Research Highlights

Abstract

Paller, A. S. (2015). The natural history of pediatric-onset discoid lupus erythematosus. Journal of the American Academy of Dermatology, 72(4), 628Y633.S ystemic lupus erythematosus (SLE) is a chronic inflammatory disease that can affect multiple organ systems including the skin, joints, kidneys, lungs, and nervous system. Approximately 20% of SLE cases present during the first 2 decades of life. Although SLE in children is fundamentally the same disease as in adults, there are several important differences between adult and pediatric SLE. Specifically, pediatric SLE tends to be more severe than adult SLE, with a more aggressive clinical course and increased mortality. In addition, there is a higher frequency of endorgan involvement at onset in children with lupus than in adults, with renal, neurologic, cardiac, and pulmonary involvement seen more commonly at the time of diagnosis. The prognosis of pediatric SLE is tied closely to disease severity at presentation; delays in diagnosis often lead to increased morbidity and mortality.There are several specific subtypes of cutaneous lupus erythematosus (CLE), including acute CLE, characterized by the localized malar butterfly rash or generalized photosensitive rash; subacute CLE, characterized by an annular rash or papulosquamous (psoriasiform) rash; and chronic CLE, characterized by a wide variety of skin lesions. Discoid lupus erythematosus (DLE) is the most common type of chronic CLE and presents clinically as a sharply demarcated, round, erythematous plaque with a well-formed adherent ''carpet-tack'' scale; when the scale is removed, follicular plugging is revealed. DLE lesions resolve with scarring.In the adult population, the frequency of progression of DLE to SLE is reported to range from 0% to 28%, with an interval between onset of DLE and progression to SLE ranging from months to decades. The risk of progression to SLE in children with DLE, however, remains poorly understood.The objectives of this study were to better understand the natural history of pediatric DLE and to determine the risk of progression of pediatric DLE to SLE. The study design was a retrospective review. The charts of all patients given the diagnosis of DLE before the age of 16 years and seen between 1995 and 2012 by faculty in rheumatology and/or dermatology at the Ann and Robert H. Lurie Children's Hospital in Chicago, IL, were reviewed. At least two health encounters were required. Patients with other manifestations of CLE including acute CLE, subacute CLE, neonatal lupus, tumid lupus, and lupus panniculitis were excluded. DLE lesions were diagnosed based on clinical features or histopathologic confirmation. A patient was considered to have progressed to SLE if four or more of the 1982 SLE American College of Rheumatology criteria were met. Demographic information and clinical data relating to disease characteristics were collected for all patients. Patients were subsequently stratified into four subgroups: (a) DLE with concurrent diagnosis of SLE, (b) DLE with progression to SLE, (c...