotretinoin
Oral: acitretin
HydroxychloroquineHydroxychloroquineA chemotherapeutic agent that acts against erythrocytic forms of malarial parasites. Hydroxychloroquine appears to concentrate in food vacuoles of affected protozoa. It inhibits plasmodial heme polymerase.Immunosuppressants
Other systemic immunosuppressantsImmunosuppressantsImmunosuppressants are a class of drugs widely used in the management of autoimmune conditions and organ transplant rejection. The general effect is dampening of the immune response.Immunosuppressants:
AzathioprineAzathioprineAn immunosuppressive agent used in combination with cyclophosphamide and hydroxychloroquine in the treatment of rheumatoid arthritis. According to the fourth annual report on carcinogens, this substance has been listed as a known carcinogen.Immunosuppressants
MethotrexateMethotrexateAn antineoplastic antimetabolite with immunosuppressant properties. It is an inhibitor of tetrahydrofolate dehydrogenase and prevents the formation of tetrahydrofolate, necessary for synthesis of thymidylate, an essential component of DNA.Antimetabolite Chemotherapy
Disease course can vary, with papulosquamous genital LP being self-limited. However, vulvovaginal LP is typically difficult to control. Additionally, erosive lesions can lead to scarringScarringInflammation.
Symptomatic treatment:[8]
Topical anestheticsAnestheticsAgents that are capable of inducing a total or partial loss of sensation, especially tactile sensation and pain. They may act to induce general anesthesia, in which an unconscious state is achieved, or may act locally to induce numbness or lack of sensation at a targeted site.Anesthesiology: History and Basic Concepts
Sedating antihistaminesAntihistaminesAntihistamines are drugs that target histamine receptors, particularly H1 and H2 receptors. H1 antagonists are competitive and reversible inhibitors of H1 receptors. First-generation antihistamines cross the blood-brain barrier and can cause sedation. Antihistamines
Low-dose tricyclic antidepressantsTricyclic antidepressantsTricyclic antidepressants (TCAs) are a class of medications used in the management of mood disorders, primarily depression. These agents, named after their 3-ring chemical structure, act via reuptake inhibition of neurotransmitters (particularly norepinephrine and serotonin) in the brain.Tricyclic Antidepressants
ClobetasolClobetasolA derivative of prednisolone with high glucocorticoid activity and low mineralocorticoid activity. Absorbed through the skin faster than fluocinonide, it is used topically in treatment of psoriasis but may cause marked adrenocortical suppression.Glucocorticoids propionate 0.05%
BetamethasoneBetamethasoneA glucocorticoid given orally, parenterally, by local injection, by inhalation, or applied topically in the management of various disorders in which corticosteroids are indicated. Its lack of mineralocorticoid properties makes betamethasone particularly suitable for treating cerebral edema and congenital adrenal hyperplasia.Glucocorticoids dipropionate 0.05%
HydrocortisoneHydrocortisoneThe main glucocorticoid secreted by the adrenal cortex. Its synthetic counterpart is used, either as an injection or topically, in the treatment of inflammation, allergy, collagen diseases, asthma, adrenocortical deficiency, shock, and some neoplastic conditions.Immunosuppressants 25 mg rectal suppository, inserted in the vaginaVaginaThe vagina is the female genital canal, extending from the vulva externally to the cervix uteri internally. The structures have sexual, reproductive, and urinary functions and a rich blood supply, mainly arising from the internal iliac artery.Vagina, Vulva, and Pelvic Floor: Anatomy, 1–2 times daily
May be considered in LP recalcitrant to topical steroidsSteroidsA group of polycyclic compounds closely related biochemically to terpenes. They include cholesterol, numerous hormones, precursors of certain vitamins, bile acids, alcohols (sterols), and certain natural drugs and poisons. Steroids have a common nucleus, a fused, reduced 17-carbon atom ring system, cyclopentanoperhydrophenanthrene. Most steroids also have two methyl groups and an aliphatic side-chain attached to the nucleus.Benign Liver Tumors
Options:
TacrolimusTacrolimusA macrolide isolated from the culture broth of a strain of streptomyces tsukubaensis that has strong immunosuppressive activity in vivo and prevents the activation of T-lymphocytes in response to antigenic or mitogenic stimulation in vitro.Immunosuppressants 0.1% ointment
High-dose preferred for severe, recalcitrant disease.
