EPIDEMIOLOGY

6-40: Lichen Planus

ESSENTIALS OF DIAGNOSIS

Pruritic, violaceous, flat-topped papules with fine white streaks and symmetric distribution

Lacy or erosive lesions of buccal, vulvar and vaginal mucosa; nail dystrophy

Commonly seen along linear scratch marks (Koebner phenomenon) on anterior wrists, penis, legs

OVERVIEW
• Relatively common (0.5-2.0% population) chronic inflammatory pruritic rash of skin and mucous
membranes characterized by distinctive papules with a predilection for flexor surfaces and trunk
• occurs in both sexes and at any age, usually between 30 and 60 years
• Three cardinal findings
o Typical skin lesions
o Mucosal lesions
o Histopathologic features of band-like infiltration of lymphocytes in upper dermis

Lichenoid drug eruptions can resemble lichen planus clinically and histologically

Hepatitis C infection is more common in lichen planus patients than in controls

Allergy to mercury and other metal containing amalgams can trigger oral lesions identical to lichen
planus

PATHOGENESIS
an autoimmune basis, associated with IBD, primary biliary cholangitis, autoimmune hepatitis, hepatitis B
and C, alopecia areata, myasthenia gravis and thymoma. Also similar with graft-versus-host disease
(GVHD).

PATHOLOGY
On skin biopsy, characteristic histological changes include hyperkeratosis, basal cell degeneration and a
heavy, band-like T-lymphocyte infiltrate in papillary dermis, with affinity for epidermis
(epidermotropism). Dermo-epidermal junction has a ‘sawtooth’ appearance.
组织学表现为基底细胞液化变性和真皮浅中层淋巴细胞带状浸润。

CLINICAL FINDINGS

Symptoms and Signs

o Mild to severe/intense itching
o Skin lesions: 1. violaceous, shiny, flat-topped, polygonal or angulated papules 红色多角形扁平丘疹、
斑 块 , 1-4 mm in diameter. 2. discrete or in clusters. 3. Contain a characteristic very fine lacy, white
streaks/network (Wickham’s striae) on the surface a. Distal limbs. most commonly on flexor (flexural)
surfaces of wrists, forearm and ankles, b. Lower back. c. Mucous membranes, including penis, lips,
tongue, buccal, vulvar, vaginal, esophageal, and anorectal mucosa. 手腕，前臂，下肢远端，骶骨前区
o Mucous membrane lesions have a lacy white network overlying them that may be confused with
leukoplakia
o Mucosal lichen planus in the oral, genital, and anorectal areas may be erosive or ulcerative
o Papules may become bullous and eroded
o Rash may become generalized

Patients with both oral and vaginal lichen planus are at much higher risk for esophageal lichen planus
o Koebner phenomenon (appearance of new lesions in areas of skin trauma)

o Individual lesions may last for many months and can become hypertrophic and modified by scratching,
particularly on lower legs. The eruption usually remits over months but can become chronic, particularly
with hypertrophic disease.
o Post-inflammatory pigmentary change is common, particularly in darker skin types.

o Mucous membrane involvement occurs in 30-70% patients, usually as a network of white, lacy striae on
buccal mucosae and tongue. often asymptomatic

Genital and other mucosal surfaces can also be affected. Nail involvement occurs in about 10% and can
range from longitudinal ridging to a destructive nail dystrophy, scarring (pterygium) and nail loss. Scalp
involvement usually presents as an inflammatory scarring alopecia, often with tufting of residual hairs.

Classical presentation is unmistakable, but less common atypical variants, which include linear 线 状 ,
annular 环形, 肥厚性, atrophic 萎缩性, actinic 光线性, bullous 大疱形, follicular, pigmented 色素性
and ulcerative types, 毛发扁平苔藓 can be a diagnostic challenge.

Squamous cell carcinoma

o Develops in 5% patients with erosive oral or genital lichen planus
o May occur in esophageal lichen planus
o Risk increased in lesions of hypertrophic lichen planus on the lower extremities

本病表现为小的、紫红色、多角形扁平丘疹，表面有光泽，可见白色网状条纹（Wickham 纹），
皮疹多分布于手腕和前臂的屈侧，手背、前臂、颈部、骶尾部，可于搔抓部位形成线状分布的新
发皮疹（同形反应）。患者自觉瘙痒，皮疹可于数月至数年后消退，部分遗留色素沉着斑。可累
及黏膜部位，最常发生于口腔，表现为双颊黏膜为重的白色网状细纹，也可出现糜烂、溃疡、大
疱，伴有烧灼感。部分患者可发生甲扁平苔藓，表现为甲板增厚、粗糙、凹凸不平，也可出现萎
缩，特征性的表现为甲翼状胬肉——甲板消失，甲小皮向前覆盖甲床。
Lichen planus. Violaceous papules on flexural aspect of forearm, arising at a site of minor linear trauma
(Köbner phenomenon).

