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Lupus

Treatment

First-line treatment: Photoprotection is critical for all patients with cutaneous lupus erythematosus (CLE). Inflammation should be targeted via topical, intralesional, or systemic immunosuppression, or a combination therapeutic approach. Treatment requires monitoring and adjustment according to the activity of cutaneous disease.

Patients with cutaneous lupus lesions should undergo diagnostic evaluation for possible systemic lupus erythematosus. All patients with CLE should have laboratory parameters checked for the development of SLE at routine intervals.

Initial therapy

  • Topical corticosteroids: Prescribe a potent topical corticosteroid such as desonide 0.05% or triamcinolone 0.1% ointment b.i.d. (face) or superpotent such as fluocinonide 0.05% or clobetasol 0.05% ointment b.i.d. (body), to be applied b.i.d. to areas of active disease.
  • Intralesional corticosteroids: Intralesional triamcinolone acetonide (such as Kenalog, 10 mg/cc) diluted 1:1 or 1:2 with lidocaine 1% (final steroid concentration 3.33-5 mg/cc) can be administered to active lesions or deeper (i.e., dermal or subcutaneous) lesions. Do not perform this therapy more often than once every 3-4 weeks to minimize atrophy.
  • All patients with CLE should practice strict photoprotection and sun avoidance, as even minimal sun exposure can result in further spread or reactivation of quiescent disease. Photoprotection should include physical barriers (hats, protective clothing, gloves), as well as broad spectrum UVA/UVB-blocking sunscreens for exposed areas of skin. Sunscreens should contain UVA-blocking agents such as mineral sunscreens (zinc oxide), mexoryl (SX or XL), and/or parsol 1789. As a result of photoprotection and sun avoidance, patients may become deficient in vitamin D levels; vitamin D should be supplemented in the diet and levels monitored.

Alternative therapy

  • Antimalarials (such as hydroxychloroquine 4-6.5 mg/kg/day and chloroquine 250 mg daily) are an effective therapeutic option. Baseline and screening ophthalmologic exams should be performed, as this therapy can be associated with some irreversible ocular toxicities. These antimalarials may be used in combination with quinacrine (50-100 mg daily), a third antimalarial not associated with ocular toxicity.
  • Systemic corticosteroids are sometimes effective for patients in whom antimalarials are contraindicated. A typical regimen consists of prednisone 0.5 mg/kg daily for 2-3 weeks, followed by tapering if a response occurs. A more prolonged 4-6 week course with tapering may be used if the response is slow.
  • Alternative treatments for refractory cases of CLE that do not respond to antimalarials include azathioprine, mycophenolate mofetil, or thalidomide. Systemic retinoids and methotrexate may also lead to resolution of symptoms and is in the therapeutic ladder for CLE.
  • Dapsone 50-200 mg/day usually leads to a rapid resolution of the bullous lesions.

Ancillary therapy

Camouflage techniques are particularly helpful in dark-skinned patients with DLE. Several lines of cosmetics are designed to mask pigmentary abnormalities such as those in DLE (e.g., Dermablend, Covermark). These cosmetics often are available only in cosmetic departments of large department stores.

Pitfalls

  • Topical and intralesional steroids may cause epidermal or dermal atrophy and can themselves produce pigmentary abnormalities. Prolonged or excessive usage can worsen the disfigurement in CLE. Intralesional steroids may be associated with exacerbation of lupus profundus lesions and should be used with caution.
  • Antimalarials may be associated with both reversible and irreversible ocular toxicity. Baseline and monitoring ophthalmological evaluation (every 6-12 months) is recommended.
  • Antimalarials can cause acute hemolysis in G6PD-deficient patients; hence, a screen for this enzyme must be obtained prior to initiation of therapy.
  • Quinacrine (Atabrine) can cause a yellow discoloration of the skin.
  • The most common side effects of dapsone therapy are hemolysis and a hypersensitivity syndrome.
  • Cosmetic surgical correction of lesions of lupus profundus, even when inactive, may be associated with exacerbation of disease and should be undertaken with caution.

When to refer to a dermatologist

  • If the diagnosis of lupus is not clear
  • If the form of cutaneous lupus cannot be distinguished
  • For patients who fail topical steroids, especially for management of systemic immunosuppression or antimalarial medication dosing and monitoring