1/55. Relevance of complement fixing antinuclear antibodies.BACKGROUND: connective tissue diseases (CTDs) are a heterogeneous group of disorders defined by the association of a variety of clinical manifestations with immunologic and other laboratory findings. Overlap of syndromes and aberrant findings appear rather frequently. methods: Sera of eight antinuclear antibody (ANA) negative, cases of subacute cutaneous lupus erythematosus (SCLE) with antibodies to Ro (SS-A) and a ninth case with clinical and laboratory signs of sjogren's syndrome and systemic lupus erythematosus (SLE) were tested for complement (C') fixing antinuclear antibodies (C-ANAs). The ninth case was examined in depth by direct immunofluorescence (DIF) and a two-step "C DIF" test of biopsies for C' fixation to in vivo bound ANAs, as well as serum tests for C-ANA, ANA, and SCLE markers. RESULTS: Sera of five of the eight ANA negative, Ro(SS-A) positive SCLE cases had C-ANAs. The ninth case, a 50-year-old woman with clinical and laboratory signs of sjogren's syndrome and SLE, gave a strong positive C DIF reaction in the skin biopsy for in vivo bound ANAs that fix C', but negative ANAs and C-ANAs in routine serum tests; they revealed antimitochondrial antibodies. serum tests on normal skin, however, revealed weak ANA and strong C-ANA reactions with in vitro fixed C'. CONCLUSIONS: ANA negative cases of SCLE or sjogren's syndrome may have C-ANAs. A case with sjogren's syndrome and signs of SLE had both in vivo and in vitro C' fixing ANAs. C-ANA tests can aid in the identification of such cases.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, lupus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
2/55. The relationship between facial annular erythema and anti-SS-A/Ro antibodies in three East Asian women.A distinct annular erythema developed on the cheeks of three East Asian women who had anti-SS-A/Ro (SSA) antibodies. The erythema was characterized by a wide, elevated border and central pallor. Histologically, there was a coat-sleeve-like infiltration of lymphocytes around the blood vessels, appendages, and secretory gland cells in the dermis. Immunohistological analysis clarified that the majority of infiltrating lymphocytes were CD4-positive T cells. Abnormal expression of hla-dr antigens in the perivascular, appendage, and secretory gland cells in the dermis was also observed. The differential diagnosis of the three patients lay between Sjogren syndrome (SjS), Sjogren/systemic lupus erythematosus overlap syndrome and an asymptomatic clinical state. These results are consistent with recent findings of major histocompatibility complex class II expression on target organs in various autoimmune diseases. Based on these findings, erythema appears to represent a broad cutaneous manifestation of these diseases. Furthermore, the presence of SSA antibodies, aberrant HLA-DR expression, and sun exposure may be responsible for the development of erythema.- - - - - - - - - - ranking = 0.50180697656421keywords = lupus erythematosus, erythematosus, lupus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
3/55. Lupus erythematosus with antiphospholipid syndrome and erythema multiforme-like lesions.The occurrence of erythema multiforme (EM) in patients with lupus erythematosus (LE) has been described previously as a coincidental association. In contrast, LE with EM-like lesions and a peculiar immunological pattern, including positive rheumatoid factor, antinuclear antibodies and a serum antibody against an extract of human tissues recently recognized as similar to Ro (SSA), constitutes an established entity named Rowell's syndrome. We describe a woman with LE and long-standing widespread vesiculobullous and necrotic haemorrhagic EM-like lesions in combination with Ro (SSA) and scl-70 antibodies and the typical laboratory findings of the antiphospholipid syndrome (APS), namely lupus anticoagulant, anticardiolipin antibodies and prolonged activated partial thromboplastin time. This case could conceivably be consistent with a diagnosis of Rowell's syndrome, if the latter is regarded as a clinicopathological spectrum. However, the coexistence of LE, persistent EM-like disease and incomplete APS may also fulfil the diagnostic criteria for the 'multiple autoimmune syndromes'. We speculate that the laboratory markers of APS play a pivotal part in such an unusual clinical presentation.- - - - - - - - - - ranking = 1.1369283287557keywords = lupus erythematosus, erythematosus, lupus (Clic here for more details about this article) |
4/55. Lupus erythematosus tumidus and chronic discoid lupus erythematosus in carriers of X-linked chronic granulomatous disease.Two Caucasian carriers for chronic granulomatous disease (CGD) developed cutaneous lupus erythematosus (LE) with clinically and morphologically characteristic appearance for chronic discoid lupus erythematosus (DLE) and lupus erythematosus tumidus (LET). Direct immunofluorescent examinations and ANA titers were positive in both young women. No systemic involvement due to the ACR criteria was evident. Their sons suffered from X-linked cytochrome-b negative CGD. The diagnosis of CGD was based on measurement of oxidative burst activity by nitroblue tetrazolium (NBT) slide test and by flow cytometry using dihydrorhodamine 123 (DHR). The absence of cytochrome b558 in neutrophilic granulocytes was confirmed photometrically and by flow cytometry using the 7D5 monoclonal antibody against cytochrome b. We report for the first time the association of the photosensitive LE subtype LET and the X-linked CGD carrier state. Tissue damage by UV radiation and a reduced antimicrobial capacity may lead to recurrent immune stimulation and may together with genetic predisposition explain the occurrence of cutaneous LE in female carriers of CGD.- - - - - - - - - - ranking = 4.0914667858568keywords = lupus erythematosus, erythematosus, lupus (Clic here for more details about this article) |
