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Lichen Sclerosus

Basics

Description

- 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)

- Eczematous dermatitis - Irritant vs. allergic contact dermatitis - Atopic dermatitis - Lichen simplex chronicus from scratching

  • 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 LSVulvar or anal pruritus (may interfere with sleep)Pain/burning/sorenessPain with urination (dysuria)Genital/anal bleeding with mild traumaPain with sexual intercourse (dyspareunia)Prepubertal girls more likely to report urinary or bowel symptoms
  • Extracutaneous LS

Physical Exam

  • Exam differs based on chronicity of lesionsEarly disease: White polygonal papules coalescing into plaquesOvertime: Smooth, porcelain-white, shiny wrinkled skin with purpura +/ " fissures or ulcerationMay progress to gradual vulvar scarring (labial adhesion/fusion/stenosis)
  • DistributionExtragenital " most commonly on back/shoulders; white, flat or raised lesionsGenital " perivaginal and perianal, atrophic plaques in a "figure 8 " or "hour-glass " patternCan 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 dermatitisIrritant vs. allergic contact dermatitisAtopic dermatitisLichen 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 neededIf 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% ointmentConsensus 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.orgNational 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].