Locations of acantholysis in pemphigus vulgaris and pemphigus foliaceus.

BACKGROUND: Acantholysis in pemphigus vulgaris (PV) and pemphigus foliaceus (PF) occurs predominantly in the suprabasal area and the granular layer, respectively. However, acantholysis can occasionally be observed in unusual locations. METHODS: We retrospectively studied the acantholysis locations in 35 PV and 27 PF cases, and analyzed them using the index value of Desmoglein (Dsg) 1 and Dsg3 by enzyme-linked immunosorbent assay, clinical data, and inflammatory cells. We also analyzed the relationship between clinical subtype and various parameters. RESULTS: In PV, acantholysis was noted in the suprabasal area in 3 cases, in the lower half of the epidermis in 19 cases, and throughout the epidermis in 13 cases. In PF, acantholysis was observed in the granular layer in 6 cases, in the upper half of the epidermis in 14 cases, and throughout the epidermis in 7 cases. Mean index value of Dsg1 in PV patients with acantholysis throughout the epidermis was 2-fold higher than other PV patients. Neutrophils tended to infiltrate the dermis and epidermis more in PF than in PV. CONCLUSIONS: Higher Dsg1 index values seem to correlate with acantholysis in the upper part of the epidermis in PV. Neutrophils may play some role in unusual acantholysis locations in PF.

A prospective analysis of anti-desmoglein antibody profiles in patients with rheumatoid arthritis treated with thiol compounds.

BACKGROUND: Drug-induced pemphigus is mainly caused by drugs containing sulfhydryl (thiol) groups in their molecules. OBJECTIVES: To understand the serial alteration of anti-desmoglein (Dsg) antibody profile in patients with rheumatoid arthritis (RA) receiving thiol compounds. METHODS: Anti-Dsg1 or -Dsg3 antibodies were analysed twice in a 1.6-year interval, in the same series of RA patients. RESULTS: Eleven of 204 serum samples (5.4%) and 6 of 139 samples (4.3%) from the same RA group showed a positive reaction against Dsg1 or Dsg3 in the first and second screening tests, respectively. The positive rates were higher than those in patients with collagen diseases including systemic lupus erythematosus, Sjögren syndrome, mixed connective tissue disease, and systemic sclerosis. In comparison with the results in the first and second screening tests, one RA patient newly gained anti-Dsg3 antibody, and at least 4 patients lost the antibodies in 1.6 years. Three patients produced antibodies to Dsg1 and/or Dsg3 in a similar fashion as did in the first screening tests. Only one RA serum sample exhibited an intercellular reactivity to human skin or monkey esophagus by immunofluorescence, and another sample bound to a 130 kDa protein suggestive of Dsg3 by immunoblotting. Most anti-Dsg antibodies in RA patients recognized EDTA-resistant epitopes of Dsg different from EDTA-sensitive epitopes recognized by pemphigus sera. CONCLUSION: RA patients receiving any of the thiol compounds may gain autoantibodies to non-conformational epitopes of either Dsg1 or Dsg3, and that such autoantibodies alone are not capable of inducing acantholysis.

High resolution two-dimensional gel electrophoresis of human erythrocyte membrane proteins.

Three different two-dimensional (2-D) gel electrophoretic techniques have been modified to provide high resolution of human erythrocyte membrane proteins. The resulting gels were referenced to the established one-dimensional (1-D) sodium dodecylsulfate (SDS) gel electrophoretic profile, and the effects of endogenous proteolysis and cytosolic contamination were studied. It is concluded that in vitro proteolysis and cytosolic contamination do not contribute significantly to the patterns observed on the 2-D gels, under the conditions used for erythrocyte ghost preparation. The procedures require only small quantities of blood; as many as twenty 2-D gel profiles can be obtained from 5 ml of blood. The combination of nonequilibrium isoelectric focusing (IEF) in the first dimension, SDS electrophoresis in the second dimension, and very sensitive silver staining techniques resolves more than 250 individual protein spots. This appears to be the most useful single procedure for the analysis of red cell membrane proteins. Membrane protein profiles from patients with Duchenne muscular dystrophy, Wernicke-Korsakoff syndrome, and acanthocytosis with degeneration of the basal ganglia were compared with normal controls. The patterns for Duchenne muscular dystrophy and Wernicke-Korsakoff syndrome were not different from normal patterns. The pattern for the patient with acanthocytosis and degeneration of the basal ganglia consistently showed a high level for one protein in the 100,000 mol. wt. range.