Lupus erythematosus: correlation of clinical and histological findings and proposal for a modified disease classification

Abstract

Background: Classification of lupus erythematosus (LE) is conflicting as it is carried out from different starting points. Whereas dermatological classifications categorize LE morphologically based on specific cutaneous lesions, rheumatologic classifications are based on symptomatic aspects. Indeed, LE is a systemic autoimmune disease with variable acuity and organ involvement. All cutaneous disease patterns may occur in both limited-cutaneous and systemic LE.Patients and Methods: 76 LE-patients with complete clinical data, clinical photographs and biopsy of cutaneous manifestations as well as paraclinical findings were retrospectively analyzed. Based on a published two-dimensional classification system that considers disease-specific skin manifestations and final disease diagnosis separately, patients' diagnoses were revised and compared with those in medical records. In addition, the extent to which patients could be clustered by diagnosis based on their LE-specific skin manifestations, corresponding histopathological changes, and paraclinical data was investigated.Results: After re-evaluation, the proportion of patients with limited-cutaneous LE decreased from 82% previously to 24%. More than two-thirds of patients indeed showed intermediate or systemic LE. Disease-specific skin manifestations, histologic characteristics and paraclinical data did not cluster with final diagnoses.Conclusions: First, the work underlines the systemic character of the disease. Second, a two-dimensional approach can help overcome classification difficulties in LE, as skin-morphologic and symptomatic aspects can be considered separately.

Microcirculation disorders of the skin

“…These autoantibodies form immune complexes that tend to be deposited in vascular branches and small vessels, leading to vasculitic and non-inflammatory vasculopathic changes and thrombotic microangiopathy. 42,43 Anti-endothelial cell antibodies are detectable in more than 80% of SLE patients and play an important role…”

“…SLE is characterized by polyclonal activation of B cells and the formation of autoreactive memory B cells that secrete a variety of autoantibodies. These autoantibodies form immune complexes that tend to be deposited in vascular branches and small vessels, leading to vasculitic and non‐inflammatory vasculopathic changes and thrombotic microangiopathy 42,43 …”

Mikrozirkulationsstörungen der Haut

“…Diese Autoantikörper bilden Immunkomplexe, die dazu neigen, sich an Gefäßverzweigungen und in kleinen Gefäßen abzulagern und vaskulitische und nichtentzündliche vaskulopathische Veränderungen sowie eine thrombotische Mikroangiopathie hervorzurufen. 42,43 Anti-Endothelzell-Antikörper sind bei mehr als 80% der SLE-Patienten vorhanden, sie sind maßgeblich an der mikrovaskulären Dysfunktion beteiligt.…”

“…Der SLE ist durch eine polyklonale Aktivierung von B‐Zellen und die Bildung von autoreaktiven Gedächtnis‐B‐Zellen gekennzeichnet, die eine Vielzahl von Autoantikörpern sezernieren. Diese Autoantikörper bilden Immunkomplexe, die dazu neigen, sich an Gefäßverzweigungen und in kleinen Gefäßen abzulagern und vaskulitische und nichtentzündliche vaskulopathische Veränderungen sowie eine thrombotische Mikroangiopathie hervorzurufen 42,43 …”

Tumid Lupus Erythematosus (TLE): A Review of a Rare Variant of Chronic Cutaneous Lupus Erythematosus (cCLE) with Emphasis on Differential Diagnosis

“…As part of the spectrum of LE, the nosology of TLE has been evolving. Whereas rheumatological classification for LE is based on symptoms and assessment of systemic involvement, from a dermatological perspective, classification has been established in order to categorize the disease mainly according to the morphology of skin lesions [22,39,40].…”