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The activities of five lysosomal hydrolases were determined fluorometrically in the serum of patients with systemic sclerosis (PSS), systemic lupus erythematosus (SLE), dermatomyositis (DM), rheumatoid arthritis (RA), or Raynaud's disease (RD). In PSS the β-galactosidase activity was significantly increased compared with controls and the other connective tissue diseases. The β-N-acetyl-glucosaminidase was significantly increased in PSS, SLE and DM. In PSS both enzymes were more active in the early stage of the disease than later. These changes of enzyme pattern seem to be a relatively reliable marker for the differential diagnosis of PSS compared to other connective tissue diseases, especially for RD, in which the β-galactosidase activity was significantly decreased. Further work is required to determine whether these polysaccharide-degrading acid hydrolases play a role in the pathogenesis of PSS. [ABSTRACT FROM AUTHOR]

In polyacrylamide gel gradient electrophoresis, serum and blister fluid of patients with bullous pemphigoid showed a nearly identical protein distribution pattern except for one more concentrated fraction in the blister fluid. This fraction could be isolated by several gel chromatographic steps and then be characterized as a protein with a molecular weight of 240,000 (BFP 240,000) consisting of several subunits. Two of these subunits could be identified as C3 fragments and two other ones as basement membrane zone antigens (BMZ-Ag) by means of a modified Laurell technique. The molecular weight of the BFP 240,000 may be small enough to allow it to penetrate through the vessel wall into the blood circulation where, with the BMZ-antibodies (BMZ-Ab), BMZ-Ag-BMZ-Ab-complexes can be formed. The occurrence of such immune complexes in the serum has been shown in a previous paper. [ABSTRACT FROM AUTHOR]

No tree anti-basement membrane antibodies were found in the sera of four patients with bullous pemphigoid, and no free anti-DNA antibodies in the sera of two patients with systemic lupus erythematosus. The sera were treated with 3 mol/l urea and by gel chromatography to separate these antibodies from any circulating complexes, and antibodies to basement membrane were detected by immunofIuorescence and to DNA by the Farr technique. The appropriate antibodies were found in all the sera, indicating that failure to detect antibodies by routine procedures may be due to binding of the antibodies to soluble antigens, forming complexes in the sera. [ABSTRACT FROM AUTHOR]

A 65-year-old man with recurrent erythema migrans arciforme et palpabile (EMAP) is reported. EMAP is characterized as a T-cell pseudolymphoma, the cell populations of which are composed of T-helper- and T-suppressor cells in a ratio of 2:1. EMAP can therefore not be differentiated immunohistologically from the lymphocytic infiltration of Jessner-Kanof.

Report on a 20 years old pregnant woman, who fulfilled the criteria of the impetigo herpetiformis as well as later those of the pustular psoriasis Zumbusch in her clinical course. The impetigo herpetiformis is interpreted as a variant of the pustular psoriasis occurring during the pregnancy with lowered calcium-level, which increases the endogeneous eruption pressure. After negative attempts of treatment with Prednisolon, antibiotics, and later with Methotrexat an excellent therapeutic effect could be achieved by oral PUVA-treatment.

One patient suffering from pemphigus foliaceus exhibited the typical intercellular deposits of immunoglobulins in the subcorneal layers of the affected and not affected skin by direct be detected by means of several antigensubstrates. They could only be found after splitting of the antigen-antibodies-complexes. The laboratory findings exclude a more distinct deviation of the humoral and cellular immune reactivity.

Three day cultures of human peripheral blood lymphocytes (PBL) contain significant numbers of lymphoblasts binding 125I-alpha 1-AT as well as those with membrane-associated alpha 1-AT detectable by immunofluorescence. Co-cultivation of PBL with T cell mitogens leads to the appearance of lymphoblasts with membrane-associated alpha 1-AT while B cell mitogens induce lymphoblasts with a binding capacity for 125I-alpha 1-AT. It could be shown that unstimulated lymphocytes neither have membrane-associated alpha 1-AT nor possess a binding capacity for it. The highest number of lymphoblasts with membrane-associated alpha 1-AT was found in Con A stimulated cultures as revealed by indirect immunofluorescence. By contrast, PHA or PPD stimulated cultures contained much less such cells of lower fluorescence intensity. Co-cultivation of PBL with the B cell mitogens LPS and rabbit-anti-human gammaglobulin-IgG-F(ab')2 induced lymphoblasts with a clear binding capacity for 125I-alpha 1-AT, while dextran-sulphate had no effect. A possible regulative role of alpha 1-AT in the immune response is discussed.

The number of peripheral blood lymphocytes (PBL) rosetting with sheep erythrocytes (SE-rosettes) was significantly decreased by preincubation with both sera containing immune complexes (IC) and prepared antigen-antibody complexes. However, this effect of decrease in rosetting ability of PBL could be completely reversed by a second incubation of the cells for 30 minutes at 37 degrees C in IC-free medium. The immune complexes and antigen-antibody complexes respectively could bind reversibly to T cells via Fc-receptors and subsequently interfere with the T cell receptors for SE by steric hindrance.

It is reported on a 42-year-old book-keeper with the granulomatous variant of the chronic mucocutaneous candidiasis which could be followed up for 28 years. The intensive systemic treatment with nystatin, 5-fluorocytosin and miconazol combined with the subcutaneous injection of transfer-factor and the local application of ointments containing nystatin and clotrimazol did not only lead to the complete clearing of the lesions (4 years without any relapse), but also to the normalization of the T-lymphocyte count and the reconstitution of the formerly negative delayed type skin reactivity to candidin.

The minimal irritation dose (MID) for dithranol in 5% salicylic acid vaseline was determined by open patch testing. From the MID, the irritation dose 50 (ID50) was evaluated. It was 0.057% in 100 controls and 0.046% in 100 psoriatic patients, which is not statistically different due to the great differences between individuals. 20 other patients claimed a dithranol hyperreactivity in their history. Only 13 of them, however, showed a decrease in the MID, which was between 0.01 and 0.02% (means = 0.014%), i.e. outside the confidence interval found in the 100 psoriatic patients. 8 of these 13 reacted with blister formation after exposure to 0.1-0.16% dithranol. An allergic contact dermatitis was excluded as the cause of the hyperreactivity. The tolerance to increasing dithranol concentrations after beginning with the MID up to clearance of the lesions, as well as the predominance of granulocytes as compared to lymphocytes in blisters due to dithranol testing, suggest an irritant inflammatory mechanism. In such hyperreactive cases therapy should be started with the MID established in the open patch test.