Bullous lichen planus= الحزاز المسطح الفقاعي |
Lichen planus
Lichen planus (LP) is a pruritic, papular eruption characterized by its violaceous color; polygonal shape; and, sometimes, fine scale. Lichen planus is most commonly found on the flexor surfaces of the upper extremities, on the genitalia, and on the mucous membranes. Lichen planus is most likely an immunologically mediated reaction. See Oral Lichen Planus for more information on this variant of lichen planus.
PathophysiologyLichen planus is a cell-mediated immune response of unknown origin. Lichen planus may be found with other diseases of altered immunity; these conditions include ulcerative colitis, alopecia areata, vitiligo, dermatomyositis, morphea, lichen sclerosis, and myasthenia gravis.
HistoryMost cases of lichen planus (LP) are insidious.
PhysicalIn addition to the cutaneous eruption, lichen planus (LP) can involve the mucous membranes, the genitalia, the nails, and the scalp. The clinical presentation of lichen planus has several forms: actinic, annular, atrophic, erosive, follicular, hypertrophic, linear, pigmented, and vesicular/bullous. The papules are violaceous, shiny, and polygonal; varying in size from 1 mm to greater than 1 cm in diameter (see the image below). They can be discrete or arranged in groups of lines or circles. Characteristic fine, white lines, called Wickham stria, are often found on the papules (see the image below)
Mucous membrane involvement is common and may be found without skin involvement. Lesions are most commonly found on the tongue and the buccal mucosa; they are characterized by white or gray streaks forming a linear or reticular pattern on a violaceous background (see the image below). Oral lesions are classified as reticular, plaquelike, atrophic, papular, erosive, and bullous. Ulcerated oral lesions may have a higher incidence of malignant transformation in men, but this observation may be confounded by other factors, such as smoking and chewing tobacco. Lesions may also be found on the conjunctivae, the larynx, the esophagus, the tonsils, the bladder, the vulva, and the vaginal vault; throughout the gastrointestinal tract; and around the anus. .
Genital involvement is common in men with cutaneous lichen planus. Typically, an annular configuration of papules is seen on the glans. Less commonly, linear white striae, similar to the lesions on the vulva and the vagina, can be seen on male genitalia. Vulvar involvement can range from reticulate papules to severe erosions. Dyspareunia, a burning sensation, and pruritus are common. Vulvar and urethral stenosis can also be present. Two reports documented that more than 50% women with oral lichen planus had undiagnosed vulvar lichen planus.10,11 Also see the clinical guideline summary, Diagnosis and management of vulvar skin disorders.12 In 10% of lichen planus patients, ungual findings are present. Most commonly, nail plate thinning causes longitudinal grooving and ridging. Hyperpigmentation, subungual hyperkeratosis, onycholysis, and longitudinal melanonychia can result from lichen planus. Rarely, the matrix can be permanently destroyed with prominent pterygium formation. Lichen planus has been linked to childhood idiopathic nail atrophy and may overlap with twenty-nail dystrophy of childhood. Lichen planus patients with a cutaneous eruption may also have follicular and perifollicular violaceous, scaly, pruritic papules on the scalp. These lesions can progress to atrophic cicatricial alopecia that can appear many weeks after the skin lesions have disappeared. Pseudopelade can be a final endpoint. Variations in lichen planus include the following:
CausesThe exact cause of lichen planus (LP) is not known. The pathogenesis of lichen planus is immunologically mediated. Whether the foreign antigen is a virus or a drug is not known. Langerhans cells process antigens, which are then presented to T lymphocytes. This stimulated lymphocytic infiltrate is epidermotropic and attacks keratinocytes. During this lymphocytotoxic process, the keratinocytes release cytokines that attract more lymphocytes. This process has been referred to as the lichenoid tissue reaction. In addition, recent studies reveal a disruption in the epithelial anchoring system.
Laboratory StudiesDirect immunofluorescence study in lichen planus (LP) reveals globular deposits of immunoglobulin M (IgM) and complement mixed with apoptotic keratinocytes. Imaging StudiesNo imaging studies are necessary for lichen planus. Histologic FindingsThe histopathologic features distinguish lichen planus based on the presence of irregular acanthosis and colloid bodies in the epidermis with destruction of the basal layer. The upper dermis has a bandlike infiltrate of lymphocytes and histiocytes.
