Lichen Planus

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Chapter 77 Lichen Planus

INTRODUCTION

Description: Lichen planus is a non-neoplastic epithelial disorder that affects glabrous skin, hair-bearing skin and scalp, nails, mucous membranes, or the oral cavity and the vulva.

Prevalence: Unknown, but relatively common.

Predominant Age: 30 to 60 years.

Genetics: No genetic pattern.

ETIOLOGY AND PATHOGENESIS

Causes: Unknown. Proposed—autoimmune disorder, possibly initiated by certain drugs such as beta blockers, angiotensin-converting enzyme (ACE) inhibitors.

Risk Factors: None known.

CLINICAL CHARACTERISTICS

Signs and Symptoms

• Red erosion and ulceration of the vulva and inner aspects of the labia minora (may precede oral lesions by years; 33% of patients)

• Loss of the labia minora with scarring, adhesions, and narrowing common (complete obliteration of the vagina possible); dyspareunia and postcoital bleeding common

• Oral lesions—reticulated gray, lacy pattern (Wickham’s striae) with gingivitis (vulvar involvement in 50% of patients with oral lesions)

DIAGNOSTIC APPROACH

Workup and Evaluation

Laboratory: No evaluation indicated.

Imaging: No imaging indicated.

Special Tests: Skin biopsy (taken from the nearby intact skin or mucous membranes rather than the ulcer). Direct immunofluorescence testing on fresh tissue.

Diagnostic Procedures: History, physical examination, and biopsy.

Pathologic Findings

Chronic inflammatory cell infiltrate (lymphocytes and plasma cells) involving the superficial dermis and the basal and parabasal epithelium. Liquefaction necrosis with colloid bodies may be present. Prominent acanthosis with a prominent granular layer and hyperkeratosis. Ulceration and bullae may be present. Hyperkeratosis is absent in the vulvar tissues.

MANAGEMENT AND THERAPY

Nonpharmacologic

General Measures: Evaluation, local cleansing, antipruritics.

Specific Measures: Therapy is often difficult, chronic, and prone to failure or relapse. Therapies include steroids, retinoids, griseofulvin, dapsone, cyclosporine, and surgery. Vaginal dilators may be necessary to maintain vaginal caliber.

Diet: No specific dietary changes indicated.

Activity: No restriction.

Patient Education: Reassurance; American College of Obstetricians and Gynecologists Patient Education Pamphlet AP088 (Disorders of the Vulva).

Drug(s) of Choice

Topical steroids (betamethasone valerate 0.1% ointment or hydrocortisone 25 mg vaginal suppository daily) or

Griseofulvin (250 mg PO twice a day) or

Dapsone (50 to 100 mg PO daily; after negative results of screening for glucose-6-phosphate dehydrogenase) or

Isotretinoin (Accutane) 0.5 to 1 mg/kg/day in divided doses (or etretinate [Tegison] 0.75 to 1 mg/kg/day in two doses) or

Cyclosporine (1 mg/kg/day, increased weekly by 0.5 mg/kg/day up to 3 to 5 mg/kg/day).

Contraindications: Vulvar cancer. Isotretinoin and etretinate are teratogenic and must not be given during pregnancy or if there is a potential for pregnancy.

Precautions: Continued or prolonged use of topical steroids may result in thinning of the skin outside the area of lichen sclerosus with subsequent atrophy and traumatic injury (splitting and cracking). The use of dapsone, isotretinoin, etretinate, or cyclosporine requires careful monitoring of complete blood counts, liver function tests, cholesterol, triglycerides, electrolytes, urea nitrogen, creatinine, and creatinine clearance. Reliable contraception must be maintained if isotretinoin or etretinate is used.

Interactions: See individual agents.

FOLLOW-UP

Patient Monitoring: Because malignant change is possible, long-term follow-up is required.

Prevention/Avoidance: None.

Possible Complications: Vulvar lesions are often chronic and may undergo malignant change.

Expected Outcome: Chronic therapy required with relapses common.

MISCELLANEOUS

Pregnancy Considerations: No effect on pregnancy.

ICD-9-CM Codes: 697.0.

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Valdatta L, Tuinder S, Thione A, et al. Lichen planus cutis and squamous cell carcinoma. Plast Reconstr Surg. 2004;113:1085.

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