Taper gradually
Other immunomodulators (mycophenolateMycophenolateImmunosuppressants mofetil, hydroxychloroquineHydroxychloroquineA chemotherapeutic agent that acts against erythrocytic forms of malarial parasites. Hydroxychloroquine appears to concentrate in food vacuoles of affected protozoa. It inhibits plasmodial heme polymerase.Immunosuppressants, and methotrexateMethotrexateAn antineoplastic antimetabolite with immunosuppressant properties. It is an inhibitor of tetrahydrofolate dehydrogenase and prevents the formation of tetrahydrofolate, necessary for synthesis of thymidylate, an essential component of DNA.Antimetabolite Chemotherapy have some use in severe flares)
Acitretin may be helpful in hypertrophic disease.
Surgery:
May be necessary to restore sexual function (vulval and vaginal adhesions)
Contraindicated in active disease
Table: Comparison of 1st– and 2nd–line genital LP treatment recommendations based on source
HydrocortisoneHydrocortisoneThe main glucocorticoid secreted by the adrenal cortex. Its synthetic counterpart is used, either as an injection or topically, in the treatment of inflammation, allergy, collagen diseases, asthma, adrenocortical deficiency, shock, and some neoplastic conditions.ImmunosuppressantssuppositoriesSuppositoriesMedicated dosage forms that are designed to be inserted into the rectal, vaginal, or urethral orifice of the body for absorption. Generally, the active ingredients are packaged in dosage forms containing fatty bases such as cocoa butter, hydrogenated oil, or glycerogelatin that are solid at room temperature but melt or dissolve at body temperature.Large Bowel Obstruction
ClobetasolClobetasolA derivative of prednisolone with high glucocorticoid activity and low mineralocorticoid activity. Absorbed through the skin faster than fluocinonide, it is used topically in treatment of psoriasis but may cause marked adrenocortical suppression.Glucocorticoids
TacrolimusTacrolimusA macrolide isolated from the culture broth of a strain of streptomyces tsukubaensis that has strong immunosuppressive activity in vivo and prevents the activation of T-lymphocytes in response to antigenic or mitogenic stimulation in vitro.Immunosuppressants
TacrolimusTacrolimusA macrolide isolated from the culture broth of a strain of streptomyces tsukubaensis that has strong immunosuppressive activity in vivo and prevents the activation of T-lymphocytes in response to antigenic or mitogenic stimulation in vitro.Immunosuppressants 0.1% ointment
Intralesional corticosteroidsIntralesional CorticosteroidsHypertrophic and Keloid Scars: triamcinoloneTriamcinoloneA glucocorticoid given, as the free alcohol or in esterified form, orally, intramuscularly, by local injection, by inhalation, or applied topically in the management of various disorders in which corticosteroids are indicated.Glucocorticoids
Preferred by some groups[16]
Painful
Topical steroidsSteroidsA group of polycyclic compounds closely related biochemically to terpenes. They include cholesterol, numerous hormones, precursors of certain vitamins, bile acids, alcohols (sterols), and certain natural drugs and poisons. Steroids have a common nucleus, a fused, reduced 17-carbon atom ring system, cyclopentanoperhydrophenanthrene. Most steroids also have two methyl groups and an aliphatic side-chain attached to the nucleus.Benign Liver Tumors (super potent and potent):
May also be used as an adjunct to 1st-line therapy
CyclosporineCyclosporineA cyclic undecapeptide from an extract of soil fungi. It is a powerful immunosupressant with a specific action on T-lymphocytes. It is used for the prophylaxis of graft rejection in organ and tissue transplantation.Immunosuppressants
AzathioprineAzathioprineAn immunosuppressive agent used in combination with cyclophosphamide and hydroxychloroquine in the treatment of rheumatoid arthritis. According to the fourth annual report on carcinogens, this substance has been listed as a known carcinogen.Immunosuppressants
Table: Comparison of 1st– and 2nd–line nail LP treatment recommendations based on source
Literature source
1st–line
2nd–line
European[8]
IM triamcinoloneTriamcinoloneA glucocorticoid given, as the free alcohol or in esterified form, orally, intramuscularly, by local injection, by inhalation, or applied topically in the management of various disorders in which corticosteroids are indicated.Glucocorticoids 0.5 mg/kg every 30 days, followed by taper
Intralesional triamcinoloneTriamcinoloneA glucocorticoid given, as the free alcohol or in esterified form, orally, intramuscularly, by local injection, by inhalation, or applied topically in the management of various disorders in which corticosteroids are indicated.Glucocorticoids 0.5–0.1 mg/nail every 2 months
