Classification of Lupus erythematosus Based upon Cutaneous Manifestations

Abstract

Background: Lupus erythematosus (LE) is a multi-organ-system disease, the characteristics of which are reflected in the 1982 American Rheumatism Association (ARA) criteria for systemic lupus erythematosus (SLE). From a dermatological point of view, only the presence of LE-specific histopathology is necessary and sufficient for the diagnosis of LE. The association between the type of LE-specific skin lesion and the severity of extracutaneous manifestations of LE has not yet been investigated systematically. Objective: The aim of this study was to evaluate, according to the type of LE skin lesions, the prevalence of the 1982 criteria for SLE. Methods: We selected 191 patients whose skin lesions were histologically diagnosed as LE specific. Patients were classified on the basis of skin disease, and their clinical and laboratory data were analyzed. Results: Of 191 patients, 130 (68%) exhibited only one type of LE-specific skin lesion (monomorphic), 55 (29%) had two types (bimorphic) and the remaining 6 (3%) displayed three types (trimorphic). Nineteen of 22 (86%) patients who presented discoid lupus skin lesions above the neck without other eruptions were classified in the cutaneous-limited LE spectrum. Of 116 patients with acute lupus skin lesions (malar rash), 83 (72%) clearly fulfilled the 1982 ARA criteria for SLE. In skin lesions of LE profundus, chilblain lupus, subacute lupus (annular-polycyclic erythema and the papulosquamous variant), there were no significant correlations between the type of eruption and the severity of extracutaneous manifestations. Conclusion: Patients with acute lupus skin lesions could usually be classified as suffering from SLE, whereas monomorphic patients with localized discoid lesions rarely exhibited extracutaneous manifestations. This tendency was less distinct in bimorphic patients. Almost all patients with subacute skin lesions were bimorphic or trimorphic, which might be due to genetic or racial differences between Japanese and other populations.

Periorbital Oedema and Erythema as a Manifestation of Discoid Lupus erythematosus

“…The histology and the direct immunofluorescence are specific. LE profundus (panniculitis) is an unusual clinical variety of LE in which the cutaneous infiltrate occurs primarily in the deeper portions of the hypodermis, with only microscopic epidermal changes, giving rise to firm, sharply defined nodules, lying beneath clinically normal skin [1, 2]. The histopathology is sufficiently characteristic to make the diagnosis in the absence of other cutaneous lesions of LE.…”

Exacerbation of Lupus Panniculitis following Anti-Hepatitis-B Vaccination

“…Lupus panniculitis lesions predominantly involve the proximal extremities, the trunk and the head, and may lead to lipoatrophic scars [3, 4]. …”

Disseminated Discoid Lupus Erythematosus in a Dog

“…It is also believed that some human patients with DLE occasionally have the higher ANA levels that are characteristically found in patients with overt SLE 25) . These phenomena have been discussed in depth in human, and several novel concepts and classifications have been proposed to date 8,9,12,24,31) . In these reports, Tsuchida et al analyzed the association between the type of LE-specific skin lesion and the severity of extracutaneous manifestations of LE according to a two-dimensional coordinate axis system, and proposed a unique classification (Table 1) 29,31) .…”

“…These phenomena have been discussed in depth in human, and several novel concepts and classifications have been proposed to date 8,9,12,24,31) . In these reports, Tsuchida et al analyzed the association between the type of LE-specific skin lesion and the severity of extracutaneous manifestations of LE according to a two-dimensional coordinate axis system, and proposed a unique classification (Table 1) 29,31) . Patients with acute lupus skin lesions could usually be classified as suffering from SLE, whereas monomorphic patients with localized discoid lesions rarely exhibited extracutaneous manifestations in the classification 31) .…”

“…In these reports, Tsuchida et al analyzed the association between the type of LE-specific skin lesion and the severity of extracutaneous manifestations of LE according to a two-dimensional coordinate axis system, and proposed a unique classification (Table 1) 29,31) . Patients with acute lupus skin lesions could usually be classified as suffering from SLE, whereas monomorphic patients with localized discoid lesions rarely exhibited extracutaneous manifestations in the classification 31) . Further assessments using these criteria showed that LE could be classified into three groups, CLE (CLE and ILE I), a mild type (ILE II and SLE I) and a serious type (SLE II) of SLE, according to the responsiveness to treatment and the clinical course 9,12) .…”