Various Manifestations of Systemic Lupus Erythematosus

July 1, 2003
Yusuf Yazici, MD

,
Doruk Erkan, MD

,
Rachelle Scott, MD

,
Stephen Paget, MD

The manifestations of this multisystemdisorder range from relativelybenign, self-limited cutaneous involvementto severe, potentially fatal systemicillness. Skin lesions associatedwith lupus erythematosus (LE) areclassified histologically as LE-specificor LE-nonspecific.

The manifestations of this multisystemdisorder range from relativelybenign, self-limited cutaneous involvementto severe, potentially fatal systemicillness. Skin lesions associatedwith lupus erythematosus (LE) areclassified histologically as LE-specificor LE-nonspecific.LE-specific lesions. These arefurther categorized as acute, subacute,or chronic. The most characteristicand well-known acute skin sign ofsystemic lupus erythematosus (SLE)is the "butterfly" rash; however, it occursin only 40% of patients. The rash(A), which is commonly precipitatedby sunlight, consists of erythematouspapules and plaques in a malar distribution.Immunofluorescent stainingtypically reveals immune deposits ofprimary IgM at the dermal-epidermaljunction.1Other acute lesions of LE includegeneralized erythema and, rarely, bullouslesions. These lesions mimic extensiveburns and, depending on theextent of involvement, can be lifethreatening.They are caused by basementmembrane antibodies againsttype IV collagen and clinically andhistologically resemble dermatitisherpetiformis.2More than half of patients withSLE have photosensitivity that canlead to subacute cutaneous lesionsand can also exacerbate systemic disease.These lesions are nonscarringand do not produce atrophy. Theystart as erythematous papules orsmall plaques with scale and evolveinto either polycyclic plaques withcentral clearing or papulosquamous lesions that resemble psoriasis orlichen planus (B). To help reduce therisk of subacute cutaneous lesions,advise patients to always cover exposedareas with sunscreen that hasa sun protection factor of at least 15.The most common chronic skinmanifestations of LE are discoid lesions(C). These round, well-demarcated lesionsare characterized by erythema,telangiectasia, adherent scale, follicularplugging, dyspigmentation, atrophy,and scarring. Discoid lesions canbe distributed locally, usually on thehead or neck, or widely, involving anypart of the body. Patients seldom, ifever, have systemic symptoms. Exceptfor their associated atrophy andscarring, discoid lesions are similar tothose of subacute LE.LE-nonspecific lesions.Alopeciais a common finding in SLE (D).It can be transient (related to diseaseexacerbations) or permanent (secondaryto the scarring of disseminatedLE). Mucous membrane lesionsmay cause ulcers of the mouth, nose,or other mucocutaneous surface.Cutaneous vasculitic lesions appear aspalpable purpura, subcutaneous nodules,splinter hemorrhages, telangiectasia,palmar erythema, or nailfoldulcerations.