Oral prednisonePrednisoneA synthetic anti-inflammatory glucocorticoid derived from cortisone. It is biologically inert and converted to prednisolone in the liver.Immunosuppressants 0.5 mg/kg for 3 weeks
TacrolimusTacrolimusA macrolide isolated from the culture broth of a strain of streptomyces tsukubaensis that has strong immunosuppressive activity in vivo and prevents the activation of T-lymphocytes in response to antigenic or mitogenic stimulation in vitro.Immunosuppressants 0.1% twice daily for 6 months
Others:
ChloroquineChloroquineThe prototypical antimalarial agent with a mechanism that is not well understood. It has also been used to treat rheumatoid arthritis, systemic lupus erythematosus, and in the systemic therapy of amebic liver abscesses.Antimalarial Drugs
Cyclosporin
5% fluorouracilFluorouracilA pyrimidine analog that is an antineoplastic antimetabolite. It interferes with DNA synthesis by blocking the thymidylate synthetase conversion of deoxyuridylic acid to thymidylic acid.Bowen Disease and Erythroplasia of Queyrat
Biotin
EtanerceptEtanerceptA recombinant version of soluble human tnf receptor fused to an IgG Fc fragment that binds specifically to tumor necrosis factor and inhibits its binding with endogenous tnf receptors. It prevents the inflammatory effect of tnf and is used to treat rheumatoid arthritis; psoriatic arthritis and ankylosing spondylitis.Immunosuppressants
US[5,16]
Intralesional triamcinoloneTriamcinoloneA glucocorticoid given, as the free alcohol or in esterified form, orally, intramuscularly, by local injection, by inhalation, or applied topically in the management of various disorders in which corticosteroids are indicated.Glucocorticoids:
M triamcinoloneTriamcinoloneA glucocorticoid given, as the free alcohol or in esterified form, orally, intramuscularly, by local injection, by inhalation, or applied topically in the management of various disorders in which corticosteroids are indicated.Glucocorticoids 0.5–1 mg/kg monthly for 3–6 months
Oral is an alternative
Oral retinoidsRetinoidsRetinol and derivatives of retinol that play an essential role in metabolic functioning of the retina, the growth of and differentiation of epithelial tissue, the growth of bone, reproduction, and the immune response. Dietary vitamin A is derived from a variety of carotenoids found in plants. It is enriched in the liver, egg yolks, and the fat component of dairy products.Fat-soluble Vitamins and their Deficiencies:
Intralesional corticosteroidsIntralesional CorticosteroidsHypertrophic and Keloid Scars: triamcinoloneTriamcinoloneA glucocorticoid given, as the free alcohol or in esterified form, orally, intramuscularly, by local injection, by inhalation, or applied topically in the management of various disorders in which corticosteroids are indicated.Glucocorticoids
Oral retinoidsRetinoidsRetinol and derivatives of retinol that play an essential role in metabolic functioning of the retina, the growth of and differentiation of epithelial tissue, the growth of bone, reproduction, and the immune response. Dietary vitamin A is derived from a variety of carotenoids found in plants. It is enriched in the liver, egg yolks, and the fat component of dairy products.Fat-soluble Vitamins and their Deficiencies: may be particularly helpful in LP with perifollicular hyperkeratosisHyperkeratosisIchthyosis Vulgaris
Other potential treatments:[8,10]
TetracyclinesTetracyclinesTetracyclines are a class of broad-spectrum antibiotics indicated for a wide variety of bacterial infections. These medications bind the 30S ribosomal subunit to inhibit protein synthesis of bacteria. Tetracyclines cover gram-positive and gram-negative organisms, as well as atypical bacteria such as chlamydia, mycoplasma, spirochetes, and even protozoa. Tetracyclines
HydroxychloroquineHydroxychloroquineA chemotherapeutic agent that acts against erythrocytic forms of malarial parasites. Hydroxychloroquine appears to concentrate in food vacuoles of affected protozoa. It inhibits plasmodial heme polymerase.Immunosuppressants
CyclosporineCyclosporineA cyclic undecapeptide from an extract of soil fungi. It is a powerful immunosupressant with a specific action on T-lymphocytes. It is used for the prophylaxis of graft rejection in organ and tissue transplantation.Immunosuppressants
AzathioprineAzathioprineAn immunosuppressive agent used in combination with cyclophosphamide and hydroxychloroquine in the treatment of rheumatoid arthritis. According to the fourth annual report on carcinogens, this substance has been listed as a known carcinogen.Immunosuppressants
Table: Comparison of 1st– and 2nd–line lichen planopilarisLichen PlanopilarisLichen Planus treatment recommendations based on source
Intralesional triamcinoloneTriamcinoloneA glucocorticoid given, as the free alcohol or in esterified form, orally, intramuscularly, by local injection, by inhalation, or applied topically in the management of various disorders in which corticosteroids are indicated.Glucocorticoids (5–20 mg/mL) every 2–4 weeks