Medical CareLichen planus (LP) is a self-limited disease that usually resolves within 8-12 months. Mild cases can be treated with fluorinated topical steroids. More severe cases, especially those with scalp, nail, and mucous membrane involvement, may need more intensive therapy. ConsultationsConsult a dermatologist. MedicationThe first-line treatments of cutaneous lichen planus (LP) are topical steroids, particularly class I or II ointments. A second choice would be systemic steroids for symptom control and possibly more rapid resolution. Many practitioners prefer intramuscular triamcinolone 40-80 mg every 6-8 weeks. Oral acitretin has been shown to be effective in published studies.13 Many other treatments, including mycophenolate mofetil (CellCept) at 1-1.5 g twice daily, are of uncertain efficacy because of the lack of randomized controlled trials. In a randomized double-blinded study, sulfasalazine at up to 2.5 g/d for 6 weeks showed improvement in lesions (>80%) and pruritus (>90%) in patients with generalized lichen planus.14 CorticosteroidsThese agents have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli. Topical steroids may be as effective as systemic steroids. Class I or II steroids in ointment form reduce pruritus in cutaneous lichen planus, but they have not been proven to induce remission. Prednisone (Deltasone, Sterapred, Orasone)May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.
Adult30-60 mg/d PO for 4-6 wk followed by gradual taper Pediatric4-5 mg/m2/d PO; alternatively, 0.05-2 mg/kg PO divided bid/qid; taper over 2 wk as symptoms resolve
Coadministration with estrogens may decrease clearance; when used with digoxin, digitalis toxicity secondary to hypokalemia may increase; phenobarbital, phenytoin, and rifampin may increase the metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics; coadministration with ritonavir may significantly increase serum concentrations of prednisone; concomitant therapy with montelukast may result in severe peripheral edema; clarithromycin may increase risk of psychotic symptoms
Documented hypersensitivity; viral, fungal, tubercular skin, or connective tissue infections; peptic ulcer disease; hepatic dysfunction
PregnancyB - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals PrecautionsMay unmask hypertension or diabetes or exacerbate peptic ulcer disease and tuberculosis; long-term sequelae associated with long-term steroid use include osteoporosis, cataracts, and pituitary-hypothalamic axis suppression; with high doses, patients may develop a steroid psychosis and are at increased risk of infections, particularly when oral steroids are used in conjunction with other immunosuppressants; frequently monitor patient's blood glucose level, blood pressure, and weight; monitor for Cushing syndrome; oral candidiasis may develop in patients treated for oral erosive LP Betamethasone (Diprolene, Betatrex)For inflammatory dermatosis responsive to steroids. Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability. Use in pediatrics with extreme caution. Children have a larger skin surface area to body weight ratio and less developed, thinner skin, which may result in greater amounts of topical steroid being absorbed compared with adults. Use nonfluorinated topical corticosteroids.
AdultOral LP: Apply gel to affected area q4-6h for 2-3 mo PediatricApply as in adults
None reported
Documented hypersensitivity; paronychia; cellulitis; impetigo; angular cheilitis; erythrasma; erysipelas; rosacea; perioral dermatitis; acne
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus PrecautionsIf treating large areas, systemic absorption may occur and produce reversible HPA-axis suppression with potential for glucocorticoid insufficiency after withdrawal of treatment; do not use in skin with decreased circulation; can cause atrophy of groin, face, and axillae; if infection develops and is not responsive to antibiotic treatment, discontinue until infection is under control Triamcinolone (Aristocort)For inflammatory dermatosis responsive to steroids. Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability. Local injections have been reported to be effective.
AdultHypertrophic lesions: 5 mg/mL (10 mg/mL formulation) injected into posterior nail fold q2-4wk for 3 treatments may improve ungual lesions; oral lesions can also be treated with intralesional injection of 5-10 mg/mL Pediatric2.5-15 mg (10 mg/mL or 40 mg/mL formulations intralesionally); repeat prn
None reported
Documented hypersensitivity; fungal, viral, and bacterial skin infections; hypertension; ocular herpes simplex (risk of corneal perforation); scleroderma; recent surgery; infection at treatment site
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus PrecautionsMay cause skin atrophy and hypopigmentation; do not use in decreased skin circulation; prolonged use, applying over large areas, and using potent steroids and occlusive dressings may result in systemic absorption; systemic absorption may cause Cushing syndrome, reversible HPA-axis suppression, hyperglycemia, and glycosuria Halobetasol (Ultravate) ointment, creamFor inflammatory dermatosis responsive to steroids. Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability. Use in pediatrics with extreme caution. Children have a larger ratio of skin surface area to body weight and less developed, thinner skin, which may result in greater amounts of topical steroid being absorbed compared with adults. Use nonfluorinated topical corticosteroids.
