Chronic Cutaneous Lupus Erythematosus
Saturday, January 01, 2011
Chronic cutaneous lupus erythematosus (LE) has several
morphologic patterns. Subacute cutaneous LE (SCLE) presents with
erythematous, annular, or papulosquamous lesions, which tend to
heal without scarring. Discoid lupus erythematosus (DLE) causes
lesions with significant hyper- and hypopigmentation and follicular
plugging, and causes significant scarring. DLE may cause
significant cicatricial alopecia and permanent hair loss. Both SCLE
and DLE are photosensitive dermatoses. Patients with SCLE, more
commonly than patients with DLE, will fulfill the criteria for the
diagnosis of systemic lupus erythematosus (SLE). Due to the low
prevalence of renal and central nervous system disease in chronic
cutaneous LE, however, the prognosis in these patients is good. In
a significant portion of patients, especially those with DLE, the
skin disease will be the primary complication of their lupus
erythematosus. The therapeutic strategy is to control exposure to
sunlight and to suppress inflammation in an effort to prevent or
- In all patients, regardless of extent of disease, maximum
photoprotection must be instituted.
- Local topical steroids: Prescribe a superpotent steroid cream
to be applied twice daily to areas of active disease. To minimize
the potential atrophogenic side effects of topical steroids
administered to facial lesions, discontinue this therapy or change
to a nonatrophogenic, low-potency preparation as soon as a response
occurs, or after a maximum of 2 weeks.
- Intralesional steroids: Intralesional triamcinolone acetonide
diluted 1:1 with lidocaine 1.0% (final concentration of 2.5-10 mg
per mL) can be administered to active or advancing lesions. Do not
perform this therapy more often than once every 3-4 weeks to
minimize atrophy, and no more than 20 mg of triamcinolone should be
injected at one time.
If systemic treatment is required, the first and most beneficial
alternative is antimalarials. Begin treatment with
hydroxychloroquine 200 mg per day for at least 4 weeks and increase
to 400 mg per day if response is slow or nonexistent. In children
the dose is 6.5 mg per kg per day.
Alternative steps and work-up
- If the patient does not respond to hydroxychloroquine,
chloroquine 250 mg per day may be used as an alternative.
Chloroquine is more effective but also has a higher risk of ocular
- Quinacrine 100 mg per day may be added to chloroquine or
hydroxychloroquine regimens in patients failing a single
- Systemic steroids sometimes are effective for rapid control of
severe disease or for systemic complications. An alternative
therapy for long-term treatment should be instituted simultaneously
(such as an antimalarial) so that the steroid course does not
exceed 4-6 weeks. Systemic steroids are safe to use during
- If the response to antimalarials is unsatisfactory,
isotretinoin at 0.5-2 mg per kg daily can be tried. Pregnancy
prevention measures are required.
- Thalidomide 50-200 mg as a single nightly dose may be effective
in refractory cases. Pregnancy prevention measures are required,
and, in general, thalidomide should be avoided in women of
- Dapsone 50-200 mg daily can be used alone or as an adjunct to
the above treatments.
- Camouflage techniques are particularly helpful in patients who
have developed dyspigmentation from DLE. Several lines of cosmetics
are designed to mask pigmentary abnormalities.
- All patients with chronic cutaneous LE should have laboratory
parameters checked at baseline and at yearly intervals to screen
for the development of SLE.
- Topical and intralesional steroids are potentially atrophogenic
and can themselves produce pigmentary abnormalities. Prolonged or
excessive usage can worsen the disfigurement in DLE.
- Antimalarials can produce retinal toxicity, and patients on
this form of therapy should be assessed by an ophthalmologist
before treatment is instituted and at 6-month intervals.
- Dapsone, and possibly antimalarials, can cause acute hemolysis
in G6PD-deficient patients; hence, a screen for this enzyme must be
obtained prior to initiation of therapy.
- Biologically aggressive squamous cell carcinomas can occur in
old scars of DLE (as in any other cutaneous scarring process).
Thus, even patients with quiescent disease need to be examined
regularly for the presence of neoplastic lesions.
- Quinacrine can cause a lemon-yellow discoloration of the
- Patients on isotretinoin and thalidomide should be seen monthly
and evaluated for specific complications of these medications,
especially psychiatric complications with isotretinoin and
neuropathy with thalidomide.