Rheumatology and Orthopedics· Topic 17.5 Systemic lupus erythematosus and overlap connective tissue disease· 17.5.4

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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