Presented by: Brett Miller, MD and Carrie Ann Cusack, MD
Drexel University College of Medicine
May 16, 2016
Lupus erythematosus (LE) is an autoimmune disorder that is associated with a wide range of cutaneous and systemic manifestations. It is more common in women. The diagnosis of systemic lupus erythematosus (SLE) is based on meeting 4 of 11 criteria established by the American College of Rheumatology, which include malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder, neurologic disorder, hematologic disorder, immunologic disorder, and positive antinuclear antibody. The criteria may be present simultaneously or serially.
Cutaneous disease is often the initial presentation of LE. According to a recent study, 17% of patients with cutaneous lupus erythematosus (CLE) met criteria for SLE with a mean time of 8.03 years from a diagnosis of CLE to SLE. The researchers also found that those who develop SLE generally have mild systemic symptoms, and the presence of moderate to severe symptoms could be predictive of future SLE. Although acute cutaneous lupus has the strongest association with systemic disease, patients with any type of CLE may develop systemic involvement.
In discoid lupus erythematosus (DLE), the most common form of chronic cutaneous lupus, about 5-10% of adult patients develop SLE. In DLE, characteristic lesions are dull red macules or indurated plaques with adherent scale that evolve into atrophic, scarred lesions with pigmentary alteration, more pronounced in darker-skinned patients. Lesions are most commonly found on the head and neck, particularly the face, scalp and ears. Mucosal surfaces may also be affected. Less commonly, lesions may be present in a generalized manner, which has a greater risk of SLE development. Additionally, lesions can lead to disfiguring scarring; rarely, squamous cell carcinoma may develop in longstanding lesions.
While DLE is very similar clinically, there are several distinct differences in the pediatric population. Isolated DLE is less common in the pediatric population making up only 5% of pediatric lupus and these patients often have a stronger family history. The female predominance is less prominent in pediatrics along with less sun sensitivity. It is important to monitor these patients as 25% to 30% of the children with DLE progress to SLE as opposed to only 5% to 10% of adults.
Histopathologically, DLE is characterized by hyperkeratosis, follicular plugging, vacuolar degeneration of basal keratinocytes and a periadnexal/periappendageal infiltrate. On DIF, a positive lupus band may be demonstrated in the majority of patients. On serology, patients have decreased rates of ANA, dsDNA, Sm, Ro/SSA and U1RNP antibodies as compared to other CLE variants. A recent study measured levels of IgG autoantibodies in DLE patients and found that anti-RNP IgG correlated strongly with disease activity as measured by the Cutaneous Lupus Disease Area and Severity Index activity score.
For treatment, sun protection, sun avoidance and discontinuation of smoking are paramount. Topical steroids and/or calcineurin inhibitors should be used as first-line treatment with or without anti-malarials. Alternative therapies for patients with recalcitrant disease include systemic steroids, methotrexate, dapsone, mycophenolate mofetil and rituximab.
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