Annular lichen planus

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Overview

Annular lichen planus is a rare morphological variant of lichen planus, a chronic inflammatory disorder that primarily affects the skin. In this variant, the characteristic violaceous (purple-hued), flat-topped papules of lichen planus arrange themselves in a ring-shaped (annular) configuration, often with central clearing or atrophy. The lesions most commonly appear on the skin of the extremities, trunk, and genital areas, particularly the glans penis in males, where it is most frequently recognized. Annular lichen planus may also be referred to as ring-shaped lichen planus. The condition arises from a T-cell-mediated autoimmune response directed against basal keratinocytes in the skin, leading to inflammation at the dermal-epidermal junction. Patients typically present with well-defined annular plaques that may be pruritic (itchy) or asymptomatic. The lesions can range from a few millimeters to several centimeters in diameter. Histopathological examination reveals the classic features of lichen planus, including a band-like lymphocytic infiltrate at the dermal-epidermal junction, irregular acanthosis, wedge-shaped hypergranulosis, and civatte bodies (apoptotic keratinocytes). Treatment of annular lichen planus follows the general approach used for lichen planus and is primarily aimed at symptom relief and resolution of lesions. First-line therapy typically includes potent topical corticosteroids. Other treatment options include topical calcineurin inhibitors (such as tacrolimus), phototherapy (narrowband UVB or PUVA), and systemic therapies such as oral corticosteroids, retinoids, or immunosuppressants for refractory or widespread disease. The condition may resolve spontaneously over months to years, though recurrences are possible. Patients should be monitored for involvement of other sites commonly affected by lichen planus, including the oral mucosa, nails, and scalp.

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Inheritance

Multifactorial

Caused by a mix of several genes and environmental factors

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

Treatments

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

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

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No specialists are currently listed for Annular lichen planus.

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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 Annular lichen planus.

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

Common questions about Annular lichen planus

What is Annular lichen planus?

Annular lichen planus is a rare morphological variant of lichen planus, a chronic inflammatory disorder that primarily affects the skin. In this variant, the characteristic violaceous (purple-hued), flat-topped papules of lichen planus arrange themselves in a ring-shaped (annular) configuration, often with central clearing or atrophy. The lesions most commonly appear on the skin of the extremities, trunk, and genital areas, particularly the glans penis in males, where it is most frequently recognized. Annular lichen planus may also be referred to as ring-shaped lichen planus. The condition ar

How is Annular lichen planus inherited?

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

At what age does Annular lichen planus typically begin?

Typical onset of Annular lichen planus is adult. Age of onset can vary across affected individuals.