PrednisonePrednisoneA synthetic anti-inflammatory glucocorticoid derived from cortisone. It is biologically inert and converted to prednisolone in the liver.Immunosuppressants 30–80 mg daily
TCyclosporine 3–10 mg/kg/day
HydroxychloroquineHydroxychloroquineA chemotherapeutic agent that acts against erythrocytic forms of malarial parasites. Hydroxychloroquine appears to concentrate in food vacuoles of affected protozoa. It inhibits plasmodial heme polymerase.Immunosuppressants 200–400 mg for 6–12 months
MethotrexateMethotrexateAn antineoplastic antimetabolite with immunosuppressant properties. It is an inhibitor of tetrahydrofolate dehydrogenase and prevents the formation of tetrahydrofolate, necessary for synthesis of thymidylate, an essential component of DNA.Antimetabolite Chemotherapy 15 mg/wk for 6 months
Topical calcineurin inhibitorsCalcineurin InhibitorsCompounds that inhibit or block the phosphatase activity of calcineurin.Immunosuppressants (tacrolimusTacrolimusA macrolide isolated from the culture broth of a strain of streptomyces tsukubaensis that has strong immunosuppressive activity in vivo and prevents the activation of T-lymphocytes in response to antigenic or mitogenic stimulation in vitro.Immunosuppressants) twice daily for ≥ 1 month
RetinoidsRetinoidsRetinol and derivatives of retinol that play an essential role in metabolic functioning of the retina, the growth of and differentiation of epithelial tissue, the growth of bone, reproduction, and the immune response. Dietary vitamin A is derived from a variety of carotenoids found in plants. It is enriched in the liver, egg yolks, and the fat component of dairy products.Fat-soluble Vitamins and their Deficiencies (for 3–6 months):
Acitretin 25–30 mg/day
Isotretinoin 0.5–1 mg/kg/day
TetracyclineTetracyclineA naphthacene antibiotic that inhibits amino Acyl tRNA binding during protein synthesis.Drug-Induced Liver Injury or doxycycline 100 mg/day for 1 month
ImmunosuppressantsImmunosuppressantsImmunosuppressants are a class of drugs widely used in the management of autoimmune conditions and organ transplant rejection. The general effect is dampening of the immune response.Immunosuppressants:
AdalimumabAdalimumabA humanized monoclonal antibody that binds specifically to tnf-alpha and blocks its interaction with endogenous tnf receptors to modulate inflammation. It is used in the treatment of rheumatoid arthritis; psoriatic arthritis; Crohn’s disease and ulcerative colitis.Disease-Modifying Antirheumatic Drugs (DMARDs)
RituximabRituximabA murine-derived monoclonal antibody and antineoplastic agent that binds specifically to the cd20 antigen and is used in the treatment of leukemia; lymphoma and rheumatoid arthritis.Immunosuppressants
ThalidomideThalidomideA piperidinyl isoindole originally introduced as a non-barbiturate hypnotic, but withdrawn from the market due to teratogenic effects. It has been reintroduced and used for a number of immunological and inflammatory disorders. Thalidomide displays immunosuppressive and anti-angiogenic activity. It inhibits release of tumor necrosis factor-alpha from monocytes, and modulates other cytokine action.Immunosuppressants
Laser therapyLaser TherapyThe use of photothermal effects of lasers to coagulate, incise, vaporize, resect, dissect, or resurface tissue.Glaucoma
Oral retinoidsRetinoidsRetinol and derivatives of retinol that play an essential role in metabolic functioning of the retina, the growth of and differentiation of epithelial tissue, the growth of bone, reproduction, and the immune response. Dietary vitamin A is derived from a variety of carotenoids found in plants. It is enriched in the liver, egg yolks, and the fat component of dairy products.Fat-soluble Vitamins and their Deficiencies
Differential Diagnosis
Irritant contact dermatitisContact dermatitisA type of acute or chronic skin reaction in which sensitivity is manifested by reactivity to materials or substances coming in contact with the skin. It may involve allergic or non-allergic mechanisms.Male Genitourinary Examination: skinSkinThe skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue.Skin: Structure and Functions condition that results from exposure to a physical or chemical irritant. PatientsPatientsIndividuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures.Clinician–Patient Relationship present with erythemaErythemaRedness of the skin produced by congestion of the capillaries. This condition may result from a variety of disease processes.Chalazion, edemaEdemaEdema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema, vesiclesVesiclesFemale Genitourinary Examination, and oozing that are limited to areas where the offending substance was present.