AdultApply to affected areas bid PediatricNot recommended
None reported
Documented hypersensitivity; paronychia; cellulitis; impetigo; angular cheilitis; erythrasma; erysipelas; rosacea; perioral dermatitis; acne
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus PrecautionsIf treating large areas, systemic absorption may occur and produce reversible HPA-axis suppression with potential for glucocorticoid insufficiency after withdrawal of treatment; do not use in skin with decreased circulation; can cause atrophy of groin, face, and axillae; if infection develops and is not responsive to antibiotic treatment, discontinue until infection is under control RetinoidsThese agents modulate cell proliferation. Isotretinoin (Amnesteem, Roaccutane)Oral agent that treats serious dermatologic conditions. Synthetic 13-cis isomer of the naturally occurring tretinoin (trans- retinoic acid). Both agents are structurally related to vitamin A. Decreases sebaceous gland size and sebum production. May inhibit sebaceous gland differentiation and abnormal keratinization.
Adult40 mg/d PO for several mo PediatricNot established
Toxicity may occur with vitamin A coadministration; pseudotumor cerebri or papilledema may occur when coadministered with tetracyclines; may reduce plasma levels of carbamazepine
Documented hypersensitivity
PregnancyX - Contraindicated; benefit does not outweigh risk PrecautionsMay decrease night vision; inflammatory bowel disease may occur; may be associated with development of hepatitis and pancreatitis; diabetes patients may experience problems in controlling blood glucose while on isotretinoin; avoid exposure to UV light or sunlight until tolerance achieved; discontinue treatment if rectal bleeding, abdominal pain, or severe diarrhea occurs; mood swings or depression may occur; caution in history of depression Tretinoin (Retin-A, Avita, Renova, Atralin)May be effective for oral LP but not for cutaneous disease. Inhibits microcomedo formation and eliminates existing lesions. Makes keratinocytes in sebaceous follicles less adherent and easier to remove. Available as 0.025%, 0.05%, and 0.1% creams. Also available as 0.01% and 0.025% gels.
AdultApply to affected area of mouth bid; begin with lowest tretinoin formulation and increase as tolerated; apply hs or qod; lower frequency of application if irritation develops Pediatric<12 years: Not established
Other skin irritants (ie, astringents, benzoyl peroxide, salicylic acid, resorcinol, topical sulfur, other keratolytics, abrasives, astringents, spices, lime) may exacerbate irritation; coadministration with other drugs causing photosensitivity (eg, tetracycline, sulfonamides) may increase risk of sunburn
Documented hypersensitivity
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus PrecautionsPhotosensitivity may occur with excessive sunlight exposure; burning, stinging, peeling, pruritus, or erythema has been reported at site of application; caution with eczema (may cause severe irritation); avoid contact with mucous membranes, mouth, and angles of nose Acitretin (Soriatane)Retinoic acid analog, like etretinate and isotretinoin. Etretinate is main metabolite and has demonstrated clinical effects close to those seen with etretinate. Mechanism of action is unknown.
Adult30 mg/d PO for 8 wk PediatricNot established
Increases toxicity of methotrexate (avoid concomitant use); interferes with effects of microdosed progestin minipill; coadministration with alcohol may enhance synthesis of etretinate, which has much longer half-life than acitretin (>120 d); topical drying or peeling agents; tetracyclines or photosensitizing medications
Documented hypersensitivity; pregnancy
PregnancyX - Contraindicated; benefit does not outweigh risk PrecautionsMost common adverse effects include cheilitis and dry mouth; hair loss, xerosis, hypertriglyceridemia, and impaired wound healing may occur; for women, a contraceptive period of 3 y after last intake of acitretin is recommended; do not use in severe obesity; women of childbearing age must be capable of complying with effective contraceptive measures; perform AST, ALT, and LDH tests prior to initiation of acitretin therapy at 1- to 2-wk intervals until stable and thereafter at intervals as clinically indicated ImmunosuppressantsThese agents modulate the immune system. Cyclosporine (Sandimmune, Neoral)Topical treatment under occlusion has been efficacious for genital lesions and may be beneficial in hypertrophic lesions. Mouthwash or oil-based solutions have been effective for oral LP but seem to be no better than corticosteroids. Systemic treatment has been used for severe resistant cutaneous disease, oral or ulcerative foot involvement, and lichen planopilaris of the scalp.
Adult1-2 mg/kg/d PO recommended starting dose; if no response, increase dose; usual dose is 5 mg/kg/d PediatricAdminister as in adults |