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Therapeutic Strategies

Lupus Erythematosus / Chronic Cutaneous Lupus Erythematosus

Timothy Berger Bruce Wintroub

Saturday, January 01, 2011

Overview

As the faces of patients with the classic facial eruption of systemic lupus erythematosus usually clear with treatment of their systemic disease, only the management of cutaneous lupus will be discussed. The four forms of cutaneous lupus treated by dermatologists are: Discoid Lupus, Subacute Cutaneous Lupus, Lupus Profundus, and Bullous Lupus.

Bullous Systemic Lupus Erythematosus

Although bullous systemic lupus erythematosus (bullous SLE) and epidermolysis bullosa acquisita are immunopathologically similar, their clinical behavior is different. The most common presentation of bullous SLE is that of a neutrophilic dermatosis with histology very similar to dermatitis herpetiformis. It presents usually in areas of sun exposure: on the head, neck, upper trunk, and proximal upper extremities.

First Steps

  1. Evaluate the patient for SLE and treat appropriately for any complications of this disorder. The appearance of bullae in SLE may or may not be associated with a flare of the underlying SLE.
  2. Dapsone 50-100 mg/day usually leads to a rapid resolution of the bullous lesions.
  3. Stress the importance of Maximum Sun Protection (see below).

Subsequent Steps

If the patient responds, continue the dapsone for a month or two, then gradually taper. If the patient fails to respond, consider the following steps:

  1. Increasing the dose of dapsone to 100 mg twice daily.
  2. Adding colchicines 0.6 mg once or twice daily to the dapsone.
  3. Thalidomide 50-100 mg nightly.
  4. Prednisone 40-60 mg once daily in a tapering course over 1 to 2 months.
  5. If the above are ineffective, consider an immunosuppressive such as azathioprine 50-100 mg daily, or mycophenolate mofeteil 2 g/day.

Pitfalls

  1. The most common side effects of dapsone therapy are hemolysis and a hypersensitivity syndrome.
  2. Epidermolysis bullosa acquisita and paraneoplastic pemphigus may occur in a patient with SLE and be confused with bullous SLE.
  3. Thalidomide is a potent teratogen and should not be used in women of childbearing potential.

Discoid Lupus Erythematosus

Discoid Lupus Erythematosus (DLE) is a chronic scarring form of LE that is usually restricted to the skin, but which results in permanent scarring. If the scalp is affected, the alopecia is permanent.

First Steps

  1. Apply superpotent topical steroids to the lesions twice daily.
  2. Maximum sun protection and sun avoidance is essential.
    These recommendations are for patients who have photosensitive skin diseases. Depending on the severity of the photosensitivity, more of the recommendations may need to be followed. The goal is for patients to continue their regular activities (exercise, occupation) while avoiding ultraviolet radiation that triggers their skin conditions. Patients should be counseled that maximizing photoprotection may reduce the amount of medication required to control their diseases. Phototesting to determine the wavelength of sensitivity may be valuable in determining how to apply the steps below:
    1. Avoid mid-day sun. All outdoor activities should occur as near to dawn and dusk as possible.
    2. If outdoors, activities should occur in shade.
    3. Wear protective clothing. Long-sleeve shirts, long pants, shirts with necks, and wide-brimmed hats are essential. Gloves are recommended, especially when patients are driving. Clothing should be impervious to light (can't see light through it) or be specially manufactured to block all UV radiation. Such clothing can be obtained from several manufacturers. Clothing may be washed in SunGuard, which contains Tinosorb. This will impart an SPF 30 UVB/UVA protection in the clothing for 20 washings.
    4. Use a sunblock: Combination sunscreens with agents that protect out into the UVA1 region are essential. Parasol 1789 and Mexoryl are two such chemical agents, and physical blockers zinc oxide and titanium dioxide also screen out these wavelengths. Ombrelle SPF 60 and Anthelios SPF 60 are two such sunscreens. The sunscreen must be applied every day, in adequate amounts, to be effective. Repeat application after sweating and every 2 hours when outdoors.
    5. Use UVA shielding: Normal car side windows and to a lesser degree windshields transmit UVA. Windshields are laminated and transmit only wavelengths beyond 370 nm, whereas nonlaminated side windows transmit more UV radiation starting at 310 nm. UVA shields for car, home and office windows, and filters on the computer screen, are recommended for the most sun-sensitive conditions. While patients are driving, they must keep their arms below the side window, and the windows must be kept closed.

Subsequent Steps

  1. If the patient responds, continue the treatment, emphasizing the need for year-round photoprotection.
  2. If the patient fails to respond and there are fewer than 10 lesions, consider intralesional triamcinolone acetonide 5-10 mg/mL to the active lesions.
  3. If there are more than 10 lesions, or there is active scalp/face disease, consider in this order:
    1. Hydroxychloroquine 200 mg twice daily.
    2. Chloroquine 250 mg daily.
    3. The combination of one of the above with quinacrine 100 mg daily.
    4. Isotretinoin 40-80 mg daily.
    5. Thalidomide 50-100 mg daily to start and increasing to as much as 400 mg if the response is inadequate.
    6. The addition of a systemic immunosuppressive agent such as azathioprine 50-100 mg daily, or mycophenolate mofeteil 2-3 g per day.

