Linear lichen planus

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1Specialists8Treatment centers

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Overview

Linear lichen planus is a rare clinical variant of lichen planus (LP) in which the characteristic lesions arrange themselves in a linear or band-like distribution along the skin. Lichen planus is a chronic inflammatory condition that primarily affects the skin and mucous membranes, and in the linear form, violaceous (purple-hued), flat-topped, polygonal papules and plaques follow the lines of Blaschko or, less commonly, a dermatomal pattern. The lesions are typically pruritic (itchy) and may appear on the extremities, trunk, or face. The condition can affect individuals of any age but is more commonly reported in adults. Linear lichen planus may sometimes be confused with other linear dermatoses such as linear psoriasis, lichen striatus, or inflammatory linear verrucous epidermal nevus (ILVEN). The exact cause of linear lichen planus remains unclear, though it is believed to involve a T-cell-mediated autoimmune response directed against basal keratinocytes, similar to classic lichen planus. The linear distribution along Blaschko lines suggests a possible role of genetic mosaicism in predisposing certain skin cell populations to the inflammatory process. Histopathologically, the condition shows the hallmark features of lichen planus, including a band-like lymphocytic infiltrate at the dermoepidermal junction, irregular acanthosis, wedge-shaped hypergranulosis, and civatte bodies (apoptotic keratinocytes). Treatment of linear lichen planus follows the general approach used for lichen planus. First-line therapy typically includes potent topical corticosteroids to reduce inflammation and pruritus. Topical calcineurin inhibitors (such as tacrolimus) may also be used, particularly for sensitive areas. For more extensive or refractory cases, phototherapy (narrowband UVB), systemic corticosteroids, oral retinoids (acitretin), or other immunomodulatory agents may be considered. The condition is generally self-limiting, with most cases resolving over months to years, though post-inflammatory hyperpigmentation may persist. Regular follow-up is recommended to monitor for recurrence and to manage symptoms.

Also known as:

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

FDA & Trial Timeline

1 event
Feb 2022Clinical and Molecular Correlates of Response to First Line Treatment in Lichen Planus

University of Aberdeen

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Linear lichen planus.

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No actively recruiting trials found for Linear lichen planus at this time.

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Specialists

1 foundView all specialists →
DH
Dr Karolin Hijazi
Specialist
PI on 1 active trial

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Linear lichen planus.

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Community

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Latest news about Linear lichen planus

Disease timeline:

New recruiting trial: Clinical and Molecular Correlates of Response to First Line Treatment in Lichen Planus

A new clinical trial is recruiting patients for Linear lichen planus

Caregiver Resources

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Family & Caregiver Grants

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Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Common questions about Linear lichen planus

What is Linear lichen planus?

Linear lichen planus is a rare clinical variant of lichen planus (LP) in which the characteristic lesions arrange themselves in a linear or band-like distribution along the skin. Lichen planus is a chronic inflammatory condition that primarily affects the skin and mucous membranes, and in the linear form, violaceous (purple-hued), flat-topped, polygonal papules and plaques follow the lines of Blaschko or, less commonly, a dermatomal pattern. The lesions are typically pruritic (itchy) and may appear on the extremities, trunk, or face. The condition can affect individuals of any age but is more

How is Linear lichen planus inherited?

Linear lichen planus follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

Which specialists treat Linear lichen planus?

1 specialists and care centers treating Linear lichen planus are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.