Cutaneous lupus erythematosus (discoid and subacute)
Cutaneous lupus erythematosus spans a chronic scarring form (discoid lupus erythematosus, the most common chronic cutaneous subtype) and a non-scarring, strongly photosensitive subacute form tied to anti-Ro/SSA antibodies, both of which can occur with or without systemic lupus erythematosus. Diagnosis rests on the clinical pattern plus skin biopsy and serology, and management is staged from sun protection and topical or intralesional corticosteroid through hydroxychloroquine for extensive or refractory disease, with culprit-drug withdrawal curing most drug-induced subacute cutaneous lupus.
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What this article covers
- Overview
Definition: cutaneous lupus erythematosus (CLE) is a family of lupus-specific skin diseases classified as acute (ACLE), subacute (SCLE), and chronic cutaneous lupus erythematosus (CCLE), of which…
- Epidemiology
DLE is the most common chronic cutaneous lupus subtype and poses approximately a 10% risk of progression to underlying systemic disease.
- Risk factors
Ultraviolet light exposure: the central trigger for SCLE, which is strongly photosensitive, and a contributor to DLE and ACLE.
- Pathophysiology
Loss of tolerance to nuclear self-antigens drives autoantibody and immune complex formation, activating innate immunity through toll-like receptors 7 and 9 and type I interferon-alpha, the shared upstream pathway across all CLE subtypes.
- Clinical features
Discoid lupus erythematosus (chronic cutaneous LE), the hallmark presentation: well-demarcated, erythematous, scaly plaques with follicular plugging that evolve from a red-to-violet…
- Differential diagnosis
Dermatomyositis: the most important mimic of a photosensitive facial rash; Gottron papules over the MCP/PIP joints and a heliotrope rash over the eyelids are absent in lupus, though biopsy can show similar interface dermatitis in both.
- Diagnosis
The diagnosis is confirmed by clinical appearance plus skin biopsy: biopsy of an active lesion shows interface dermatitis with vacuolar degeneration of the basal keratinocyte layer; discoid lesions…
- Management
Sun protection is foundational for every subtype and lesion: apply broad-spectrum sunscreen SPF 30 or higher daily to exposed skin, reapplied at least every 2 hours during sun exposure, with a…
- Complications
Permanent scarring alopecia from scalp discoid lupus, which does not reverse even with control of active inflammation.
- Prognosis
Isolated cutaneous disease has a favorable prognosis: most DLE (~90%) and most SCLE (75%) never progress to systemic lupus erythematosus.
- Prevention
Photoprotection is the primary preventive measure for flares of every subtype: broad-spectrum sunscreen SPF 30 or higher at approximately 2 mg/cm2, reapplied at least every 2 hours outdoors, with protective clothing and a broad-brimmed hat.
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Related articles
- Systemic lupus erythematosus
Systemic lupus erythematosus is a chronic multisystem autoimmune disease in which antinuclear and other autoantibodies drive immune-complex deposition and organ injury across skin, joints, kidneys, blood, serosa, and the nervous system. Diagnosis rests on a positive antinuclear antibody plus weighted 2019 EULAR/ACR criteria totaling 10 or more points. Hydroxychloroquine is lifelong first-line therapy for nearly every patient, with glucocorticoids and steroid-sparing immunosuppression added for moderate to severe or organ-threatening disease.
- Drug-induced lupus
Drug-induced lupus is a milder, reversible lupus-like syndrome triggered by an offending medication, marked by transient antinuclear and antihistone antibodies with sparing of the kidney and central nervous system. Management is stopping the culprit drug, with an NSAID or a short low-dose glucocorticoid course for residual arthritis, fever, or serositis.
- Photosensitizing drug reactions
Photosensitizing drug reactions are drug-induced skin eruptions confined to sun-exposed areas, occurring as phototoxic reactions (common, dose-dependent, an exaggerated sunburn from UVA-absorbing drugs) or photoallergic reactions (uncommon, a delayed eczematous type IV response). Diagnosis is clinical from the photodistribution and drug history, and management is stopping the culprit, photoprotection, and topical corticosteroids.
- Rosacea
Rosacea is a chronic inflammatory facial skin disorder with recurrent flushing that produces persistent central-face erythema, telangiectasias, and papulopustules without comedones, triggered by heat, sun, alcohol, and spicy food. Diagnosis is clinical based on subtype; treatment is matched to subtype, with topical metronidazole, azelaic acid, or ivermectin and oral doxycycline for papulopustular disease, laser therapy for telangiectasias, and laser or surgery for rhinophyma.
- Dermatomyositis
Dermatomyositis is a complement-mediated inflammatory myopathy causing symmetric proximal weakness with pathognomonic skin findings, heliotrope rash and Gottron papules, that separate it from polymyositis. It carries a 3- to 12-fold increased malignancy risk and a distinct interstitial lung disease burden that drive workup and therapy. First-line treatment is high-dose prednisone with an early steroid-sparing agent.
- Psoriasis
Psoriasis is a chronic, relapsing, immune-mediated dermatosis of accelerated keratinocyte turnover, classically presenting as sharply demarcated, silvery-scaled plaques on extensor surfaces, with guttate, inverse, pustular, and erythrodermic variants and an associated psoriatic arthritis. Mild-to-moderate disease is treated with emollients and topical corticosteroids with or without a vitamin-D analogue (calcipotriene 0.005% twice daily), while widespread (over 10% body surface area), recalcitrant, or severe disease escalates to phototherapy, methotrexate (weekly single dose 7.5 to 25 mg), or a biologic such as adalimumab, ustekinumab, or secukinumab, never a systemic glucocorticoid.
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- Seborrheic dermatitis
Seborrheic dermatitis is a common chronic inflammatory reaction to Malassezia yeast that produces ill-defined erythema with dry or greasy scale in sebum-rich sites such as the scalp, nasolabial folds, brows, and ears. Diagnosis is clinical based on distribution; first-line treatment is topical ketoconazole cream or shampoo with a short course of low-potency hydrocortisone for inflamed flares, and disseminated or severe disease should prompt screening for HIV or a neurologic disorder.
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