Subacute cutaneous lupus erythematosus

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Overview

Subacute cutaneous lupus erythematosus (SCLE) is a specific subset of cutaneous lupus erythematosus characterized by widespread, photosensitive, non-scarring skin lesions. It primarily affects the skin, though some patients may develop mild systemic manifestations. SCLE accounts for approximately 10-15% of all lupus erythematosus cases and predominantly affects women. The condition presents with two main morphological patterns: annular/polycyclic lesions with raised red borders and central clearing, or papulosquamous (psoriasiform) lesions that resemble psoriasis. These lesions typically appear on sun-exposed areas such as the upper back, shoulders, extensor surfaces of the arms, neck, and upper chest, while the face is relatively spared compared to other forms of lupus. The lesions heal without scarring but may leave long-lasting depigmentation or hyperpigmentation. SCLE is strongly associated with anti-Ro/SSA antibodies, which are found in approximately 70-90% of affected individuals. Anti-La/SSB antibodies are also frequently present. The condition has a notable association with HLA-DR3 and HLA-B8 haplotypes. Importantly, SCLE can be drug-induced in a significant proportion of cases, with common culprits including hydrochlorothiazide, terbinafine, calcium channel blockers, ACE inhibitors, proton pump inhibitors, and TNF-alpha inhibitors. While most patients with SCLE have a relatively benign course compared to systemic lupus erythematosus (SLE), approximately 10-15% may meet criteria for SLE, and patients should be monitored for systemic involvement including arthralgia, fatigue, and mild renal or hematologic abnormalities. Treatment of SCLE centers on strict sun protection and avoidance of photosensitizing triggers. First-line pharmacological therapy includes topical corticosteroids and antimalarial agents, particularly hydroxychloroquine. For refractory cases, additional immunosuppressive or immunomodulatory agents such as methotrexate, mycophenolate mofetil, dapsone, or thalidomide may be considered. In drug-induced SCLE, discontinuation of the offending medication is essential and often leads to resolution of skin lesions, though this may take weeks to months. Smoking cessation is also recommended, as tobacco use can reduce the efficacy of antimalarial therapy.

Clinical phenotype terms— hover any for plain English:

Anti-Ro/SS-A antibody positivityHP:0033555Rheumatoid factor positiveHP:0002923Psoriasiform lesionHP:0025526Annular cutaneous lesionHP:0025528Antiphospholipid antibody positivityHP:0003613Discoid lupus rashHP:0007417Anti-dsDNA antibody positivityHP:0020151Linear IgG deposits along the epidermal basement membrane zoneHP:0031540Linear C3 deposits along the epidermal basement membrane zoneHP:0031541Anti-La/SS-B antibody positivityHP:0032235Anti-histone antibody positivityHP:0033558Malar rashHP:0025300CheilitisHP:0100825Vasculitis in the skinHP:0200029
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 Subacute cutaneous lupus erythematosus.

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No actively recruiting trials found for Subacute cutaneous lupus erythematosus at this time.

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No specialists are currently listed for Subacute cutaneous lupus erythematosus.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

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 Subacute cutaneous lupus erythematosus.

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Caregiver Resources

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

Common questions about Subacute cutaneous lupus erythematosus

What is Subacute cutaneous lupus erythematosus?

Subacute cutaneous lupus erythematosus (SCLE) is a specific subset of cutaneous lupus erythematosus characterized by widespread, photosensitive, non-scarring skin lesions. It primarily affects the skin, though some patients may develop mild systemic manifestations. SCLE accounts for approximately 10-15% of all lupus erythematosus cases and predominantly affects women. The condition presents with two main morphological patterns: annular/polycyclic lesions with raised red borders and central clearing, or papulosquamous (psoriasiform) lesions that resemble psoriasis. These lesions typically appea

How is Subacute cutaneous lupus erythematosus inherited?

Subacute cutaneous lupus erythematosus follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Subacute cutaneous lupus erythematosus typically begin?

Typical onset of Subacute cutaneous lupus erythematosus is adult. Age of onset can vary across affected individuals.