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Classical variants of specific cutaneous LE lesions are chronic discoid LE (CDLE) and subacute cutaneous LE (SCLE). CDLE and SCLE may appear at any age; however, the most common age of onset is between 20 and 40 years, with a female predominance of 3:1 in CDLE and 3-6:1 in SCLE. Nonspecific LE skin lesions such as generalized or acrolocalized vasculitis (4-30%), livedo reticularis (22-35%), and alopecia (38-78%) are frequently seen in patients with cutaneous LE. Other typical cutaneous LE subsets such as LE profundus/panniculitis, LE tumidus, urticaria vasculitis, hypertrophic LE, and bullous LE are rather rare variants. Butterfly rash and/or macular exanthema are characteristic skin lesions of systemic lupus erythematosus (SLE) rarely found in patients with cutaneous LE.

Fibromyalgia has been reported to occur with high prevalence in systemic lupus erythematosus. Data on fibromyalgia in other subsets of lupus erythematosus are not available. Risk factors for fibromyalgia have not been defined. We investigated 60 patients with different subsets of lupus erythematosus for the presence of fibromyalgia, association with clinical and laboratory parameters and disease activity. Our data were compared with the multicentre lupus erythematosus registry at the Free University of Berlin. Ten out of 60 patients with more than 11 tender points and widespread pain for more than 3 months were classified as positive for fibromyalgia. All of them were female. Fibromyalgia-positive patients suffered significantly more often from headache, morning stiffness, diffuse alopecia, muscle pain, arthralgia, renal involvement, and disclosed peripheral blood cell cytopenia, rheumatoid factor, hypergammaglobulinaemia and intake of corticosteroids and azathioprine. Fibromyalgia was more frequent in systemic lupus than in other lupus subsets. Evaluation of fibromyalgia symptoms and lupus disease activity was performed in 30 patients in a 1-year (range 9-13 months) follow-up. These 30 patients consisted of 9 fibromyalgia-positive and 21 fibromyalgia-negative patients. Both groups were characterized by stable clinical features such as number of tender points and ECLAM index. Fibromyalgia did not show a correlation with lupus activity. We suggest that fibromyalgia and lupus erythematosus are distinct complaints. Patients with lupus are at risk of developing secondary fibromyalgia. The clinical features of fibromyalgia-positive patients may contribute to misinterpretation of lupus activity.

Lupus erythematosus (LE) is an autoimmune disorder, involving the skin and/or other internal organs. As cutaneous variants, chronic discoid LE (CDLE) and subacute cutaneous LE (SCLE) usually have a better prognosis, however, involvement of internal organs with transition into systemic disease may occur. The aim of this study was to assess the significance of some clinical and laboratory criteria that could serve as markers for early recognition of systemic involvement in cutaneous LE. Three hundred and seventy-nine patients with LE, seen in five cooperating Departments of Dermatology during the years 1989-1994, were documented by electronic data processing according to a common protocol. Two hundred and forty-five of these patients had cutaneous LE (CDLE or SCLE), and 51 had systemic LE (SLE) and were included in this study. Forty-nine patients with either CDLE/SCLE or SLE were not evaluated because of incomplete documentation; also, 34 patients suffered from other LE subsets and were likewise excluded from the evaluation. Multivariate statistical analysis was used to assess the value of seven selected variables for distinguishing between the CDLE/SCLE and SLE groups: ESR, titers of antinuclear antibodies, anti-dsDNA-antibodies, photosensitivity, presence of arthralgias, recurrent headaches and signs of nephropathy. Univariate and multivariate analysis of the obtained data showed that signs of nephropathy (proteinuria, hematuria) was the variable with the highest statistical relevance for distinguishing between patients with cutaneous (CDLE/SCLE) and with systemic LE (SLE) in all statistical models tested, followed by the presence of arthralgias and of high ANA titers (> or =1:320). In contrast, low ANA titers as well as anti-dsDNA antibodies showed little or no statistical relevance as a criterion for distinction. It seems, therefore, that cutaneous LE patients showing signs of nephropathy, presence of arthralgias and elevated ANA titers (> or =1:320) should be carefully monitored, because they may be at risk of developing systemic LE involvement.

