Does the malar rash of systemic lupus erythematosus spare the nasolabial folds?

Does the Malar Rash of Systemic Lupus Erythematosus Spare the Nasolabial Folds?

Yes, the classic malar ("butterfly") rash of systemic lupus erythematosus characteristically spares the nasolabial folds, which is a key distinguishing feature from other facial erythematous conditions such as rosacea. 1, 2

Clinical Characteristics of the Malar Rash

The malar rash is one of the most recognizable cutaneous manifestations of SLE and appears in approximately 80% of patients with systemic lupus erythematosus. 3 This acute cutaneous lupus erythematosus (ACLE) presents as:

• Erythematous patches distributed over the malar eminences (cheeks) and nasal bridge in a butterfly configuration 2, 4
• Characteristic sparing of the nasolabial folds, which helps differentiate it from rosacea and other facial dermatoses 1, 2
• Photosensitive nature, often triggered or worsened by sun exposure 3, 4
• May be transient or persistent, with variable duration depending on disease activity 1

Dermoscopic Features That Aid Diagnosis

When clinical examination is uncertain, dermoscopy can provide additional diagnostic clarity:

• "Inverse strawberry" pattern (reddish/salmon-colored follicular dots surrounded by white halos) is present in approximately 54% of SLE malar rash cases, with 86.7% specificity for lupus versus rosacea 5
• This contrasts with rosacea, which shows network-like vessels (vascular polygons) in 93.3% of cases 5

Important Diagnostic Caveats

While nasolabial sparing is characteristic, clinicians should be aware of atypical presentations:

• Unilateral facial involvement can occur, though it is uncommon and may initially be misdiagnosed as cellulitis, blepharitis, or other dermatoses 6
• The absence of typical malar rash does not exclude SLE—only 30% of patients present initially with skin lesions, though virtually all develop cutaneous manifestations during disease course 3
• Malar rash strongly correlates with systemic disease activity, making it an important clinical marker for multiorgan involvement 3, 2

Differential Diagnosis Considerations

The nasolabial sparing feature is particularly useful when distinguishing malar rash from:

• Erythematotelangiectatic rosacea, which typically involves the nasolabial folds and shows characteristic malar erythema with telangiectasias 7, 5
• Seborrheic dermatitis, which prominently affects the nasolabial folds
• Contact dermatitis or other inflammatory facial conditions

Clinical Management Implications

When malar rash is identified with nasolabial sparing:

• Perform skin biopsy for histologic confirmation if the diagnosis is uncertain, as several non-lupus dermatoses can mimic the presentation 1
• Order comprehensive autoantibody panel (ANA, anti-dsDNA, anti-Ro/SSA, anti-La/SSB) and complement levels (C3, C4) to assess for systemic involvement 1
• Initiate hydroxychloroquine (maximum 5 mg/kg real body weight) as first-line therapy, which reduces disease activity and improves survival 8, 1, 9
• Implement strict photoprotection (SPF ≥60, sun avoidance, protective clothing) as mandatory for all SLE patients 1, 9
• Apply topical glucocorticoids for localized cutaneous lesions 9, 4