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PubMedDate not supplied

[Experience with FITC-labeled antihuman IgG "Weissensee" in dermatological diagnostics].

It is reported on results and experiences with immunofluorescent technique in dermatological diagnostic, using FITC-conjugated anti-human IgG "Berlin-Weissensee". While immunofluorescene on our conditions is an important diagnostic help in systemic and discoid lupus erythematosus as well as bullous pemphigoid, there are many nonspecific results in patients suffering from pemphigus vulgaris.

Fluorescent Antibody Technique↗

PubMed1978

Association of systemic lupus erythematosus and SLE-like syndromes with hereditary and acquired complement deficiency states.

The most prominent association of rheumatic diseases with hereditary complement deficiency is systemic lupus erythematosus (SLE) and discoid lupus erythematosus with homozygous C2 deficiency in females. The lupus disease in these patients differ from classic lupus in 1) the increased incidence of discoid lesions, 2) the low incidence of renal disease, 3) the low or absent titers of antibodies to native DNA, and 4) the infrequent finding of immunoglobulin and complement in skin lesions. The strong positive linkage disequilibrium between C2 deficiency and HLA genes raises the possibility that genes other than those determining C2 levels may have the primary role in determining predisposition to disease in these patients. However, the finding of similar diseases in certain patients with hereditary angioedema and SLE-related syndrome who have acquired deficiency of the early components of complement supports a primary role of the C2 deficiency gene in predisposing to lupus disease in these patients.

Arthritis, Rheumatoid↗

PubMed1999

[Connective tissue diseases in the hospital outpatient service in Lomé (Togo)].

PURPOSE: There have been few studies of connective tissue diseases in Africa. METHODS: A retrospective study was conducted in order to describe the various connective tissue diseases and their semiological profile in patients attending the dermatology and rheumatology units at Lomé hospital. RESULTS: Clinical examinations showed that eighty-four (0.2%) out of the 34,169 patients were suffering from connective tissue diseases. Diseases that were encountered were the following: scleroderma (18 cases), systemic lupus erythematosus (four cases), discoid lupus erythematosus (15 cases), rheumatoid arthritis (29 cases), polymyositis and dermatomyositis (16 cases), juvenile rheumatoid arthritis (one case), giant cell arteritis (one case). Raynaud's syndrome was present in six out of the 18 patients suffering from scleroderma. Nephrotic syndrome was observed in a patient suffering from systemic lupus erythematosus. A septicemia caused this patient's death. Two patients suffering from polymyositis had cancer. No etiology was found in the 14 other patients. Hip involvement was present in two patients suffering from rheumatoid arthritis. Patients with rheumatoid arthritis had no systemic involvement (nodulitis, vasculitis). CONCLUSION: Our results are in agreement with those of previous studies on connective tissue diseases in Africa. However, further studies are required to better understand the epidemiological and semiological profiles of connective tissue diseases in Africa.

Adolescent↗

PubMed1985

Detection of cross reactive anti-DNA antibody idiotypes on tissue-bound immunoglobulins from skin biopsies of lupus patients.

Cross-reactive anti-DNA antibody idiotypes have been identified on tissue-bound immunoglobulins from skin biopsies of patients with systemic lupus erythematosus (SLE) and discoid lupus erythematosus (DLE). Four polyclonal and two monoclonal anti-idiotypic reagents were used to screen biopsies from 24 patients with SLE, 23 patients with DLE, and 15 other patients with IgM-positive skin biopsies. Up to 46% of the SLE patients and 30% of the DLE patients were found to share idiotypes present on immunoglobulins deposited at the dermal-epidermal junction. Inhibition studies in four patients indicated that the idiotypes were on anti-DNA antibodies. In contrast, none of the anti-idiotypic antibodies bound to any of the control biopsies. These findings imply that some tissue-bound autoantibodies are derived from related families of high-frequency germ-line genes that are expressed in both SLE and DLE.

Antibodies, Antinuclear↗

PubMed1992

Immune-mediated disease in the old English sheepdog.

Immunological data are reported from 19 cases of immune-mediated disease recorded in the old English sheepdog breed in Western Australia between 1978 and 1989. The conditions included autoimmune haemolytic anaemia (seven), idiopathic thrombocytopenia (one), Evans' syndrome (five), multiple myeloma (two), systemic lupus erythematosus (one), discoid lupus erythematosus (one) and hypothyroidism (two). The most consistent serological findings were raised serum IgG (60 per cent), depressed serum IgM (60 per cent) and the presence of multiple autoantibodies (anti-red blood cell 78 per cent, antinuclear antibody 44 per cent, rheumatoid factor 19 per cent). An underlying, breed-related disorder of immune regulation may account for these observations.

Animals↗

PubMed1999

Patient with lichen planus and conjunctival immunopathologic features of lupus erythematosus.

PURPOSE: To report a case of severe cicatricial conjunctivitis in a patient with lichen planus, which is known to affect the skin and mucous membranes. The conjunctival immunopathologic features were consistent with lupus erythematosus rather than with lichen planus. METHOD: Oral mucosal and skin biopsies were performed with histopathologic and immunofluorescent studies consistent with lichen planus. The patient later had a biopsy of cicatrized conjunctiva with histopathologic and immunofluorescent findings consistent with lupus erythematosus. Evaluation by rheumatology and dermatology consultants demonstrated no evidence of active systemic lupus erythematosus or discoid lupus erythematosus. RESULTS: The patient was treated with topical and systemic immunosuppressives and her disease eventually stabilized. CONCLUSION: The case demonstrates two coexisting autoimmune disease entities: lichen planus of the skin and oral mucosa and a cicatricial conjunctivitis consistent with lupus erythematosus.

