Deficient Phagocytic Function in Papillon-Lefèvre Syndrome

The function of microphages has been studied &lt;i&gt;in vitro &lt;/i&gt;in a 5 year-old girl with Papillon-Lefèvre syndrome. The results showed a weakness in chemotactic activity, a defect of intracellular killing of &lt;i&gt;Staphylococcus aureus, &lt;/i&gt;and an almost normal phagocytosis but decreased intracellular killing of &lt;i&gt;Candida aibicans &lt;/i&gt;by the microphages.</jats:p

Recurrent Chronic Pyoderma with Cellular Immunodeficiency

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.</jats:p

Lymphocytotoxicity for Oral Epithelial Cells in Behçet’s Disease

Apart from nonspecifically altered laboratory data, enhanced chemotaxis of polymorphonuclear leukocytes to chemotactic stimuli, HLA-B5 and circulating cytotoxic lymphocytes for oral epithelial cells were demonstrated in a 21-year-old patient suffering from Behçet’s disease (BD). Pathological liver function became indicative of concomitant hepatic involvement. The pathogenetic role cytotoxic lymphocytes have on the oral mucosa in BD is discussed.</jats:p

Skin Window Examination According to Rebuck and Cutaneous Pathergy Tests in Patients with Behçet’s Disease

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 vene puncture. 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.</jats:p

Immune Phenomena in Patients with Pustular Bacterid

In 20 patients with histologically confirmed pustular bacterid the immuno-logical 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 &lt;i&gt;Candida albicans&lt;/i&gt; cells was found to be slightly impaired (p ≤ 0.01 and 0.05, respectively). Killing of &lt;i&gt;C. albicans&lt;/i&gt; cells by polymorphonuclear leukocytes was strikingly impaired (p ≤ 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.</jats:p