CUTANEOUS LUPUS ERYTHEMATOSUS

By Gary M. White, MD

Cutaneous lupus erythematosus.  Tumid lupus in a child


Cutaneous lupus erythematosus (CLE), also commonly called discoid lupus erythematosus is a variant of lupus in which skin lesions are found in a patient who does not satisfy the criteria for SLE.

Clinical           

Erythematous, hypopigmented, atrophic, hyperkeratotic lesions scattered on the face are characteristic. The scale may fill the follicular orifices and if confluent, may be peeled back and the undersurface found to resemble the undersurface of a carpet (so-called carpet-tack sign). Sun exposure is a precipitating factor. The border may be hyperpigmented. Scarring can occur. Rarely, SCC may develop in chronic CLE lesions Indian J of Derm.

Workup

A typical workup includes skin biopsy for H&E, direct immunoflourescence, plus blood work for LFTs, creatinine, urine analysis, ANA, Ro, La and dsDNA.

Those who present with CLE lesions less than 15 years of age should be checked for deficiencies of complement especially C4 and C1q.

Treatment

Sun Protection

A broad spectrum sunscreen and sun avoidance are crucial. The SPF should be at least 50 and it should be both a UVB and UVA blocker. Zinc and titanium-containing sunscreens are particularly effective. UVA can go thru glass so a desk by the window at work can cause flares. UVA from a photocopier was reported to flare one patient. Sun protective clothing is important as well.

Topical Therapy

A potent topical steroid may be tried initially. Topical tacrolimus mixed with clobetasol applied BID was very helpful in two cases [BJD 2002;147;385]. Topical tacrolimus lotion, 0.3% in an alcohol base has been advocated [JAMA Derm 2015;115;1113].

Pimecrolimus 1% cream BID (the night-time dose was occluded) for 3 weeks has been reported effective.   Intralesional steroids, e.g. triamcinolone 5 mg/cc, are effective but risk atrophy. For tumid LE, circumscribed on a child, one may try kenalog 5 mg/cc after topical anesthetic (e.g. EMLA).

Antimalarials

If topical or IL therapy fails, hydroxychloroquine (HCQ), or another antimalarial should be tried. The usual dose for HCQ is 200-400 mg/day. If this fails, atabrine (quinacrine) 100 mg/day may be added. Chloroquine is also efficacious and may be tried. This approach is effective for the majority of cases. Smoking cuts in half the response to antimalarials [JAAD 2015;72;634]. For patients seemingly resistant to HCQ, the blood levels should be checked. Increasing the dose to get blood levels above 750 ng/ml significantly improved results in 81% of patients analyzed [JAAD 2016;74;693].

Resistant Cases

Phenytoin (100 mg TID) gave excellent results in 88% of patients in one study. Another review concluded that thalidomide (e.g. 100 mg/day with excellent response after 3 months) was most effective for resistant disease, followed by methotrexate (e.g. given as for psoriasis), dapsone and belimumab [JAMA Derm 2017;153;937]. Isotretinoin and azathioprine have been used. Any hyperkeratotic nodules or growths should be evaluated for biopsy as SCC's have developed in chronic CLE lesions.

What Percent of Patients go on to Develop Systemic Lupus Erythematosus?

One prospective study of 77 patients found that 17% of patients eventually met the criteria for SLE [JAMADerm March 2014;150;291]. The mean duration between CLE and SLE was 8 years. Risk factors for progression included positive ANA, widespread discoid lesions, female sex and increased numbers of SLE criteria initially.

It is thus recommended that patients get a CBC, ANA if applicable, urinalysis and ROS pertinent to the lupus criteria annually.

Additional Pictures

Cutaneous lupus erythematosus Cutaneous lupus erythematosus
Scarring alopecia of the scalp. Pigmentary change is common. (first photo courtesy of Michael O. Murphy, MD)

Cutaneous lupus erythematosus. hair loss in a child Cutaneous lupus of the scalp
Cutaneous lupus causing hair loss. In contrast to alopecia areata, the lesions are not round to oval and the skin has inflammation and/or pigmentation.

Cutaneous lupus erythematosus. Lupus erythematosus tumidus Cutaneous lupus erythematosus. Lupus erythematosus tumidus
Lupus erythematosus tumidus.

Discoid lupus erythematosis.  Cutaneous lupus Cutaneous lupus erythematosus

Hypertrophic Variant of cutaneous lupus, here on the arms.
Hypertrophic Variant of cutaneous lupus, here on the arms.

Cutaneoius involvement of the hands in a patient with systemic lupus erythematosis.
Cutaneous lupus erythematosus.

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