Mycophenolate mofetil is a promising option in immunosuppressive treatment of patients with moderate and severe systemic lupus erythematosus who did not show a satisfactory response to other immunosuppressives.
Hailey-Hailey disease is a blistering genodermatosis that shows acantholytic dyskeratosis throughout the epidermis. The aim of our study was to investigate the involvement of adherens structures and cytofilaments in this particular type of acantholysis. Both lesional and non-lesional skin from 18 patients was studied histologically and ultrastructurally. Additionally, the samples were stained for desmosomes, adherens junctions, keratin filaments, actin filaments, and actin-associated proteins, and finally investigated with an electron and a confocal laser scanning microscope (CLSM), respectively. Acantholytic dyskeratosis was not only confined to lesions, but was also focally detectable in clinically unaffected skin. Despite disruption and internalization of the desmosomes, keratinocytes remained linked together by well-preserved adherens junctions. Staining for actin filaments with fluorochrome-labeled phalloidin showed a remarkable formation of actin stress fibers in these keratinocytes. Thus, incomplete acantholysis, as demonstrable in both lesional and non-lesional skin of Hailey-Hailey patients, may be due to a cohesive function of the adherens junction-actin system succeeding the dissolution of desmosomes. Most remarkably, none of the adnexal epithelia expressed the intrinsic defect of cell adhesion. This finding offers an explanation for the successful treatment of Hailey-Hailey disease by dermabrasion, which after complete removal of the involved epidermis results in reepithelialization from skin appendages.
Our objective was to determine the frequency of antibodies to cyclic citrullinated peptides (CCPs) in a series of patients with a variety of rheumatic diseases. Seven hundred consecutive serum samples from patients at an outpatient clinic were tested for the presence of rheumatoid factor (RF) and anti-CCP. Clinical diagnosis, radiographic information, and other laboratory data were taken from patients' charts. The sensitivity and specificity of anti-CCP reactivity for the diagnosis of rheumatoid arthritis (RA) were 74.0% and 94.5%, respectively; the corresponding results for RF were 69.7% sensitivity and 81.0% specificity. Highest rates of false-positive RF tests were found in patients with SLE (18.3% vs. 12.7% CCP), Sjögren's syndrome (73.3% vs. 3.3% CCP), and a control group with chronic hepatitis (24.7% vs. 1.3% CCP). The detection of anti-CCP is useful for the diagnosis of RA because of its similar sensitivity but higher specificity compared with RF. Anti-CCP also helps to diagnose other inflammatory and noninflammatory diseases (especially connective tissue diseases) by reducing the rate of false-positive results in comparison with RF.
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