Discoid lupus erythematosusDiscoid Lupus ErythematosusAlopecia: a chronic autoimmune skinSkinThe skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue.Skin: Structure and Functions condition characterized by erythematous scaly plaques that often result in inflammationInflammationInflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body’s defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation, scarringScarringInflammation, and atrophyAtrophyDecrease in the size of a cell, tissue, organ, or multiple organs, associated with a variety of pathological conditions such as abnormal cellular changes, ischemia, malnutrition, or hormonal changes.Cellular Adaptation on the face, neckNeckThe part of a human or animal body connecting the head to the rest of the body.Peritonsillar Abscess, ears, and scalp. The condition may occur with or without concurrent systemic lupus erythematosusSystemic lupus erythematosusSystemic lupus erythematosus (SLE) is a chronic autoimmune, inflammatory condition that causes immune-complex deposition in organs, resulting in systemic manifestations. Women, particularly those of African American descent, are more commonly affected.Systemic Lupus Erythematosus.
Lichenoid drug eruption: the symmetric cutaneous eruption that appears as erythematous papules on the trunk and extremities after taking a drug. Wickham’s striae is usually not present. In most cases, the condition will spontaneously resolve with discontinuation of the offending drug.
EczemaEczemaAtopic dermatitis, also known as eczema, is a chronic, relapsing, pruritic, inflammatory skin disease that occurs more frequently in children, although adults can also be affected. The condition is often associated with elevated serum levels of IgE and a personal or family history of atopy. Skin dryness, erythema, oozing, crusting, and lichenification are present. Atopic Dermatitis (Eczema) (seen in atopic dermatitisDermatitisAny inflammation of the skin.Atopic Dermatitis (Eczema)): a chronic pruritic skinSkinThe skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue.Skin: Structure and Functions condition commonly found in children. EczemaEczemaAtopic dermatitis, also known as eczema, is a chronic, relapsing, pruritic, inflammatory skin disease that occurs more frequently in children, although adults can also be affected. The condition is often associated with elevated serum levels of IgE and a personal or family history of atopy. Skin dryness, erythema, oozing, crusting, and lichenification are present. Atopic Dermatitis (Eczema) is associated with asthmaAsthmaAsthma is a chronic inflammatory respiratory condition characterized by bronchial hyperresponsiveness and airflow obstruction. The disease is believed to result from the complex interaction of host and environmental factors that increase disease predisposition, with inflammation causing symptoms and structural changes. Patients typically present with wheezing, cough, and dyspnea. Asthma. Lesions are dry, pruritic, red papules mainly located on the cheeksCheeksThe part of the face that is below the eye and to the side of the nose and mouth.Melasma or scalp (in infants) and flexural areas of extremities (older children).
