Lichen planus pigmentosus

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1Active trials2Specialists8Treatment centers

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Overview

Lichen planus pigmentosus (LPP) is a rare variant of lichen planus characterized by the insidious onset of dark brown to slate-gray hyperpigmented macules and patches on the skin, predominantly affecting sun-exposed areas such as the face, neck, and flexural folds. It is also sometimes referred to as lichen planus pigmentosus-inversus when it predominantly involves intertriginous (skin fold) areas. The condition primarily affects the integumentary system (skin) and is considered a pigmentary disorder within the lichen planus spectrum. It is more commonly reported in individuals with darker skin types, particularly in populations from the Indian subcontinent, the Middle East, Latin America, and other tropical regions. Clinically, LPP presents as diffuse or reticulated (net-like) hyperpigmented patches that develop gradually without a preceding inflammatory phase, which distinguishes it from classic lichen planus. The lesions are typically asymptomatic, though some patients may experience mild pruritus (itching). Unlike classic lichen planus, the characteristic Wickham striae and violaceous papules are usually absent. Histopathologically, the condition shows pigment incontinence with melanophages in the upper dermis, along with a lichenoid lymphocytic infiltrate at the dermoepidermal junction and basal layer degeneration. The exact cause of lichen planus pigmentosus remains unclear, though it is thought to involve an immune-mediated mechanism similar to other forms of lichen planus. Potential triggers include mustard oil application, certain hair dyes, hepatitis C virus infection, and other environmental factors. Treatment is often challenging and results can be variable. Therapeutic options include topical corticosteroids, topical calcineurin inhibitors (such as tacrolimus), topical retinoids, and photoprotection with broad-spectrum sunscreens. Chemical peels, laser therapy, and oral dapsone have also been used with varying degrees of success. The condition tends to follow a chronic, relapsing course, and complete resolution of pigmentation may be difficult to achieve.

Also known as:

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

FDA & Trial Timeline

1 event
Mar 2020The Efficacy of Tranexamic Acid in the Treatment of Lichen Planus Pigmentosus and Erythema Dyschromicum Perstans

Henry Ford Health System — PHASE2

TrialRECRUITING

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

Treatments

No FDA-approved treatments are currently listed for Lichen planus pigmentosus.

1 clinical trialare actively recruiting — trials can provide access to cutting-edge therapies.

View clinical trials →

Clinical Trials

1 recruitingView all trials with filters →
Phase 21 trial
The Efficacy of Tranexamic Acid in the Treatment of Lichen Planus Pigmentosus and Erythema Dyschromicum Perstans
Phase 2
Actively Recruiting
PI: Henry W Lim, MD (Henry Ford HS) · Sites: Detroit, Michigan · Age: 1899 yrs

Specialists

2 foundView all specialists →
HM
Henry W Lim, MD
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 Lichen planus pigmentosus.

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Community

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Latest news about Lichen planus pigmentosus

Disease timeline:

New recruiting trial: The Efficacy of Tranexamic Acid in the Treatment of Lichen Planus Pigmentosus and Erythema Dyschromicum Perstans

A new clinical trial is recruiting patients for Lichen planus pigmentosus

Caregiver Resources

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Social Security Disability

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

Common questions about Lichen planus pigmentosus

What is Lichen planus pigmentosus?

Lichen planus pigmentosus (LPP) is a rare variant of lichen planus characterized by the insidious onset of dark brown to slate-gray hyperpigmented macules and patches on the skin, predominantly affecting sun-exposed areas such as the face, neck, and flexural folds. It is also sometimes referred to as lichen planus pigmentosus-inversus when it predominantly involves intertriginous (skin fold) areas. The condition primarily affects the integumentary system (skin) and is considered a pigmentary disorder within the lichen planus spectrum. It is more commonly reported in individuals with darker ski

How is Lichen planus pigmentosus inherited?

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

At what age does Lichen planus pigmentosus typically begin?

Typical onset of Lichen planus pigmentosus is adult. Age of onset can vary across affected individuals.

Are there clinical trials for Lichen planus pigmentosus?

Yes — 1 recruiting clinical trial is currently listed for Lichen planus pigmentosus on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat Lichen planus pigmentosus?

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