Clinical and Histologic Classifications of Lupus Erythematosus

On establishing the unifying concept that LE comprised a common disease with heterogenous expressions, attempts were undertaken to develop a clinical classification of the different forms of LE.

In 1921, Brocq (Brocq 1921) subdivided LE into three different forms: DLE, disseminated DLE, and SLE. In 1934, O'Leary (O'Leary 1934) further developed the classification, trying to connect the cutaneous manifestations to certain systemic involvements. He stressed that disseminated DLE represented the transition from a localized cutaneous disease to a multisystem systemic illness, namely, SLE. At that time, no serologic markers had been identified, which later helped better characterize the different subsets in the LE spectrum. The concept of a disease spectrum was, finally, emphasized by Dubois and Tuffanelli (Dubois and Tuffanelli 1964). They extensively described 520 consecutive patients with SLE, with precise characterization of the various cutaneous and systemic disease expressions, therefore demonstrating the disease continuum from the relatively benign forms of limited skin disease such as localized DLE to the potentially fatal fully expressed SLE. Between these two poles a spectrum of characteristic disease manifestations was recognized in which clinical, histopathologic, serologic, immunologic, and photobiologic features composed the fundamentals for the specific diagnosis of an LE subset with a well-established prognostic value.

The modern and generally accepted classification of LE is based mostly on the work of Gilliam, Sontheimer, and their coworkers (Gilliam and Sontheimer 1981a, b, 1982,1983). Based on the spectrum concept of Dubois, they concentrated on the relationships that exist between the various cutaneous and systemic manifestations of LE. Clinical, histopathologic, photobiologic, immunologic, and genetic studies led

Gilliam and Sontheimer to the extensive in-depth description of the immunogeneti-cally homogeneous LE subset, namely, subacute cutaneous LE (SCLE). This work led to the modern classification of LE and has been widely agreed on. This progress had been made possible by the rapid developments in biochemistry, immunology, and molecular biology that were integrated into clinical science and helped investigate the various phenomenologic expressions of LE. Examples include the discovery of the LE cell factor by Hargraves et al. (Hargraves et al. 1948) in 1948 and the development of the antinuclear antibody assay in 1957 by Friou (Friou 1957). These laboratory investigations prompted the era of intense research on clinical-serologic correlations.

The discovery and characterization of numerous antibodies led to a better understanding of the various expressions of LE sometimes assuming a marker function for the disease.

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