Is Chronic Cutaneous Discoid Lupus Protective Against Severe Renal Disease in Patients With Systemic Lupus Erythematosus?
December 2011 | Volume 10 | Issue 12 | Original Article | 1413 | Copyright © 2011
Joseph F. Merola MD,a Caroline A. Chang MD,b Miguel R. Sanchez MD,c Stephen D. Prystowsky MDc
aHarvard Medical School, Brigham and Women's Hospital, Boston, MA bTufts Medical Center, Boston, MA cNew York University School of Medicine, New York, NY
Objective: The aim was to assess the level of systemic involvement and character of renal disease in patients with chronic cutaneous lupus erythematosus of the discoid lupus variety (hereafter referred to as 'discoid lupus') and features of systemic lupus erythematosus (SLE). Clinical confusion with other types of cutaneous lupus erythematosus complicates interpretation of some previously reported studies.
Methods: Over three years, sixteen patients met the diagnostic criteria of discoid lupus, positive anti-nuclear-antibody, and at least one extracutaneous manifestation.
Results: Most patients (14/16) were female, between 26 to 66 years old. Arthritis was the most common extracutaneous manifestation followed by Raynaud's phenomenon. The anti-nuclear-antibody was speckled in ten patients with titers ranging from 1:40 to 1:1280 IU/mL. Elevated levels of double-stranded-DNA in low titers were found in four patients, anti-Smith-antibody in four; anti-Sjogren-syndrome-A-antibody in seven, and anti-ribonucleoprotein-antibody in seven. Renal function markers were transiently high in some patients but normalized over time. Hematuria and/or proteinuria were present at some time in seven patients. The highest BUN and creatinine levels were 42 mg/dL and 1.5 mg/dL, respectively. One patient had membranous glomerulonephropathy class 5; however, discoid lupus developed well after the onset of renal disease during a time when renal function had returned to normal.
Conclusion: Our observational data supports previous reports suggesting that patients with active discoid lupus rarely have progressive renal insufficiency. The mechanism for the development of discoid lupus may involve an immunologic mechanism that differs from that which produces severe organ involvement, especially advanced immune-complex-mediated renal disease. Patients with discoid lupus rarely have sustained high levels of antibodies to double-stranded-DNA. Discoid lupus appears to be a marker for a more benign lupus course. This clinical observation lays the groundwork for a larger prospective, longitudinal cohort study for further validation.
J Drugs Dermatol. 2011;10(12):1413-1420.
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In 1975 Gilliam et al. divided the manifestations of cutaneous lupus into acute, subacute, and chronic categories based on clinical-histopathologic lesional patterns. The most common type of chronic lupus erythematosus is the discoid subtype. Hypertrophic/verrucous, mucosal variants, lupus panniculitis, chilblains lupus, and lupus tumidus are relatively rare forms of chronic cutaneous lupus. Skin lesions that may be lupus-specific or lupus-nonspecific develop in up to 81 percent of cases with systemic lupus erythematosus (SLE) at some point in the course of the disease.1 In select study populations1-16 discoid lesions have been found in 8 to 28 percent of patients with SLE.4,5,16 The presence of cutaneous lupus lesions generates significant morbidity from disfigurement, discomfort, and occupational disability to the degree that approximately 45 percent of patients with cutaneous lupus experience some degree of vocational handicap.17,18
The lesions of discoid lupus may be "localized" to specific body areas, especially the head and neck, or occur in a more "generalized" distribution. As reported thirty years ago by Gilliam and one of the authors of this paper (SDP), patients with discoid lesions have less frequent and less severe systemic organ involvement. 19 Patients with localized discoid lesions have a risk of about 6 percent of going on to develop systemic lupus, patients with generalized discoid lesions have a 15-28 percent chance of going on to systemic lupus.20-22 Only 20 percent of discoid lupus erythematosus patients develop detectable serum autoimmune antibodies, such as anti-nuclear antibody (ANA), double-stranded DNA (dsDNA), ribonucleoprotein (RNP), and anti-Sjogren syndrome A antibody and B antibody (SSA/SSB), and then usually in low titers.2,3,20,23 It is further thought that if patients have discoid lesions as well as positive serologic markers and/or