Lupus band test and disease activity in systemic lupus erythematosus: does it still matter?

BACKGROUND: Lupus band test still has no clearly defined position within either diagnostic or disease activity measuring tools for systemic lupus erythematosus (SLE). OBJECTIVES: We tested the hypothesis that positive LBT correlates with global activity of SLE measured by the SLE Disease Activity Index (SLEDAI) score. METHODS: In total, 90 SLE patients who underwent biopsy of sunprotected non-lesional (SPNL) skin were studied prospectively. The skin specimen was processed for standard direct immunofluorescence. The patients were classified into groups as negative and positive LBT, and the latter were further subdivided on the basis of the type and morphology of the deposits. Every patient was thoroughly examined and assigned a SLEDAI score. The relationship between LBT findings and SLEDAI score was analysed. RESULTS: The disease was significantly more active in patients with positive LBT and in those with a higher number of deposited immunoreactants. Almost all patients with renal involvement had a positive LBT. CONCLUSIONS: LBT on SPNL skin may be a good marker of severe disease at presentation, particularly when three immunoglobulins are found at the dermoepidermal junction.

Adamantiades-Behçet's disease, deep venous thrombosis and anticardiolipin antibodies: report of two cases.

Vascular thrombosis was found in different proportions of patients with Adamantiades-Behçet's disease (ABD), depending on the ethnicity of the population under study. Various thrombophilic factors, including the levels of anticardiolipin antibodies (ACA), were investigated for their role in the thrombotic process with conflicting results. The prevalence of ACA varies considerably in different studies, but their presence has not been associated with increased risk for vascular thrombosis. We present two cases with ABD, deep venous thrombosis (DVT) and elevated levels of ACA that fulfil the criteria for both ABD and antiphospholipid syndrome (APS).

Pyoderma gangrenosum with spleen involvement and monoclonal IgA gammopathy.

A 46-year-old man with a 3-year history of pyoderma gangrenosum was admitted with ulceration (6 x 5 cm), on the right leg. Previously he had been treated with tapering doses of prednisone (maximum dose 1 mg/kg per day); however, he had had a few exacerbations following each taper of prednisone dose. Immunoelectrophoresis demonstrated monoclonal IgA gammopathy of lambda light chains. Abdominal echography and abdominal computed tomographic scan revealed multiple splenic abscesses. Treatment was started with oral prednisone (1 mg/kg per day) and cyclosporin (5 mg/kg per day) and 6 weeks later complete remission was achieved. Systemic involvement in pyoderma gangrenosum is very rare, and according to our knowledge there are only a few cases with spleen involvement.

[Significance of deposits of immunoglobulins G, A, M and C3 complement component in the basal membrane zone of clinically unchanged skin for the diagnosis and evaluation of systemic lupus erythematosus activity]. / Znacaj nalaza deposita imunoglobulina G, A, M i C3 komponente komplementa u zoni bazalne membrane klinicki neizmenjene koze za dijagnozu i procenu aktivnosti sistemskog eritemskog lupusa.

Direct immunofluorescent test of clinically unaltered skin, the so-called lupus band test (LBT) was performed in 38 patients with systemic lupus erythematosus. Test was positive in 23 patients or 60.52%, respectively. It was established that the relative number of LBT positive patients was increased, from 37.5% in the group with the lowest disease activity determined by SLEDAI score, over 66.66% in the group with moderate activity till 100% in the patients with extremely active disease. Comparison of SLEDAI score (disease activity) mean values in the group of patients with systemic lupus erythematosus (SLE) with positive LBT compared to the group with negative LBT revealed statistically significant difference (< 0.05) in favour of the first group. No significant disease activity among patients' groups, formed according to the types and number of registered immunoreactants was observed. LBT in patients with SLE should be done in prognostic purposes. Positive LBT indicates the more active disease. Besides, the test can be a valuable diagnostic indicator in patients who do not fulfill the diagnostic criteria of American College of Rheumatology.