Seborrheic dermatitisDermatitisAny inflammation of the skin.Atopic Dermatitis (Eczema): a chronic inflammatory dermatosis characterized by the presence of greasy, red (inflamed) skinSkinThe skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue.Skin: Structure and Functions lesions affecting the scalp (as dandruffDandruffExcessive shedding of dry scaly material from the scalp in humans.Seborrheic Dermatitis), posterior aspect of the neckNeckThe part of a human or animal body connecting the head to the rest of the body.Peritonsillar Abscess, and the foreheadForeheadThe part of the face above the eyes.Melasma. The condition is linked to increased sebumSebumThe oily substance secreted by sebaceous glands. It is composed of keratin, fat, and cellular debris.Infectious Folliculitis production and possible colonizationColonizationBacteriology of the skinSkinThe skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue.Skin: Structure and Functions by fungiFungiA kingdom of eukaryotic, heterotrophic organisms that live parasitically as saprobes, including mushrooms; yeasts; smuts, molds, etc. They reproduce either sexually or asexually, and have life cycles that range from simple to complex. Filamentous fungi, commonly known as molds, refer to those that grow as multicellular colonies.Mycology of the genus MalasseziaMalasseziaMalassezia is a lipophilic yeast commonly found on the skin surfaces of many animals, including humans. In the presence of certain environments or triggers, this fungus can cause pathologic diseases ranging from superficial skin conditions (tinea versicolor and dermatitis) to invasive disease (e.g., Malassezia folliculitis, catheter-associated fungemia, meningitis, and urinary tract infections). Malassezia Fungi.
Pityriasis roseaPityriasis roseaPityriasis rosea is an acute, self-limited skin disease. The etiology is not known, and it commonly occurs in young adults. Patients initially present with a single, ovoid “herald patch.” This is followed by diffuse, pruritic, scaly, oval lesions over the trunk (often in a “Christmas tree” distribution on the back) and extremities. Pityriasis Rosea: skinSkinThe skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue.Skin: Structure and Functions condition affecting mostly older children and young adults. Pityriasis roseaPityriasis roseaPityriasis rosea is an acute, self-limited skin disease. The etiology is not known, and it commonly occurs in young adults. Patients initially present with a single, ovoid “herald patch.” This is followed by diffuse, pruritic, scaly, oval lesions over the trunk (often in a “Christmas tree” distribution on the back) and extremities. Pityriasis Rosea begins with a single “herald” patchPatchNonpalpable lesion > 1 cm in diameterGeneralized and Localized Rashes on the chest, neckNeckThe part of a human or animal body connecting the head to the rest of the body.Peritonsillar Abscess, or back. This is followed by smaller pruritic salmon-colored oval plaques with scaling located on the trunk and extremities. The characteristic morphology is a “Christmas tree” pattern on the back.
PlaquePlaquePrimary Skin LesionspsoriasisPsoriasisPsoriasis is a common T-cell-mediated inflammatory skin condition. The etiology is unknown, but is thought to be due to genetic inheritance and environmental triggers. There are 4 major subtypes, with the most common form being chronic plaque psoriasis. Psoriasis: lesions are characterized by well-demarcated, erythematous, symmetrically distributed plaques with overlying silver scalesScalesDry or greasy masses of keratin that represent thickened stratum corneum.Secondary Skin Lesions involving the scalp and extensor surfaces (elbows and knees).
Lichen simplex chronicusLichen Simplex ChronicusA benign vulvar skin disorder characterized by hyperkeratosis (thickening of the skin) that occurs secondary to chronic vulvar irritationBenign Vulvar Conditions: a form of neurodermatitis caused by the itch-scratch cycle, leading to plaques and thickened skinSkinThe skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue.Skin: Structure and Functions. Locations affected are areas accessible to scratching. Management includes potent topical steroidsSteroidsA group of polycyclic compounds closely related biochemically to terpenes. They include cholesterol, numerous hormones, precursors of certain vitamins, bile acids, alcohols (sterols), and certain natural drugs and poisons. Steroids have a common nucleus, a fused, reduced 17-carbon atom ring system, cyclopentanoperhydrophenanthrene. Most steroids also have two methyl groups and an aliphatic side-chain attached to the nucleus.Benign Liver Tumors.
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Diagnosis and management of vulvar skin disorders: ACOG practice bulletin, number 224. (2020). Obstetrics & Gynecology, 136(1), e1–e14. https://doi.org/10.1097/AOG.0000000000003944
van der Meijden, W. I., Boffa, M. J., ter Harmsel, B., et al. (2022). 2021 European guideline for the management of vulval conditions. Journal of the European Academy of Dermatology and Venereology, 36(7), 952–972. https://doi.org/10.1111/jdv.18102
Iorizzo, M., et al. (2020). Isolated nail lichen planus: an expert consensus on treatment of the classical form. Journal of the American Academy of Dermatology, 83(6), 1717–1723. https://www.jaad.org/article/S0190-9622(20)30300-5/pdf
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