Lupus erythematosus panniculitis is a rare disease characterized by deep subcutaneous nodules, most commonly localized on the upper limbs and face. Unique clinical presentations, such as linear configuration or ‘over...Lupus erythematosus panniculitis is a rare disease characterized by deep subcutaneous nodules, most commonly localized on the upper limbs and face. Unique clinical presentations, such as linear configuration or ‘overlap’forms between lupus erythematosus panniculitis and localized scleroderma have been reported. We present here the clinical characteristics, course and laboratory findings of 2 patients having linear lupus erythematosus panniculitis with localized scleroderma-like changes. The 2 patients (of the 14 patients with lupus erythematosus pan niculitis seen by us since 1990) were females with a young age at the onset of disease (median, 25 years). In 1 case, evolution into systemic lupus erythematosus with severe renal involvement occurred whereas the other patient, who had a spontaneous abortion and exhibited anticardiolipin antibodies, should be followed and screened for the emergence of antiphospholipid syndrome. Thus, the clinical behavior of this variant seems to be more aggressive, as compared with the usual course of lupus erythematosus panniculitis, which is considered to be a benign disease, although some reports have suggested that its prognosis is not always favorable. The linear distribution could be the clinical hallmark of such a unique, ‘sclerodermic’subset of lupus erythematosus panniculitis.展开更多
Background:Lupus erythematosus profundus(lupus panniculitis)is a rare variant of lupus erythematosus with predominant involvement of the subcutaneous tissue.There are only a few reported series of patients with this c...Background:Lupus erythematosus profundus(lupus panniculitis)is a rare variant of lupus erythematosus with predominant involvement of the subcutaneous tissue.There are only a few reported series of patients with this condition;none in individualsof African ancestry.The aim of the study was to evaluate clinical,histopathological and laboratory findings in black South African patients with lupus profundus.Patients and methods:Ten prospectively observed patients were studied.Skin biopsies were performed,and several laboratory tests routinely employed in patients with lupus erythematosus were carried out.All patients were followed up for at least a year and their response to treatment was assessed.Results:All patients were females.Mean age at diagnosis was 28.5 years.Face was the most common site of involvement.In five patients,the periorbital edema was the initial manifestation.Only one patient had systemic lupus,and three patients also showed lesions of discoid lupus.In only 30%of the patients were the ANA titers greater than 1 :80,and positivity in other lupus tests was rare and inconsistent.In general,patients responded to antimalarials and/or systemic corticosteroids.Conclusion:African patients with lupus profund us when compared with other series comprising Asian and white patients showed greater frequency of periorbital edema as the initial manifestation.Review of the literature disclosed that this subset of lupus profund us appears to have a more benign course.展开更多
文摘Lupus erythematosus panniculitis is a rare disease characterized by deep subcutaneous nodules, most commonly localized on the upper limbs and face. Unique clinical presentations, such as linear configuration or ‘overlap’forms between lupus erythematosus panniculitis and localized scleroderma have been reported. We present here the clinical characteristics, course and laboratory findings of 2 patients having linear lupus erythematosus panniculitis with localized scleroderma-like changes. The 2 patients (of the 14 patients with lupus erythematosus pan niculitis seen by us since 1990) were females with a young age at the onset of disease (median, 25 years). In 1 case, evolution into systemic lupus erythematosus with severe renal involvement occurred whereas the other patient, who had a spontaneous abortion and exhibited anticardiolipin antibodies, should be followed and screened for the emergence of antiphospholipid syndrome. Thus, the clinical behavior of this variant seems to be more aggressive, as compared with the usual course of lupus erythematosus panniculitis, which is considered to be a benign disease, although some reports have suggested that its prognosis is not always favorable. The linear distribution could be the clinical hallmark of such a unique, ‘sclerodermic’subset of lupus erythematosus panniculitis.
文摘Background:Lupus erythematosus profundus(lupus panniculitis)is a rare variant of lupus erythematosus with predominant involvement of the subcutaneous tissue.There are only a few reported series of patients with this condition;none in individualsof African ancestry.The aim of the study was to evaluate clinical,histopathological and laboratory findings in black South African patients with lupus profundus.Patients and methods:Ten prospectively observed patients were studied.Skin biopsies were performed,and several laboratory tests routinely employed in patients with lupus erythematosus were carried out.All patients were followed up for at least a year and their response to treatment was assessed.Results:All patients were females.Mean age at diagnosis was 28.5 years.Face was the most common site of involvement.In five patients,the periorbital edema was the initial manifestation.Only one patient had systemic lupus,and three patients also showed lesions of discoid lupus.In only 30%of the patients were the ANA titers greater than 1 :80,and positivity in other lupus tests was rare and inconsistent.In general,patients responded to antimalarials and/or systemic corticosteroids.Conclusion:African patients with lupus profund us when compared with other series comprising Asian and white patients showed greater frequency of periorbital edema as the initial manifestation.Review of the literature disclosed that this subset of lupus profund us appears to have a more benign course.
