Lichen Sclerosus

Basics

Description

Lichen sclerosus (LS) is an inflammatory skin disease that results in white plaques and epidermal atrophy.
Also called lichen sclerosus et atrophicus or LS&A
Predilection for genital skin. Extragenital skin less common (1:5)
Genital lesions can cause significant itching and pain.
Long-standing disease can lead to adhesions and scarring of the genitalia.

Epidemiology

Female > male (6:1)
No known racial predilection
Anogenital area affected in ¢ ¼85% of patients

Incidence

Unknown
In women, the peak incidence occurs at age 40 " 60 (typically postmenopausal).
A second peak occurs in prepubertal girls age 8 " 13.

Pathophysiology

Although inflammation seems essential for initiation and progression of LS, the mechanisms leading to subsequent sclerosis remain unclear.

Etiology

Unknown
Genetics: Familial clusters; HLA-DQ7
Autoimmunity: Clusters with autoimmune disease; autoantibodies to ECM-1 found in 80% patients
Koebnerization: Exacerbation of lesions with local trauma
Inconsistently implicated: Infectious (including Borrelia burgdorferi); hormonal

Diagnosis

Diagnosis can often be made on clinical grounds, especially in children.

History

May be asymptomatic initially

Vulvar or perineal LS
- Vulvar or anal pruritus (may interfere with sleep)
- Pain/burning/soreness
- Pain with urination (dysuria)
- Genital/anal bleeding with mild trauma
- Pain with sexual intercourse (dyspareunia)
- Prepubertal girls more likely to report urinary or bowel symptoms
Extracutaneous LS
- Typically asymptomatic

Physical Exam

Exam differs based on chronicity of lesions
- Early disease: White polygonal papules coalescing into plaques
- Overtime: Smooth, porcelain-white, shiny wrinkled skin with purpura +/ " fissures or ulceration
- May progress to gradual vulvar scarring (labial adhesion/fusion/stenosis)
Distribution
- Extragenital " most commonly on back/shoulders; white, flat or raised lesions
- Genital " perivaginal and perianal, atrophic plaques in a "figure 8 " or "hour-glass " pattern
- Can occur in areas of old scars or repeated trauma (Koebner phenomenon)

Tests

Lab
Despite reports of autoantibodies in patients with LS, a work-up for autoimmune disease is generally not warranted.
Surgery
Punch biopsy of the affected area for confirmation of diagnosis
Pathological Findings
Characteristic findings on skin biopsy: Thinning/atrophy of the epidermis, homogenization of papillary dermis, with band of lymphocytic infiltrate below

Differential Diagnosis

Eczematous dermatitis
- Irritant vs. allergic contact dermatitis
- Atopic dermatitis
- Lichen simplex chronicus from scratching
Lichen planus
Morphea
Candidal vulvovaginitis
Bacterial vaginosis
Vitiligo
Postinflammatory hypopigmentation
Graft vs. host disease
Sexual abuse

Treatment

Goal: Relieve symptoms and prevent progression to scarring
Extragenital LS is less responsive than genital LS.

Medication

Extragenital LS

First line:
- If asymptomatic, no treatment needed
- If symptomatic: High-potency topical steroid or topical retinoid (1)[C]

Genital LS
If asymptomatic but evidence of active disease (ecchymosis, hyperkeratosis, progressing atrophy) treatment is recommended.

First line:
- High-potency corticosteroid ointment (1)[A]
  - Cream too irritating and has reduced potency (only use ointment)
  - Clobetasol propionate 0.05% or halobetasol propionate 0.05% ointment
  - Consensus regimen proposed: Daily 1 month, then every other day 1 month, then twice weekly 1 month (1)[C]. 30 g tube should last 3 months.
  - Maintenance: If symptoms recur, increase frequency until symptoms resolve then taper. Typically requires 30 " 60 g tube annually.
Second line:
- Topical calcineurin inhibitors (Protopic ®, Elidel ®) (1,2)[B]
  - Theoretical increased risk of neoplasia. Case reports of squamous cell carcinoma development with use of topical calcineurin inhibitor.
Third line:
- Topical retinoids (1)[C]: With hyperplastic disease
Others:
- Topical vitamin D analogs (calcipotriene; calcipotriol) (1)[C]
- Immunosuppressant agents (cyclosporine, methotrexate (MTX), MTX " + pulsed steroids) (1)[C]
- Light therapy with UVA1 (1)[C], photodynamic therapy (1)[C]
- Note: Topical testosterone creams have not been shown to have efficacy (1).

