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.
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
1 eventHenry Ford Health System — PHASE2
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 →Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Lichen planus pigmentosus.
Community
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Start the conversation →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
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
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.