Understanding Coexisting Conditions
and
Diagnosis of Those with Lupus Erymatosus
Patients with lupus erymatosus (LE) often have concurrent chronic urticaria, but the pathogenesis behind this is not fully understood (Costner and Sontheimer, 2008).
LE is also linked with cutaneous mucinosis, lichen planus, acanthosis nigricans, acquired ichthyosis, cutis laxa, and interstitial granulomatous dermatitis.
The clinical scoring tool called The Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) has been developed to assist physicians in monitoring patients with cutaneous LE (CLE) and their response to treatment (Jolly et al., 2013).
CLASI score is calculated based on the scoring of erythema, scale, and the presence of mucous membrane lesions or nonscarring alopecia, ranging from 0 to 70.
The affected areas of the skin that are most often visible, such as the face, are given higher scores with the CLASI.
The CLASI has been validated against the Safety of Estrogens in Lupus Erythematosus National Assessment-Systemic Lupus Erythematosus Disease Activity Index, Systemic Lupus International Collaboration Clinics-American College of Rheumatology Damage Index, and LupusPRO scoring systems. Jolly et al. (2013) demonstrated a strong correlation between the CLASI and these existing LE indices.
The CLASI is useful in clinical practice as an objective measure of clinical response and is used in clinical trials.
The CLASI has been validated for all subtypes of CLE, except for bullous lupus and lupus panniculitis, both of which are rare (Bonilla-Martinez et al., 2008; Jolly et al., 2013).
At this point, we need to move towards the diagnosis of CLE.
The diagnosis of CLE is made using a combination of clinical assessments, serologic testing, and histopathologic findings.
Histologic findings in each subtype of specific CLE lesions can be explored but histology alone cannot differentiate among the subtypes.
The American College of Rheumatology classification system includes the universally accepted criteria for the diagnosis of LE (Hochberg, 1997).
The system consists of 11 clinical and laboratory criteria and assesses all potential manifestations of LE.
However, this is a general score for all manifestations of LE.
It is not always useful in assessing patients with CLE without systemic involvement.
Researchers have since proposed alternative classification systems for the diagnosis of CLE.
Gilliam and Sontheimer initially proposed the classification of LE lesions into specific lesions, which are further categorized into CLE subtypes (and its variants), and bullous LE, and nonspecific lesions, such as urticaria or vasculitis (Kuhn et al., 2005; Sontheimer et al., 1979).
Further, classification systems have been described that expand on the work of Gilliam and Sontheimers. The most recent suggested classification system is the Dusseldorf classification created by Kuhn, which made small changes to already classified subsets (Kuhn and Landmann, 2014).
Autoantibody testing is useful in both the diagnosis and monitoring of CLE. Antinuclear antibodies (ANA) is typically positive in systemic LE (SLE) at moderate-to-high titers and positive in 60% to 80% of patients with CLE (Costner and Sontheimer, 2008).