[Treatment of eosinophilic fasciitis with cimetidine]. / Lecenje eozinofilnog fasciitisa cimetidinom.

A case of a female patient with clinical and histological manifestation of eosinophilic fascitis has been reported. After five months cimetidine therapy a significant improvement was achieved. During the therapy some adverse effects occurred and cimetidine therapy had to be ceased.

[Livedo vasculitis]. / Livedo vasculitis.

Twenty one patients with livedoid vasculitis are presented. Diagnosis was based on the clinical, histopathologic and direct immunofluorescent findings. The majority of patients were women (2:1), aged up to 30 years. Except for two patients with chronic venous insufficiency, the rest were with idiopathic-primary livedoid vasculitis. They all reacted favorably to the therapy with pentoxifylline, dipyridamole and acetylsalicylic acid. It is necessary to observe the difference to the "real" vasculitis of the skin, which requires more aggressive therapy.

Skin lesions--an indicator of disease activity in systemic lupus erythematosus?

Cutaneous manifestations have great diagnostic value for systemic lupus erythematosus (SLE). In this study we tried to establish a correlation between lupus erythematosus LE-specific and LE-nonspecific cutaneous lesions and disease activity measured by the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Sixty-six patients with SLE were evaluated. They were divided into three groups having: (1) only LE-specific lesions (38 or 58.46%); (2) only LE-nonspecific lesions (4 or 6.15%); and (3) both types of lesions (23 or 35.38%). Results were analyzed using the Student t-test. Patients with LE-nonspecific skin manifestations had significantly increased disease activity compared to those with only LE-specific lesions. The number of different skin lesion types also correlated with disease activity. It was significantly increased in a group with three different types of lesion, either specific or nonspecific. Patients with only one type of lesion had mild disease. An intermediate disease activity was found in the group with two different lesion types. Lupus-specific skin manifestations serve primarily as an important diagnostic clue. In conclusion, patients with LE-nonspecific lesions have significantly more active SLE than those with LE-specific lesions and may therefore require more intensive therapy and disease monitoring.

[Tuberous sclerosis]. / Tuberozna skleroza.

Tuberous sclerosis is a rare hereditary disease which appears immediately after birth of during the second and third year of life. It is a multiorgan disorder characterized by convulsions, mental retardation and focal angiofibromyoma. The main findings are brain lesions including tuberous and astrocytes hamarthomas by which this disease was named. Renal alterations are angiofibromyolipoma and cysts, which are present in 40-80% of patients. The diagnosis is based on clinical, radiological and histological findings. This disease has a progressive course and fatal outcome. The therapy is symptomatic and surgical. The aim of this paper was to present this rare disease, which occurred in this patient during fourth year of life. Besides brain changes the patient also has extensive morphological renal alterations and renal failure. She died in 40th year of life due to multiorgan dysfunction.

[Onychatrophy in a female patient with lichen planus]. / Onihatrofija kod bolesnice sa lichen planus-om.

A case of female patient with onychatrophy, as a single clinical manifestation of lichen planus is reported. Nail histopathologic and direct immunofluorescent findings confirmed the diagnosis.

Bullous delayed pressure urticaria; pressure testing may produce a systemic reaction.

We report a patient with bullous delayed pressure urticaria (DPU) and chronic idiopathic urticaria (CIU) in whom a systemic reaction occurred. The reaction occurred 18 h after a pressure test had been performed on the right forearm. Blood histamine levels were more elevated in the sample taken from the forearm on which the test had be applied. Skin biopsy revealed both intraepidermal and subepidermal bullae with a sparse dermal inflammatory infiltrate and direct immunofluorescence showed linear deposition of fibrinogen along the epidermodermal basement membrane. As far as we are aware this is only the third case of bullous DPU reported and the first associated with generalized urticaria and angioedema and severe broncho-obstruction. Possible pathophysiological mechanisms are discussed.