Pitfalls

  1. Five percent of patients with discoid skin lesions will have SLE. Do the appropriate laboratory evaluation (ANA, CBC, urinalysis) at the initial assessment, with change in systemic symptoms and yearly thereafter.
  2. Isotretinoin and thalidomide are teratogens, and exposure of them to pregnant women should be avoided. Potentially pregnant women must be enrolled in appropriate pregnancy prevention programs.
  3. Antimalarials may be associated with ocular toxicity. Regular (every 4-6 months) ophthalmologic evaluation is recommended.

Subacute Cutaneous Lupus Erythematosus (SCLE)

SCLE is a nonscarring form of cutaneous lupus that may be more frequently associated with systemic symptoms and laboratory abnormalities than DLE. Lesions are papulosquamous or annular. The face upper trunk and back are favored. SCLE is triggered by multiple medications, including terbinafine, hydrochlorthiazide, and calcium channel blockers. Stopping the triggering medication will lead to resolution, but only over several months.

First Steps

  1. Maximum sun protection and sun avoidance is essential.
    These recommendations are for patients who have photosensitive skin diseases. Depending on the severity of the photosensitivity, more of the recommendations may need to be followed. The goal is for patients to continue their regular activities (exercise, occupation) while avoiding ultraviolet radiation that triggers their skin conditions. Patients should be counseled that maximizing photoprotection may reduce the amount of medication required to control their diseases. Phototesting to determine the wavelength of sensitivity may be valuable in determining how to apply the steps below:

    1. Avoid mid-day sun. All outdoor activities should occur as near to dawn and dusk as possible.
    2. If outdoors, activities should occur in shade.
    3. Wear protective clothing. Long-sleeve shirts, long pants, shirts with necks, and wide-brimmed hats are essential. Gloves are recommended, especially when patients are driving. Clothing should be impervious to light (can't see light through it) or be specially manufactured to block all UV radiation. Such clothing can be obtained from several manufacturers. Clothing may be washed in SunGuard, which contains Tinosorb. This will impart an SPF 30 UVB/UVA protection in the clothing for 20 washings.
    4. Use a sunblock: Combination sunscreens with agents that protect out into the UVA1 region are essential. Parasol 1789 and Mexoryl are two such chemical agents, and physical blockers zinc oxide and titanium dioxide also screen out these wavelengths. Ombrelle SPF 60 and Anthelios SPF 60 are two such sunscreens. The sunscreen must be applied every day, in adequate amounts, to be effective. Repeat application after sweating and every 2 hours when outdoors.
    5. Use UVA shielding: Normal car side windows and, to a lesser degree, windshields transmit UVA. Windshields are laminated and transmit only wavelengths beyond 370 nm, whereas nonlaminated side windows transmit more UV radiation starting at 310 nm. UVA shields for car, home, and office windows, and filters on the computer screen, are recommended for the most sun-sensitive conditions. While patients are driving, they must keep their arms below the side window, and the windows must be kept closed.
  2. High-potency or superpotent topical steroids applied twice daily.

Subsequent Steps

  1. In patients failing to improve with topical treatments, consider in the following order:
    1. Hydroxychloroquine 200 mg twice daily.
    2. Chloroquine 250 mg daily.
    3. The combination of one of the above with quinacrine 100 mg daily.
    4. Isotretinoin 40-80 mg daily.
    5. Thalidomide 50-100 mg daily to start and increasing to as much as 400 mg if the response is inadequate.
    6. The addition of a systemic immunosuppressive agent such as azathioprine 50-100 mg daily, or mycophenolate mofeteil 2-3 g per day.

Pitfalls

  1. Isotretinoin and thalidomide are teratogens and exposure of them to pregnant women should be avoided. Potentially pregnant women must be enrolled in appropriate pregnancy prevention programs.
  2. Antimalarials may be associated with ocular toxicity. Regular (every 4-6 months) ophthalmological evaluation is recommended.

Lupus Profundus

Lupus profundus is an inflammatory disorder of the fat. Permanent loss of the fat, with significant disfigurement with facial lesions, may occur. The patient may meet the criteria for SLE.

First Steps

  1. Hydroxychloroquine 200 mg twice daily.
  2. Chloroquine 250 mg daily.
  3. The combination of one of the above with quinacrine 100 mg daily.
  4. Thalidomide 50-100 mg daily to start and increasing to as much as 400 mg if patient is not responding.
  5. The addition of a systemic immunosuppressive agent such as azathioprine 50-100 mg daily, or mycophenolate mofeteil 2-3 g per day.

Pitfalls

  1. Intralesional triamcinolone acetonide may be associated with exacerbation of the lesion and should be used with caution.
  2. Surgical correction, even when inactive, may be associated with exacerbation of lupus profundus and should be undertaken with caution.
  3. Thalidomide is a potent teratogen and exposure of it to pregnant women should be avoided. Potentially pregnant women must be enrolled in appropriate pregnancy prevention programs.
  4. Antimalarials may be associated with ocular toxicity. Regular (every 4-6 months) ophthalmological evaluation is recommended.

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