Subacute Cutaneous Lupus Erythematosus (SCLE) Clinical Presentation

Updated: Apr 14, 2020
  • Author: Elizabeth Brezinski Wallace, MD; Chief Editor: William D James, MD  more...
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Presentation

History

Subacute cutaneous lupus erythematosus (SCLE) typically manifests in 1 of 2 forms: annular/polycyclic or psoriasiform/papulosquamous. SCLE lesions can begin as erythematous macules or papules that evolve into either annular polycyclic or psoriasiform plaques. Lesions typically occur in a photosensitive distribution. Many patients notice that sun exposure results in an exacerbation of their disease, and some report worsening each spring and summer. Photosensitivity is more frequently observed in patients with SCLE compared with discoid lupus erythematosus (DLE) lesions. Patients may report mild pruritus, but most persons with SCLE are asymptomatic.

Approximately 50% of patients with SCLE have accompanying joint involvement. Arthralgias are common, often symmetrical, and usually affect small joints, such as the wrists or those of the hands. Arthritis may occur but is unusual (< 2%).

Patients commonly report fatigue. Some patients have Sjögren syndrome, while others note dryness of their eyes and mouth. Patients may manifest symptoms of systemic lupus erythematosus; therefore, the history should include an assessment for symptoms of pleuritis, pericarditis, neurologic involvement, and renal impairment.

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Physical Examination

The primary lesion of subacute cutaneous lupus erythematosus (SCLE) is an erythematous papule or a small plaque, often with slight scaling. Primary lesions expand and may merge and eventually form either plaques with scaling, in the papulosquamous variant, or annular and/or polycyclic lesions, in the annular variant. (See the images below.)

Early lesions of subacute cutaneous lupus erythema Early lesions of subacute cutaneous lupus erythematosus may simulate polymorphous light eruption.
Papulosquamous lesions of subacute cutaneous lupus Papulosquamous lesions of subacute cutaneous lupus erythematosus may simulate psoriasis.
Annular lesions of subacute cutaneous lupus erythe Annular lesions of subacute cutaneous lupus erythematosus.

Papulosquamous lesions may mimic psoriasis or lichen planus, while annular lesions may mimic erythema annulare centrifugum or tinea corporis. Most patients exhibit one predominant type of lesion. Some patients also manifest isolated lesions of discoid lupus erythematosus (DLE) during the course of their disease.

SCLE lesions primarily are photodistributed. Affected areas frequently include the upper back, shoulders, extensor arms, neck, and upper torso, while the face is often spared. When lesions occur on the lower extremities, they often are purpuric. Early lesions of SCLE may be difficult to distinguish from polymorphous light eruption (PMLE). Despite this, and the fact that both disorders have prominent photosensitivity, SCLE and PMLE are distinct disorders with differing histopathologic features.

Unusual variants of SCLE

Neonatal lupus erythematosus (NLE) most often manifests as a nonscarring form of lupus erythematosus (see the image below). Skin lesions are worsened by ultraviolet light and usually resolve by age 4-6 months. The cutaneous findings resemble those of SCLE. NLE lesions are typically annular erythematous plaques with a slight scale, which appear predominately on the scalp, neck, or face, but similar plaques may appear on the trunk or extremities. Infants with NLE should be evaluated for cardiac, hematologic, hepatic, and neurologic involvement. NLE is thought to be caused by the transplacental passage of maternal autoantibodies, most often anti-SS-A/Ro, but also anti-SSB/La or anti-RNP antibodies.

Neonatal lupus erythematosus. Neonatal lupus erythematosus.

Annular erythema of Sjögren syndrome has been reported in Japanese and Polynesian patients. The authors believe that this is not a distinct entity, but rather SCLE with Sjögren syndrome in a particular ethnic population. [24]

An infrequent variant including erythema multiforme–like lesions in association with lupus erythematosus (known as Rowell syndrome) and chilblains may exist, but it is not clear whether this is a distinct entity.

Patients with SCLE may have nonspecific cutaneous manifestations of lupus erythematosus, such as livedo reticularis, palpable purpura, urticaria, ischemic changes of the distal fingertips (resulting from Raynaud phenomenon), or mucosal leukoplakic or ulcerative lesions.

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