The prevalence rates of systemic lupus erythematosus (SLE) may vary within 17-48/100,000 population worldwide. Although population-based epidemiological studies are still missing, the cutaneous variants of lupus erythematosus (LE) are 2-3 times more frequent than SLE itself. The most common age of onset is 20-40 y. Overall, cutaneous LE is regarded as a variant with less severe course and better prognosis. However, CDLE and SCLE last for many years and may lead, like SLE, to severe disability for work and limited life quality; also, a small proportion of patients with cutaneous LE develops SLE during the course of their disease. This implies considerable amount of medical management and costs for the community. Early recognition of cutaneous LE patients at risk to develop SLE and preventive measures against disease triggering factors are important tasks for physicians attending with cutaneous LE patients. It seems that signs of nephropathy, elevated ANA-titers and arthralgias may serve as prognostic predictors for transition into SLE. Characteristic features of cutaneous LE are photosensitivity and female predominance. UV light is a major environmental triggering factor in cutaneous LE. Skin lesions may be induced or preexistent lesions may exacerbate due to UV light in up to 80-90% of all patients. Therefore, socioeconomic counseling of the young patients, for example choice of occupation and sun protection, are essentials in compliant patients. Also, since females are 3-6 times more frequently affected than males, the possibility of hormonal influences including pregnancy and estrogen-containing drugs should be discussed. Risk considerations for females wishing to become pregnant are required, and avoidance of estrogen-containing contraceptives should be recommended.

An immunohistochemical analysis of skin biopsies was performed in 18 patients with cutaneous lupus erythematosus (LE), using the alkaline phosphatase and monoclonal anti-alkaline phosphatase method (APAAP). The study group was subdivided on the basis of clinical criteria into 10 patients with chronic discoid LE (CDLE) and eight patients with subacute cutaneous LE (SCLE). Using a panel of monoclonal antibodies the following results were obtained: (i) ICAM-1 was expressed on epidermal keratinocytes, dermal inflammatory cells, and endothelial cells in most biopsies, whereas LFA-1 was confined to the dermis. Attachments between keratinocytes or endothelial cells and activated T lymphocytes via ICAM-1/LFA-1 may be a possible mechanism of target/effector recognition in cutaneous LE. (ii) HLA-DR was expressed on epidermal keratinocytes and cells of the dermal infiltrate, but not on endothelial cells. HLA-DR+ cells probably function as antigen-presenting cells, leading to major histocompatibility complex-restricted cellular cytotoxicity in cutaneous LE. (iii) Interleukin 2 receptor expression on dermal inflammatory cells was weak, indicating non-specific activation of T lymphocytes. (iv) The dermal inflammatory cells were T lymphocytes, mainly of the helper/inducer subtype. B lymphocytes were rarely found in the dermis. In general, no significant immunohistochemical differences were found between CDLE and SCLE, suggesting that these variants represent clinical subtypes rather than different pathogenetic entities.

34 patients with subacute cutaneous lupus erythematosus (SCLE) were prospectively followed up and examined twice in 1986-1989 and 1990-1991, with a minimal interval of 2 years. The findings obtained were documented in a computerized registry and were evaluated by computer-assisted analysis. Thirty-one women and three men aged 23-66 years at the time of the first documentation (FD) were included in the study. The disease had lasted an average 6.5 years (from a few months to 39 years). Prominent cutaneous manifestations of SCLE were shown by 79.4% of the patients at the first visit compared to 58.8% at the second documentation (SD), whereas the incidence of acrolocalized vasculitis increased (FD 20.6%, SD 41.2%), as did that of Raynaud phenomenon (FD 20.6%, SD 26.5%). Mild extracutaneous symptomatology was found in 70.6% (FD) and in 85.3% (SD) of the SCLE patients. Arthralgia (FD 47%, SD 61.8%) and symptoms suggesting some involvement of the peripheral or central nervous system (FD 29.4%, SD 50%) were noted most frequently. Overall, during the follow-up there was a quantitative mild increase of extracutaneous symptomatology; a significant correlation between the presence of acrolocalized vasculitis and arthralgia was found. In conclusion, the prognosis of SCLE appears rather benign; fulminant courses were not observed. However, the risk of mild systemic involvement clearly increases during the prospective course of the disease, whereby acrolocalized vasculitis can be regarded as a cutaneous marker for extracutaneous manifestations.

