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A cellular immunodeficiency was revealed in a 39-year-old female patient suffering from Werner's syndrome. Relevant findings were negative intracutaneous tests to candidin, trichophytin, tuberculin and bacterial antigens, reduced lymphocyte response to PHA, and impaired intracellular killing of Candida albicans by granulocytes. The inclination of patients suffering from Werner's syndrome to develop neoplasms may be related to this immunodeficiency.

In 20 patients with histologically confirmed pustular bacterid of immunological status was checked including lymphocyte and granulocyte components of the cellular immune system as well as HLA typing. For comparison the same investigations were carried out in 20 healthy controls. In contrast to the controls, in the group of patients the chemotactic activity of polymorphonuclear leukocytes as well as their ability to engulf either vital or heat-inactivated Candida albicans cells was found to be slightly impaired (p less than or equal to 0.01 and 0.05, respectively). Killing of C. albicans cells by polymorphonuclear leukocytes were strikingly impaired (p less than or equal to 0.001). NADPH-dependent oxidase activity, however, was found to be normal. Impairment of chemotaxis and killing activity could not be substantiated in AB pool serum, thus a serum-dependent disorder of granulocyte function has to be assumed. Lymphocyte response to phytohemagglutinin was reduced in all patients, whereas intracutaneous test reactivity to microbial antigens was normal. T and B cell counts in peripheral blood were within normal range. HLA typing revealed a significantly increased prevalence rate of HLA-B14 as compared with the control group and other patients.

The function of microphages has been studied in vitro in a 5 year-old girl with Papillon-Lefèvre syndrome. The results showed a weakness in chemotatic activity, a defect of intracellular killing of Staphylococcus aureus, and an almost normal phagocytosis but decreased intracellular killing of Candida albicans by the microphages.

Comparative in vivo examinations of skin (cutaneous pathergy test, skin window technique over 24 h according to Rebuck) were carried out in 12 patients with Behçet's disease (BD) and in 13 healthy volunteers serving as controls. 10 out of 12 patients at active phases of BD showed a positive papular (-pustular) skin reactivity to needle prick or venepuncture. These 10 patients simultaneously revealed an increased chemotactic activity of polymorphonuclear leukocytes (PMNL) in vitro. In the controls all pathergy tests were negative and chemotaxis of PMNL was within normal range. The proportion of PMNL initially coming up to 100% in the skin window preparations, very rapidly decreased in patients with BD as compared to a distinctly slower drop in the controls. Conversely to the number of PMNL, the skin window cellular pattern was built up by monophagocytic cells replacing the PMNL. The phenomenon of 'rapid depletion of PMNL', from skin window can be considered an additional hint on the functional disturbance of the PMNL involved in the pathogenesis of BD.

In a 21-year-old male patient afflicted with widespread recurrent pyoderma resistent to antimicrobial therapy since 3 years, a deficient state of cellular immunity including dysfunctions of both microphages and T lymphocytes was disclosed. After 6 weeks of systemic treatment with levamisole all lesions cleared completely, whereas appropriate long-term antibiotic therapy previously administered had failed to succeed. The immunological parameters, as far as being reexamined, also returned to normal levels during levamisole therapy.

Twenty-seven patients suffering from long-standing, severe, recurrent herpes simplex (14 labial and 13 genital) who had not responded adequately to prior therapy were recruited for this open, monitored study. They were treated with thymopentin 50 mg subcutaneously three times weekly for a period of 6 weeks. Clinical controls were performed once a week and then again 6 weeks after cessation of therapy; laboratory investigations were done at time points 0, 3, and 6 weeks. Additionally, information was collected with regard to the clinical course during the following year. Thirteen of 14 patients with labial infection and 10 of the 13 with genital herpes improved markedly (p less than 0.05) as shown by decrease in the relapse rate of at least 50%, shorter relapse episodes, and improvement of symptoms such as pain and itching. Fourteen of these 27 patients experienced no relapse for a period longer than 4 months after cessation of therapy. These favorable results were paralleled by a statistically significant increase in the T cell helper/suppressor ratio. This finding indicates that thymopentin acts as an immunodomulator; it is assumed that the activation of T helper cells induces-presumable via interleukin 2-the proliferation of cytotoxic T lymphocytes and natural killer cells which play a major role in the natural immune defense. No serious side effects of thymopentin were recorded.

The present randomized, placebo-controlled double-blind multicenter study included a population of 36 subjects with frequent recurrences (at least once a month) of herpes labialis. Most of the patients had failed to respond adequately to previous treatment with other therapeutic tools, including acyclovir. Either 50 mg of thymopentin or of placebo was administered 3 times a week, by the subcutaneous route, for 6 weeks. Subsequently, the patients were observed for nearly 6 months on the average. The results achieved with thymopentin for the individual parameters were significantly superior to those obtained with placebo; thus significant improvement was seen in patients on thymopentin in the duration of the longest symptomfree period (prolonged from 2.1 weeks to 20.9 weeks, p = 0.000), in the number of relapses (reduced from 1.6 to 0.4 episodes/month, p = 0.001), and in the total duration of herpes symptoms per month (shortened from 2.0 to 0.3 weeks, p = 0.000). Placebo treatment also resulted in considerable improvement (p less than 0.05 or 0.01), but was significantly inferior to the improvement obtained with thymopentin. The longest symptomfree period in the placebo group was prolonged from 2.4 to 11.2 weeks. The number of relapses per month was reduced from 1.4 to 0.8, and the total duration of herpes symptoms per month from 2 to 0.9 weeks. The results of intergroup analyses, in which the observed parameters and the improvement achieved in either group were compared, significantly favored thymopentin treatment. The effect of thymopentin was in all but one parameters superior to that of placebo and highly significant (p less than 0.01).

This paper presents longitudinal clinical and immunological findings in a patient with a life-long history of pluriorificial pyoderma vegetans, a disease characterized by a distinct immunodeficiency of T lymphocytes. After treatment with thymopentin, 50 mg s.c. three times weekly for 12 weeks, the number of T lymphocytes in peripheral blood normalized, while other impaired immunological functions failed to improve, however. The most impressive observation was the significant clinical improvement of the patient's condition.