Annular atrophic lichen planus

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ORPHA:254411L43.8
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Overview

Annular atrophic lichen planus is a rare clinical variant of lichen planus, a chronic inflammatory disorder that primarily affects the skin. In this variant, lesions characteristically present as ring-shaped (annular) plaques or patches with central atrophy, meaning the skin in the center of the ring becomes thin, depressed, or scarred. The lesions may appear violaceous (purple-hued) at the active border, consistent with typical lichen planus morphology, while the center shows atrophic changes. This subtype predominantly affects the skin, though other forms of lichen planus can involve the mucous membranes, nails, and scalp. Annular atrophic lichen planus is classified under the broader category of lichen planus variants (ICD-10: L43.8 — Other lichen planus). It is considered an uncommon presentation and may be confused with other annular dermatoses such as granuloma annulare, morphea, or annular elastolytic giant cell granuloma. Diagnosis is typically confirmed through clinical examination and skin biopsy, which reveals the characteristic band-like lymphocytic infiltrate at the dermoepidermal junction with damage to basal keratinocytes seen in lichen planus. Treatment follows the general approach used for lichen planus and may include topical corticosteroids, topical calcineurin inhibitors, phototherapy, or systemic immunomodulatory agents in refractory cases. The condition may resolve spontaneously over months to years, though atrophic changes in the skin may persist. There is no specific targeted therapy for this variant, and management is largely symptomatic, aimed at reducing inflammation and preventing progression of skin atrophy.

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Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

Treatments

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

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

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No specialists are currently listed for Annular atrophic 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

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No travel grants are currently matched to Annular atrophic lichen planus.

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Common questions about Annular atrophic lichen planus

What is Annular atrophic lichen planus?

Annular atrophic lichen planus is a rare clinical variant of lichen planus, a chronic inflammatory disorder that primarily affects the skin. In this variant, lesions characteristically present as ring-shaped (annular) plaques or patches with central atrophy, meaning the skin in the center of the ring becomes thin, depressed, or scarred. The lesions may appear violaceous (purple-hued) at the active border, consistent with typical lichen planus morphology, while the center shows atrophic changes. This subtype predominantly affects the skin, though other forms of lichen planus can involve the muc

At what age does Annular atrophic lichen planus typically begin?

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