FIGURE 6-33. Lichen planus. White and brown irregularly shaped lesions covering top of foot.
EFIGURE 6–89. Hypertrophy—hypertrophic lichen planus. Hypertrophy in lichen planus, appearing as
red, raised lesions with dry, scaly, and slightly pitted centers.

EFIGURE 6–90. Lichenification—lichen simplex chronicus. A dry, brownish patch of thickened skin on
lower arm. Peripheral erythema is also visible.

A special form of lichen planus is erosive or ulcerative variety, in mouth or genitalia. Squamous cell
carcinoma develops in up to 5% of patients with erosive oral or genital lichen planus and may occur in
esophageal lichen planus. There is also an increased risk of squamous cell carcinoma developing in
lesions of hypertrophic lichen planus on lower extremities.

DIAGNOSIS
Histopathologic examination is diagnostic, confirmed by skin biopsy showing a band-like infiltration
of lymphocytes in dermis. A careful drug history must be taken, as, although the classical presentation of
lichen planus is usually ‘idiopathic’, the main differential is a drug-induced lichenoid reaction (see
below). Other differential diagnoses include psoriasis, pityriasis rosea, pityriasis lichenoides chronica and
secondary syphilis. Screening for underlying disease, such as hepatitis, must be considered.

DIFFERENTIAL DIAGNOSIS
Lichen planus must be distinguished from similar lesions produced by medications and other papular
lesions, such as psoriasis, lichen simplex chronicus (eFigure 6–91), graft-versus-host disease, and syphilis
(eFigure 6–27). Lichen planus on mucous membranes must be differentiated from leukoplakia. Erosive
oral lesions require biopsy and often direct immunofluorescence for diagnosis since lichen planus may
simulate other erosive diseases, especially autoimmune blistering diseases that involve oral mucosa.

FIGURE 6–91. Lichen simplex chronicus (dark raised bumps on a Black female). A photo shows dark
raised bumps on the skin of a black female.

Drug-induced lichenoid eruptions

clinically and histologically difficult to distinguish from idiopathic lichen planus. gold, quinine, proton
pump inhibitors, sulphonamides, penicillamine, antimalarials, antituberculous drugs, thiazide diuretics, β-
adrenoceptor antagonists (β-blockers), ACE inhibitors, NSAIDs, sulphonylureas, lithium and dyes in
colour developers.

Graft-versus-host disease
In acute stage of GVHD, there is a distinctive dermatitis associated with hepatitis. After about 3 months,
chronic GVHD can present with a lichenoid eruption on palms, soles, face and upper trunk. Progressive
sclerodermatous skin thickening, associated with pigmentary changes, may lead to contractures and
limited mobility.
TREATMENT
Most common medications: Sulfonamides, Tetracyclines, Quinidine, NSAIDs, β-Blockers,
Hydrochlorothiazide

A. Topical Therapy
Superpotent topical corticosteroids applied twice daily are most helpful for localized disease in
nonflexural areas. Alternatively, high-potency corticosteroid cream or ointment may be used nightly
under thin, pliable plastic film.

Topical tacrolimus appears effective in oral and vaginal erosive lichen planus, but long-term therapy is
required to prevent relapse. If tacrolimus is used, lesions must be observed carefully for development of
squamous cell carcinoma. Since absorption can occur through mucous membranes, serum tacrolimus
levels should be checked at least once if widespread mucosal application (more than 5–10 cm2) is used. If
erosive oral lichen planus are adjacent to a metal-containing amalgam, removal of amalgam may result in
clearing of erosions.

B. Systemic Therapy
Phototherapy: NB-UVB (narrow-band ultraviolet B), bath PUVA (psoralen and ultraviolet A), oral PUVA,
and combination of an oral retinoid plus PUVA (re-PUVA)

Hydroxychloroquine, acitretin, cyclosporine, and mycophenolate mofetil, are effective in mucosal and
cutaneous lichen planus. Apremilast, has reported efficacy in case series. Corticosteroids may be required
in severe cases or in circumstances where the most rapid response to treatment is desired. Unfortunately,
relapse almost always occurs as the corticosteroids are tapered, making systemic corticosteroid therapy an
impractical option for chronic lichen planus.

Management
The condition is usually self-limiting, although rarely it may persist for years, particularly oral lichen
planus. Treatment is symptomatic and potent local glucocorticoids (topical, with occlusion or by injection
for hypertrophic disease, or as oral rinse for oral involvement) may help the intense itch; short courses of
systemic glucocorticoids are sometimes required for extensive disease. UVB, PUVA or UVA1 can be
beneficial and, for recalcitrant disease, retinoids or immunosuppressants, such as ciclosporin or
methotrexate, may be needed. A low but significant risk of malignant transformation exists with persistent
oral and genital disease, so active treatment, surveillance and smoking cessation are important.

PROGNOSIS
Benign disease, usually self-limiting. but may persist for months or years (particularly oral lichen planus).
and may be recurrent. Hypertrophic lichen planus and oral lesions tend to be especially persistent, and
neoplastic degeneration has been described in chronically eroded lesions.