Additional Treatment

General Measures

Avoid synthetic underwear or tight clothing
Use tampons instead of panty liners
Unscented, pH-neutral soap, avoid washcloths, pat dry
Emollients are helpful

Issues for Referral

Dermatology or gynecology: For confirmation of diagnosis by punch biopsy and monitoring for squamous cell carcinoma
Surgery and/or urology if functional impairment

Surgery

A variety of destructive procedures have been reported of some benefit:
- Cryotherapy (1)[C]
- CO2 laser (1)[C]
In general, surgery should be avoided given tendency to recur (1)[C].
However, surgery may be required to repair functional impairment caused by scarring (e.g., narrowed introitus, fused labia, buried clitoris).

Ongoing Care

Follow-Up Recommendations

Treatment response considered successful if resolution of hyperkeratosis, ecchymosis, fissuring, and erosions.
Consensus groups recommend one follow-up at 3 months to assess responsiveness, then yearly or twice yearly surveillance for malignancy (given increased risk of squamous cell carcinoma) and to evaluate for steroid-induced skin atrophy.

Patient Education

Inform patients that atrophy, scarring, and associated pallor persist despite treatment.
Inform patients of changes that might indicate malignant transformation (persistent areas of well-defined erythema, ulceration, or new growth).
Patient information source:
- National Lichen Sclerosus Support Group. Website: www.lichensclerosus.org
- National Vulvodynia Association. Website: www.nva.org

Prognosis

With treatment, symptoms generally improve in days, while skin changes improve over a course of months.
Prepubertal LS often resolves spontaneously; however, may have increased risk for dyspareunia (pain with intercourse) as an adult.
Increased risk for developing squamous cell carcinoma within LS ( ¢ ¼5%)
Unclear if treating cutaneous disease decreases risk for developing squamous cell carcinoma

Complications

Increased risk of vulvar squamous cell carcinoma
Scarring with urinary tract obstruction
Pain with sexual intercourse

References

1Neill SM, Lewis FM, Tatnall FM. British Association of Dermatologists ' guideline for management of lichen sclerosus 2010. Br J Dermatol. 2010;163(4):672 " 682. [View Abstract]2Hengge UR, Krause W, Hofmann H. Multicentre, phase II trial on the safely and efficiency of topical tacrolimus ointment for treatment of lichen sclerosus. Br J Dermatol. 2006;155:1021 " 1028. [View Abstract]

Additional Reading

1Cooper SM, Gao XH, Powell JJ. Does treatment of vulvar lichen sclerosis influence its prognosis? Arch Dermatol. 2005;140:702 " 706.

Codes

ICD9

701.0 Circumscribed scleroderma

ICD10

L90.0 Lichen sclerosus et atrophicus

SNOMED

25674000 lichen sclerosus et atrophicus (disorder)
238932004 genital lichen sclerosus (disorder)
403566002 anogenital lichen sclerosus (disorder)
402715000 lichen sclerosus of female genitalia (disorder)

Clinical Pearls

An ultra-potent topical steroid (e.g., clobetasol 0.05% ointment) is first-line treatment for lichen sclerosus (LS) (1)[A].
There are currently no randomized controlled trials comparing steroid potency, frequency of application, and duration of treatment. However:
- Asymptomatic patients with evidence of clinically active LS (ecchymosis, hyperkeratosis, and progressing atrophy) should be treated (1)[A].
- Anogenital LS is associated with risk of squamous cell carcinoma (<5%) and requires long-term monitoring (1)[A].
- It is unclear if treatment decreases the risk for developing squamous cell carcinoma, but is important in preventing functional impairment (1)[A].

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