In 67 patients with lupus erythematosus (LE), 33 patients with the chronic discoid type (CDLE), 22 with the subacute cutaneous type (SCLE) and 12 with systemic LE (SLE), the presence of circulating anticardiolipin antibodies (ACA) of the IgG and IgM isotypes was investigated with an enzyme immunoassay (ELISA). ACA of the IgG isotype were found in 13 patients (= 39.4%) with CDLE and ACA-IgM in 5 other cases (= 15.2%); among the SCLE patients ACA-IgG was increased in 8 (= 36.4%) and ACA-IgM in 1 other (= 4.5%), while in 3 further patients (= 13.6%) both ACA-IgG and IgM were detected. In total, ACA were found in 54.6% of the CDLE and in 54.5% of the SCLE patients. In SLE ACA-IgG alone was seen in 41.7% of the patients and ACA of both isotypes in another 33.3%. The serum levels of ACA were higher in SLE than in the cutaneous LE subsets. No correlation with the presence of ANA and binding capacity was detected in any of these groups. Vascular symptoms were seen in 19 of 39 patients with detectable ACA (= 48.7%), whereas only 9 of 27 ACA-negative patients (= 33.3%) were found to have vascular symptoms on clinical examination. Only 1 of the patients with isolated ACA-IgM elevation (5 CDLE patients, 1 SCLE patient) had vascular symptoms. The detection of circulating ACA is a non-specific but rather sensitive marker for cutaneous LE. In particular, the ACA-IgG isotype shows a statistical correlation with the presence of vascular symptoms and is useful as a complement to the immune serological tests for the diagnosis of LE.

In this retrospective study, the data of 97 patients with lupus erythematosus (LE) were evaluated according to clinical and laboratory criteria. 30 patients had localized chronic discoid LE (CDLE); 44 patients showed disseminated LE lesions either of the chronic discoid or the subacute cutaneous (SCLE) type; and 23 patients had systemic LE (SLE). The mean age ranged between 21 and 50 years. The male/female ratio was 1:3 in localized LE, 1:13 in disseminated LE with general symptoms, and 1:10 in SLE. Localized LE did not, as a rule, show any general symptoms. On the other hand, 14/44 patients (= 30%) with disseminated LE revealed general symptoms such as BSR elevation, arthralgia, anemia, and leukopenia. In addition, 4/44 patients (= 9%) with disseminated skin lesions showed various extracutaneous manifestations: nephritis (2), pericarditis (2), pleuritis (2), polyarthritis (1). Arthropathy was the major clinical manifestation in SLE (18/23 patients). Immunological parameters were usually negative in localized chronic discoid LE. 7/23 patients (= 30%) with disseminated LE had elevated ANA titers; 4/26 patients (= 15%) showed increased DNA binding capacity. In 57% of the patients with disseminated LE associated with general clinical symptoms, in contrast, we found elevated ANA titers; 71 of them revealed increased DNA binding capacity. Our findings suggest that disseminated LE, especially the SCLE type, may be regarded as variant of LE which tends to transition into SLE. Moreover, ANA titers may serve as a screening method; the detection of circulating DNA antibodies, however, is considered a rather specific parameter with regard to the diagnosis of systemic manifestation.