5/55. Linear eruptions of the nose in childhood: a form of lichen striatus?We report four children with linear eruptions on the nose, with overlapping features of lichen striatus and linear cutaneous lupus erythematosus. However, linear lupus erythematosus has rarely been reported, and lichen striatus, although classically linear, rarely affects the face. The linear distribution of lesions from the glabella to the ala nasi may represent distribution following Blaschko's lines.- - - - - - - - - - ranking = 0.99638604687157keywords = lupus erythematosus, erythematosus, lupus (Clic here for more details about this article) |
6/55. Neonatal lupus erythematosus.Neonatal lupus erythematosus (NLE) is a rare disease characterized by the transplacental passage from the mother to the fetus of autoantibodies, in particular anti-Ro(SS-A), anti-La(SS-B), or both. The majority of infants with NLE exhibit isolated congenital heart block, cutaneous lesions analogous to those of adult subacute cutaneous lupus erythematosus, or both. We report a case of NLE in a 3-month-old male infant, born to a clinically asymptomatic mother, presenting small, annular, erythematous plaques with sharp, hyperkeratotic borders and central clearing localized at the eyebrow region. Both the infant and the mother were positive for anti-Ro(SS-A).- - - - - - - - - - ranking = 2.9891581406147keywords = lupus erythematosus, erythematosus, lupus (Clic here for more details about this article) |
7/55. Successful treatment of discoid lupus erythematosus with argon laser.Vascular lesions with telangiectasias on visible areas, such as the face, are common in discoid lupus erythematosus (DLE); however, an efficient management of these skin lesions can sometimes be difficult. Since argon laser light is able to specifically coagulate vascular structures, it has been used in the treatment of various vascular skin malformations. Therefore, we addressed the issue whether argon laser treatment could be a therapeutic alternative for this disease. Here, we report on a patient with DLE, who suffered from long-standing erythematous, telangiectatic plaques on the face refractory to standard regimens of therapy. After 2 laser applications, a significant improvement was observed and after 5 sessions of argon laser therapy the treated skin lesions had completely resolved with an excellent cosmetic result. The patient tolerated the laser treatment well without any short-term side effects. These data indicate that argon laser therapy might be a powerful alternative approach in the treatment of vascular skin lesions of DLE.- - - - - - - - - - ranking = 2.4909651171789keywords = lupus erythematosus, erythematosus, lupus (Clic here for more details about this article) |
8/55. F.I.GU.R.E.: facial idiopathic granulomas with regressive evolution. is 'lupus miliaris disseminatus faciei' still an acceptable diagnosis in the third millennium?We report the case of a 69-year-old woman who presented a papular eruption on the eyelids. Histological features revealed a tuberculoid granuloma with a central caseating necrosis. Laboratory and radiological investigations revealed no tuberculosis and no systemic granulomatosis. Absence of vascular symptoms, inefficiency of cyclines and histopathological findings excluded granulomatous-type rosacea. Lupus miliaris disseminatus faciei (LMDF) was our final diagnosis. On the basis of our findings and a literature review, we believe that LMDF is an entity distinct from either skin tuberculosis or granulomatous-type rosacea. However, its name is confusing, and we propose to change it to 'facial idiopathic granulomas with regressive evolution (FIGURE)'.- - - - - - - - - - ranking = 47.833727064996keywords = disseminatus, lupus (Clic here for more details about this article) |
9/55. Discoid lupus erythematosus (DLE)-like lesion induced by uracil-tegafur (UFT).A mixture of uracil and tegafur (UFT) is a common antineoplastic agent in japan. We report a 64-year-old Japanese woman with discoid lupus erythematosus (DLE)-like lesions which were induced by UFT. After surgery to treat lung cancer, UFT (300 mg/day) was administered and she developed round erythema on her right cheek. A skin biopsy specimen taken from the right cheek site revealed atrophy of the epidermis, a slight liquefaction of the basal cell layer, and patchy lymphocytic infiltration in the perivascular and perifollicular regions. A test for antinuclear antibody was weakly positive (80 fold), and rheumatoid factor was slightly elevated (7.6 IU/ml). After discontinuation of UFT, the erythema completely regressed within 2 months. We reviewed 17 cases of DLE-like lesions induced by fluorouracil agents and summarized the common features.- - - - - - - - - - ranking = 2.4909651171789keywords = lupus erythematosus, erythematosus, lupus (Clic here for more details about this article) |
10/55. Lupus erythematosus panniculitis in a patient with autoimmune hepatitis.Lupus erythematosus panniculitis (profundus), a rare variant of chronic panniculitis, sometimes develops during the course of discoid lupus erythematosus or systemic lupus erythematosus. A 61-year-old woman had suffered from autoimmune hepatitis type I for 5 years. prednisolone had been administered as maintenance therapy and her hepatitis had been well controlled. However, asymptomatic erythematous indurated nodules developed symmetrically in both pre-auricular regions, and skin biopsy revealed lupus erythematosus panniculitis (profundus). Increase in dosage of prednisolone resolved the skin lesion, leaving depressed atrophic scars. This is the first report of lupus erythematosus panniculitis complicating autoimmune hepatitis.- - - - - - - - - - ranking = 2.7515305741294keywords = lupus erythematosus, erythematosus, lupus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
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