SYSTEMIC LUPUS ERYTHEMATOSUS

By Gary M. White, MD


Systemic lupus erythematosus


Systemic lupus erythematosus (SLE) is a multi-system autoimmune disease characterized by the presence of antinuclear antibodies (ANA). Fever, photo-induced skin changes and arthritis are common. The ANA is usually positive--often with a positive anti-dsDNA.

Black patients, women, and especially younger black women are particularly at risk. Women outnumber men about 8:1. When SLE develops in a young child, one should exclude C1q deficiency.

Clinical

Bilateral erythema of the cheeks and malar eminences (butterfly rash) or a more extensive photodistributed rash may be seen in systemic lupus erythematosus. The following findings are characteristic: Raynaud'’s phenomenon, photosensitivity, mucous membrane lesions, butterfly poikiloderma, urticaria, alopecia (both scarring and non-scarring types), chronic discoid lesions, chilblains, and vasculitis.

Diagnosis

A positive ANA in the setting of photosensitivity is strongly suggestive of the diagnosis.

If there is any doubt, a 3 mm punch biopsy closed with 6-0 Prolene on any rash of the face is most direct and cost effective. Scarring is minimal. The biopsy should be studied for any interface changes.

Alternatively, the rheumatologic criteria is often used and is diagnostic if four of the following eleven criteria are positive. This system, however, has its flaws. For example, a malar rash is diagnostic of photosensitivity.

ANA-negative SLE occurs. These patients are often positive for ssDNA or Ro (SSA). In fact, anytime one draws an ANA for diagnosis, one should probably also draw Ro and La.

Trigger Factors

UVA from photocopiers appeared to induce the skin lesions in one photocopy technician with SLE [Arth Rheum 1995;38;1152]. Also note that UVA goes through glass, so work desks or places next to windows can be problematic.

Treatment

Treatment of systemic issues is beyond the scope of this work. Referral to a rheumatologist is appropriate.

With regard to the skin, photoprotection is of course mandatory. Daily use of an SPF 50 or higher sunscreen that blocks UVA is recommended. Sunscreens containing the metal titanium, zinc and iron are best as they block more of the UVA. It cannot be stressed enough that much of the skin changes are precipitated by sun exposure. The patient must be carefully questioned about the number of minutes (or hours) outside each day.

In a study of 1346 patients with SLE, current smokers were 63% more likely than past or never smokers to have an active SLE rash [SAD Aug 2013]. In addition, smoking makes the use of antimalarials less effective. So it is very important that smokers stop smoking.

An antimalarial can be very helpful in clearing the skin. Hydroxychloroquine 400 mg/day is very effective with side effects. If this fails after several months, Atabrine 100 mg/day may be added.

If prednisone is needed for control, 20-30 mg/day is usually adequate for most patients. Once control is achieved, the prednisone may be tapered slowly, e.g., dropping 2.5 mg/month.

See also cutaneous lupus erythematosus.

Additional Pictures

Hand involvement in a child with SLE.
Systemic lupus erythematosus Systemic lupus erythematosus

Systemic lupus erythematosus

Vasculitis of the ankle (leukocytoclastic vasculitis on biopsy) in a patient whose SLE was flaring. Exam showed necrotic, vesicular lesions.
Lupus Vasculitis

Vasculitis in RA/Lupus overlap.
Vasculitis in rheumatoid arthritis/Lupus overlap

Hair loss in systemic lupus erythematosus.
Alopecia in systemic lupus erythematosus

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