Gerhard Tappeiner

Systemic lupus erythematosus in hereditary deficiency of the fourth component of complement

Three patients from two families with complete hereditary deficiency of the fourth component of complement (C4) and systemic lupus erythematosus are described. The syndrome presented by these patients is characterized by early onset in life; exquisite sensitivity to sunlight and to cold exposure, the latter resulting Raynauds phenomenon; and skin lesions involving not only exposed areas of the body but also palms and soles and presenting as butterfly rashes, maculopapular eruptions, and lesions similar to those of chronic discoid lupus erythematosus, with marked scaling, atrophy, and scarring. Lupus erythematosus (LE) cell tests were negative and antinuclear antibody (ANA) titers low or negative. The male patient of our series died at the age of 31/2 years from septicemia, whereas the two girls, aged 18 and 11 years, respectively, were alive at the time of writing. The C4-deficient gene is associated with HLA-Aw32, Bw38, and Bf S in one family and with HLA-A30, B18, DR7, and Bf S1 in the other family; the latter is the second family in which this HLA haplotype has been found to be associated with hereditary C4 deficiency.

The carcinoembryonic antigen (CEA) in carcinomata of the stomach

The localization of CEA in gastric carcinomata of different histological types was studied by indirect immunofluorescence. In well differentiated adenocarcinomata CEA was present on the surface of the tumor cells. CEA was absent in anaplastic cancers. Signet ring cells contained both CEA and mucus in their cytoplasm; this may indicate that signet ring cells are fairly well-differentiated tumor cells. In areas with intestinal metaplasia, CEA was detected on the luminal surface of the cells similar to a malignant tumor.

Pyoderma Gangrenosum in a Patient With Seronegative Rheumatoid Arthritis During Therapy With Adalimumab : Toxic Effects of Adalimumab or Failure of Adalimumab to Prevent the Onset of This Phenomenon?

Comment. In 1997, Langley et al presented a case, reviewed the literature, and proposed a classification system for this rare condition. Since then, other cases have been reported. In the analysis, eruptive milia were noted to occur in 3 settings: (1) spontaneously with no known cause or association; (2) in familial syndromes with autosomal dominant transmission; or (3) as a component of genodermatosis. For example, it has been described in a 3-year-old child who presented with palmoplantar keratoderma, solitary nodular calcinosis, and numerous milia during the first few months of life. Milia in that case cleared spontaneously after several months. Four generations in this family had similar constellations of findings. The case presented herein, like that reported by Langely et al, fits into the first group. Rapid clearing with topical tretinoin cream was demonstrated, which suggests superficiality more responsive to a desquamative effect.

Influence of the antigenicity of target cells on the antibody-mediated cytotoxicity of nonsensitized lymphocytes

The effect of various antigen concentrations on the surface of target cells on IgG-mediated cytotoxicity of nonsensitized lymphocytes was studied using benzylpenicilloyl (BPO)-coated chicken erythrocytes as target cells, anti-BPO rabbit IgG, and human lymphocytes as effector cells. The number of antigenic sites on the target cell was calculated. The results indicate that a critical density of antigenic sites and consequently a close association of the Fc-parts of the antibody molecules is necessary for the induction of cytotoxicity.

Vergleichende Untersuchungen über Auftreten und Lokalisation des carcinoembryonalen Antigens (CEA) und eines normalen perchlorsäureextrahierbaren Dickdarmschleimhaut-Antigens (NC) in Carcinomen und Polypen des Dickdarmes

Occurrence and localization of the carcinoembryonic antigen (CEA) and of a perchloric acid-soluble antigen of normal colonic mucosa (NC) were compared in polyps and carcinomas of the colon using the indirect immunfluorescence technique. NC is located in the secretory part of the goblet cells and diffusely distributed in the mucus. Therefore, it can be considered as a marker of large-bowel mucus. NC is found in all mucus-producing tumors regardless of their degree of differentiation in intra- and extracellular mucus deposits. Tumor cells which closely resemble normal goblet cells produce and secrete NC. With respect to their NC content, there is no immunologically detectable qualitative difference between the secretions of normal and of neoplastic large bowel mucosa. It is clearly shown that production and secretion of CEA is independent of mucus production and secretion. In contrast to earlier results, CEA was found in polyps and in normal mucosa surrounding the carcinoma, confirming the observations of Burtin et al. (1972a). Auftreten und Lokalisation eines aus normaler Colonmucosa mit Perchlorsäure extrahierbaren Antigens (NC) in benignen und malignen Colontumoren wurden mittels indirekter Immunfluorescenz untersucht und mit dem carcinoembryonalen Antigen (CEA) verglichen. In der normalen Dickdarmschleimhaut trat das NC intracellulär im sekretorischen Anteil der Becherzellen, extracellulär diffus im Schleim auf. Es kann somit als Schleimantigen angesehen werden. In Tumoren fand es sich unabhängig vom Differenzierungsgrad immer im intra- und extracellulären Schleim. In hochdifferenzierten Tumoren wurde es reichlich in Zellen gefunden, die normalen Becherzellen ähnelten. Diese Zellen sezernierten NC ins Lumen. Bezüglich des NC verhält sich der Schleim verschiedener Tumoren und der normalen Schleimhaut gleich. Es zeigt sich deutlich, daß die vom Differenzierungsgrad des Tumors abhängige CEA-Produktion von der Schleimproduktion und vom Auftreten des NC unabhängig ist. Entgegen früheren Befunden konnte CEA auch in Polypen und in der normalen Schleimhaut in der Umgebung von Carcinomen nachgewiesen werden, womit die Befunde von Burtin et al. (1972a) bestätigt werden.

The influence of 2-mercaptoethanol (2-ME) treatment of IgG antibody on its ability to induce cytotoxicity in nonsensitized lymphocytes

Abstract The effect of 2-ME treatment of IgG on its ability to induce cytotoxicity in non-committed lymphocytes was studied. An IgG fraction of a rabbit anti-BPO serum was tested with BPO-coated chicken erythrocytes as target cells and human peripheral blood lymphocytes as effector cells. 2-Mercaptoethanol alters the properties of the Fc fragment of the IgG antibody molecule by cleaving inter-H-chain-disulfide bonds. Treatment of the antibodies with 2-ME resulted in a significant reduction of their capacity to induce cytotoxicity of lymphocytes against the respective antigen-antibody complex. 2-Mercaptoethanol treatment may prevent conformational changes of the Fc part of the antibody molecule essential for its interaction with the Fc receptor on the effector lymphocyte.