Lichen sclerosus

Overview 
Theory 
Diagnosis 
Management 
Follow up 
Resources 

Last reviewed: 21 Oct 2024

Last updated: 12 Sep 2024

Lichen sclerosus (LS, also known as balanitis xerotica obliterans in men) is an inflammatory skin condition with a chronic relapsing and remitting course.

Characteristic findings on exam of the cutaneous lesions are atrophic white plaques, typically found in the anogenital area, which may lead to scarring and anatomic changes.

There is a small but significant risk (approximately 5%) of vulvar squamous cell carcinoma in women with anogenital LS, especially if left untreated.

If extragenital disease is found, genital exam is recommended, as extragenital disease in isolation is rare.

The condition is often associated with autoimmune conditions (such as thyroid disease and vitiligo) in women.

Diagnosis is predominantly clinical, but confirmation by biopsy is recommended if there is diagnostic uncertainty or suspicion of malignancy.

First-line treatment for all patients with anogenital LS is a very-high potency corticosteroid. Initial management comprises daily therapy until the disease is controlled. In men and boys with genital LS, early circumcision is often curative.

Treatment of extragenital LS should be overseen by a specialist who will individualize the approach according to the site involved, typically only initiating therapy if symptoms are bothersome to the patient.

Once disease control is achieved, maintenance therapy (usually with a topical corticosteroid) may be required for the prevention of anogenital disease flares in particular.

Definition

Lichen sclerosus (LS) is a chronic inflammatory skin condition. LS typically affects the genitalia but can also manifest as extragenital lesions.[1][2] In women, it predominantly affects the vulva and anus and is associated with pruritus, pain, and dyspareunia; it can also lead to scarring and increases the risk of malignancy.[3][4][5] In men, genital disease almost exclusively occurs in those who are uncircumcised and may cause pain, dyspareunia, and scarring-related signs and symptoms such as changes in urinary stream and phimosis.[6] Girls may present with similar symptoms as women; however, boys with the condition are usually asymptomatic and often present with phimosis.[7][8][9]

History and exam

Key diagnostic factors

changes in skin color and/or texture
purpura and/or ecchymoses
pruritus
sclerosis/scarring and/or loss of normal anatomic structures
dyspareunia and/or sexual dysfunction
fissures

Full details

Other diagnostic factors

skin irritation and/or discomfort and/or pain
uncircumcised state
erosions
excoriations
Koebnerization
urinary dribbling and/or incontinence
dysuria
lichenification and/or hyperkeratosis
painful defecation
constipation
anogenital and/or vulval bleeding
changes in urinary stream
lesions of the oral cavity and/or lips

Full details

Risk factors

family history
uncircumcised state
urinary incontinence (in men/boys)
female sex

Full details

Diagnostic tests

1st tests to order

clinical diagnosis

Full details

Tests to consider

skin biopsy

Full details

Emerging tests

optical coherence tomography (OCT)
high-frequency ultrasound

Treatment algorithm

ACUTE

initial presentation

ONGOING

response to initial treatment

refractory disease

Contributors

Authors

Melissa Mauskar, MD, FAAD

Associate Professor

Department of Dermatology

Department of Obstetrics and Gynecology

UT Southwestern Medical Center

Dallas

Disclosures

MM has received a grant from the Dermatology Foundation to study lichen sclerosus in postmenopausal women. MM has given lectures on lichen sclerosus.

Christina Kraus, MD, FAAD

Assistant Professor

Department of Dermatology

UC Irvine Health

Irvine

Disclosures

CK is a recipient of the Dermatology Foundation Career Development Award and a consultant for Nuvig Therapeutics. CK has received consulting fees from LEO Pharma.

Peer reviewers

Libby (Irene Elizabeth) Edwards, MD

Director

Southeast Vulvar Clinic

Charlotte

Disclosures

LE is co-author of the Genital Dermatology Manual, 1-4th edition, Wolters Kluwer, and a speaker for Symposium Medicus.

Fiona M. Lewis, MB BCh, MD, FRCP

Consultant Dermatologist

St John’s Institute of Dermatology

Guy’s & St Thomas’ Foundation Trust

London

Disclosures

FML has received reimbursement for editing textbooks; has written national and international guidelines for the management of lichen sclerosus (the British Association of Dermatology, the International Union Against Sexually Transmitted Infections, and the British Association for Sexual Health and HIV); and has received reimbursement for lecturing on lichen sclerosus and vulval disease, but not from any commercial organization or for endorsing any drugs.

Differentials
- Vitiligo
- Lichen planus
- Candidiasis
More Differentials
Guidelines
- EDF EuroGuiDerm guideline: lichen sclerosus
- NASPAG clinical opinion: diagnosis and management of lichen sclerosis in pediatric and adolescent patients
More Guidelines
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Lichen sclerosus

Summary

Definition

History and exam

Key diagnostic factors

Other diagnostic factors

Risk factors

Diagnostic tests

1st tests to order

Tests to consider

Emerging tests

Treatment algorithm

initial presentation

response to initial treatment

refractory disease

Contributors

Authors

Melissa Mauskar, MD, FAAD

Disclosures

Christina Kraus, MD, FAAD

Disclosures

Peer reviewers

Libby (Irene Elizabeth) Edwards, MD

Disclosures

Fiona M. Lewis, MB BCh, MD, FRCP

Disclosures

Differentials

Guidelines

Log in or subscribe to access all of BMJ Best Practice

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