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A study was made of the number of T lymphocytes and thier functional activity in 16 patients with pemphigus vulgaris, 5 with involvement of the mucosa, 3 with involvement of the skin and 8 with involvement of both; 2 patients had been suffering from the disease for 7 years, one for one year and the remainder for one to 6 months prior to this investigation. Patients were tested prior to institution of treatment, while receiving initially high doses of steroids and subsequently on maintenance doses. T lymphocyte number was determined by the E rosette technique and their functional activity by a local xenogeneic graft-versus-host reaction (GVHR) developed in our laboratories. Prior to therapy 50% of the patients showed impairment in T cell function, with no improvement during intensive steroid therapy; subsequently, when the disease was under control, there was a clear tendency for function to return to normal. There was no correlation between duration of the disease and immune competence but there appeared to be a partial correlation between the latter and the degree of involvement. Our study indicates that long-term steroid therapy not only benefits the clinical condition of the patient but is associated with an improvement in the immunological status.

Prednisone is a widely used medication in dermatology, particularly in pemphigus patients. Simple objective laboratory methods are suggested for the evaluation of prednisone intake, taking advantage of the similar chemical structure of cortisol and prednisolone. A linear correlation was found between the dose of prednisone, above 40 mg/day, free urinary prednisolone, measured with an radioimmunoassay kit for cortisol that cross-reacts with prednisolone, and 17-hydroxycorticosteroids, measured by Metcalf's method. Approximately 9% of the prednisone administered was recovered as free prednisolone and about 40% as 17-hydroxycorticosteroids. It is suggested that these simple laboratory methods constitute a useful substitute for the more costly, sophisticated methods available at present for determining levels of prednisone and prednisolone.

In two patients with pemphigus vulgaris a rash identical with that seen in disseminated superficial porokeratosis developed on the trunk and extremities during periods in which they were receiving relatively high doses of steroids. Some time after reduction of the daily dose or discontinuance of treatment this rash disappeared. In one patient, however, this rash subsequently recurred twice, both times after reinstitution of treatment because of the reappearance of pemphigus lesions. It is suggested that disseminated superficial porokeratosis should be added to the list of possible skin eruptions developing as the result of the systemic use of corticosteroids at a high dose for a prolonged period of time, although the mechanism of its appearance in these cases is not yet clear.

Immunofluorescent antibodies against the intercellular material of the malpighian layer were detected in the sera of 22 of 24 patients suffering from pemphigus vulgaris. A clear-cut agreement between pretreatment antibody titers and the severity of the clinical manifestations was established only with the extreme values, i.e., patients without antibodies were among the mildest cases while the highest titers were found exclusively among the most severe cases. There was a considerable overlapping of all 3 states of the disease--mild, moderate and severe--in the range of the intermediate titers. On the individual level, 20 of the 24 patients showed a positive correlation between steroid-induced clinical improvement and a fall in the antibody titer. Conversely, in some cases, an exacerbation of the clinical state was associated with a rise in antibody titer. The results of this investigation indicate a positive correlation between the immunological findings and the clinical activity of the disease.

Five patients had a form of pemphigus which in its early stages resembled dermatitis herpetiform, although the immunofluorescent findings were typical of pemphigus. Potassium iodide tests, performed for the first time in such patients, showed positive results in two patients. Follow-ups ranging from 5 to 14 years have shown a benign course with low to absent dosages of steroids.

The association of HLA antigens with pemphigus in Jewish patients of various ethnic origins was investigated. Of 45 Ashkenazi patients, 31 (68.6%) had A26 (p less than 10(-5) and 32 (7175) had BW38 ( less than 10(-5)0. Among the 11 non-Ashkenazi patients, 7 (63.6%) had A26 and only 1 had BW38. The question whether the antigen A26 or BW38 is linked with the disease is discussed. Our findings may suggest that the primary association of pemphigus is with A26. This association may be stronger in Jewish male patients than in females. In view of the recent literature and our results it may be presumed that pemphigus in Jews is associated with antigens of Loci A and DR. No differences between pemphigus vulgaris and pemphigus erythematosus were observed.