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Lupus erythematosus (LE) is the root designation for a diverse array of illnesses that are linked together by the development of autoimmunity directed predominantly at the molecular constituents of nucleosomes and ribonucleoproteins. Some patients present with life-threatening manifestations of systemic LE (SLE); whereas others, who are affected with what likely represents the same basic underlying disease process, express little more than discoid LE (DLE) skin lesions throughout their illness. It is convenient to conceptualize LE as a clinical spectrum ranging from mildly affected patients with only localized DLE skin lesions to those at risk of dying from the systemic manifestations of LE such as nephritis, central nervous system disease, or vasculitis. The pattern of skin involvement expressed by an individual patient with LE can provide insight about the position on the spectrum where the patient's illness might best be placed.
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The nomenclature and classification system originally devised by James N. Gilliam divides the cutaneous manifestations of LE into those lesions that show characteristic histologic changes of LE (LE-specific skin disease) and those that are not histopathologically distinct for LE and/or may be seen as a feature of another disease process (LE-nonspecific skin disease). Within this context, the term “LE-specific” relates to those lesions displaying an interface dermatitis. The term cutaneous LE (CLE) is often used synonymously with “LE-specific skin disease” as an umbrella designation for the three major categories of LE-specific skin disease: acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE), and chronic cutaneous LE (CCLE). This will be the framework used in our discussion of the extraordinarily diverse set of cutaneous lesions that occur in patients with LE (Table 155-1).
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