Biopsy↗

PubMed1992

[Tumorous eyelid changes in systemic diseases].

Lid tumors appear to invite the physician to make a diagnosis at first glance. This poster displays examples of lid tumors that occur in the context of malignancies, inflammatory and metabolic diseases and phacomatoses. The most frequent primary sites in metastatic eyelid tumors are the breast, the lung and cutaneous melanoma. Because of their comparatively low incidence and variable appearance these lid tumors are, at least in the beginning, often misinterpreted. Careful differential diagnosis may help in avoiding the diagnostic pitfalls of the masquerade syndromes. Lid tumors associated with sarcoidosis, lupus erythematosus discoides, lupus vulgaris, syphilis are briefly mentioned. Xanthelasmas occur more frequently in diabetics than in the normal population. About 5% of patients with xanthelasma suffer from hyperlipidemia. Neurofibromas and cavernous hemangiomas of the lid may accompany von Recklinghausen's and Sturge-Weber's diseases.

Blepharitis↗

PubMed2004

Apparent diffusion coefficient mapping of the normal parotid gland and parotid involvement in patients with systemic connective tissue disorders.

PURPOSE: We hypothesized that a difference in restricted diffusion would exist in patients with connective tissue disorders (CTD) as compared with those without CTD. Our purpose was to determine whether the apparent diffusion coefficient (ADC) measurement could be used to identify parotid abnormalities in patients with CTD. METHODS: One neuroradiologist, who was unaware of patient histories, retrospectively measured the ADC values for the parotid glands in 121 patients who underwent clinically indicated brain MR imaging in which the parotid glands were sufficiently depicted. Regions of interest were obtained from both the left and right parotid glands. After the medical records were reviewed and exclusion criteria were used, 90 non-CTD and seven CTD patients (systemic lupus erythematosus = 5; discoid lupus erythematosus = 1; Sjögren syndrome = 1) remained. The two groups were then compared. Statistical analysis consisted of Wilcoxon sign rank and Mann-Whitney tests. RESULTS: The combined mean ADC for both parotid glands in 90 healthy patients was 0.50 +/- 0.28 x 10(-3) mm(2)/s (95% CI, 0.44 x 10(-3), 0.56 x 10(-3)). The combined mean ADC for both parotid glands in the seven CTD patients was 0.96 +/- 0.24 x 10(-3) mm(2)/s (95% CI, 0.79 x 10(-3), 1.14 x 10(-3)). The mean ADC for the CTD patients' parotid glands was significantly higher than that of the non-CTD patients (P =.0001), which suggests there is less restricted diffusion in parotid glands affected by CTD when compared with normal parotid glands. CONCLUSION: These results suggest that ADCs may be used to detect parotid abnormalities in patients with CTD that are not identified by standard imaging. Although preliminary, the results indicate a potential role for ADC mapping in detection of subclinical parotid disease.

Adolescent↗

PubMed1984

Complement deficiency states and infection: epidemiology, pathogenesis and consequences of neisserial and other infections in an immune deficiency.

Inherited deficiencies of the complement proteins are rare in unselected populations. Examination of patients with the clinical correlates of complement deficiency (autoimmune disease and certain bacterial infections) shows the frequency of inherited complement deficiency to rise enormously (5.9% of patients with systemic lupus erythematosus, 10 to 25% of adults with sporadic meningococcal disease). Autoimmune diseases of all types, but especially systemic lupus erythematosus, discoid lupus and glomerulonephritis, are seen in all categories of complement deficiency, most typically in those of the early classical pathway (C1, C4, C2). Pneumococcal infections are characteristic of deficiencies of the early classical pathway, as well. Deficiencies of C3 are associated with severe disease including autoimmune phenomena, pneumococcal and neisserial infections. C3-deficient patients become ill substantially earlier in life. Infections with N. meningitidis and N. gonorrhoeae are most typical of the late component deficiencies, with over 40% of homozygotes affected. Despite the presence of this deficiency from birth and the peak age-specific incidence of meningococcal disease in the general population at ages 3-8 months, the median age of first infection in the late component-deficient patients is 17 years. Relapse of infection is ten times more common in these patients, and discrete recurrences are seen in 45% of affected individuals. An unusual and unexplained predilection for infection with serogroup Y N. meningitidis exists. Despite an immune deficiency, and problems with ascertainment bias, it appears that persons with late component complement deficiency enjoy less mortality than normals who contract meningococcal disease. Attempts to explain the pathogenesis of neisserial infection in late component deficiencies have focused on the concept that normally non-pathogenic serum-sensitive bacteria are etiologic in the absence of serum bactericidal activity. Data to support this concept remain to be developed and contrary data exist. A separate mechanism may predispose properdin-deficient patients to meningococcal infection, since they appear to develop fulminant infections with high mortality.

Adolescent↗

PubMed1978