N. Simon

Transfusion therapy for erythropoietic protoporphyria

Five patients with erythropoietic protoporphyria, in whom beta‐carotene treatment had proved unsuccessful, were treated on 5 or 6 occasions with washed, packed red blood cell transfusions. Following the transfusions, the photosensitivity and the protoporphyrin levels in the red blood cells decreased considerably. This treatment may be useful in reducing the high protoporphyrin level in patients with a rapidly deteriorating hepatic function.

Hepato-erythropoietic porphyria presenting as scleroderma and acrosclerosis in a sibling pair.

Clinical and biochemical data are reported on a sibling pair with clinical and biochemical features of both erythropoietic protoporphyria and hepatic cutaneous porphyria. After many years of photosensitivity the clinical picture resembles systemic sclerosis.

Impairment of axon reflex vasodilatation after herpes zoster

Examination of the neurogenic inflammatory response of the skin renders direct information on the functional state of the chemosensitive sensory nerve endings involved in neurogenic vasodilatation of skin vessels. Using a recently developed diagnostic test, the present study furnishes experimental evidence on the impairment of axon reflex vasodilatation following herpes zoster. The possible mechanisms by which herpes zoster may effect the vascular reactions of the skin are briefly discussed.

Lymphocyte transformation test in detection of drug hypersensitivity.

Four thousand, three hundred and ninety‐two lymphocyte transformation tests with seventy‐nine different drugs were performed in 1723 patients because of the clinical suspicion of drug hypersensitivity. The optimal stimulating concentration and the lowest cytotoxic concentration were determined for every drug, and transformation tests were carried out only with those drugs whose optimal stimulating concentration was lower than its lowest cytotoxic concentration.

Autoimmune anti‐T cell antibodies in the sera of patients with mycosis fungoides

The serum of persons with mycosis fungoides contains IgG antibody believed to be specific for T cells, similar to the auto-antibodies reported in patients with systemic lupus erythematosus, Hodgkins disease and sarcoidosis. The antibody is present during the active, and disappears during the inactive, phase of the disease.

Porphyrinstoffwechsel-und klinisch-laboratoriumsmäßige Untersuchungen in Modellversuchen und an Patienten mit Porphyria cutanea tarda

Summary1.In Hexachlorobenzol (HCB) poisoned rats both the DALA synthetase activity of the liver and the total amount of the porphyrine excreted in the urine are simultaneously increased.2.The activity of the DALA synthetase in the liver continues to increase for several months even after the HCB administration has been abolished.3.The distribution pattern of the porphyrine fractions excreted in the urine of the HCB-poisoned rats resembles to a great extent that of patients suffering from PCT.4.The porphyrines containing in sequence the 8, 7 and 6 carboxyl groups are stored in the liver and kidneys.5.Transaminases and A/G quotient in the animals poisoned with HCB as well as in the patients suffering from PCT showed similar pathologic values.6.In the poisoned rats and in the PCT patients the amount of the DALA excreted in the urine was only slightly increased.Zusammenfassung1.Bei mit Hexachlorbenzol (HBC) vergifteten Ratten war sowohl die DALA-Synthetaseaktivität in der Leber als auch die mit dem Harn ausgeschiedene Gesamtporphyrinmenge parallel weigehend erhöht.2.Die DALA-Synthetaseaktivität in der Leber zeight auch dann noch Monate hindurch einen Anstieg, wenn die HCB-Behandlung der Tiere schon abgesetzt ist.3.Die Verteilung der im Harn der HCB-vergiftenten Ratten ausgeschiedenen Porphyrinfraktionen stimmt weitgehend mit der bei PCT-Kranken überein.4.In der Leber und Niere werden der Reihe nach die 8, 7 und 6 Carboxylgruppen enthaltenden Porphyrine gespeichert.5.Die Gestaltung der Transaminasen und des A/G-Quotienten zeigten bei HCB-vergifteten Tieren, wie auch bei den PCT-Kranken gleichermaßen pathologische Werte.6.Die mit dem Harn entleerte DALA-Menge wurde sowohl bei den vergifteten Ratten, als auch bei den PCT-Kranken als nur mäßig erhöht befunden.

Disseminated Atypical Granuloma Annulare

Generalized granuloma annulare commonly occurs in older adults. Atypical (perforating) forms are well known, but treatment can sometimes be complicated. A case of atypical generalized granuloma annulare successfully treated with PUVA is reported. The histology and immunohistology of the skin, histopathologic, immunohistopathologic and electronmicroscopic studies of the liver, immunoserology and laboratory investigations are detailed. The most important findings were as follows; diabetic glucose tolerance test, typical histology of the skin disease, steatosis and non‐specific reactive hepatitis, focal Kupffer cell proliferation, Kupffer cell autoantibodies in the liver and slightly enhanced liver functions. The hepatic lesions observed and latent diabetes mellitus are considered to be correlated with the generalized dermal symptoms.

Untersuchung der cellulären Immunität mit Hilfe eines Leukocytenmigrations-Testes bei mit verschiedenen Antistreptolisyntitern einhergehenden Dermatosen

SummaryEffect of Streptolysin O on leukocyte migration has been examined in cases of 27 patients suffering from various dermatoses. Determination of the optimal concentration of this antigen inhibiting cell migration most effectively, has been carried out. 0.2 IU/ml Streptolysin O, except for two cases, markedly inhibited proliferation of leukocytes. No relation has been found between the degree of migration inhibition, that is the degree of cellular immunity, and antistreptolysin titre.ZusammenfassungEs wurden an 27 Patienten mit verschiedenen Dermatosen die vom Streptolysin O auf die Leukocytenmigration entfaltete Wirkung untersucht und jene optimale Konzentration dieses Antigens bestimmt, welche die Zellmigration am wirksamsten hemmt. Die Dosis 0,2 IE/ml Streptolysin O hatte — abgesehen von 2 Fällen — eine ausgesprochene Hemmung der Leukocytenproliferation zur Folge. Ein Zusammenhang zwischen dem Inhibitionsgrad der Migration, d.h. dem Ausmaß der cellulären Immunität, und dem Antistreptolysintiter wurde nicht gefunden.

Suppressor activity of peripheral blood mononuclear cells in patients with chronic discoid lupus erythematosus

SummarySignificantly increased suppressor activity of peripheral blood mononuclear cells was demonstrated in patients suffering from chronic discoid lupus erythematosus.ZusammenfassungDie signifikant erhöhte Suppressoraktivität der mononukleären Zellen des peripherischen Blutes wurde bei Patienten mit chronischdiscoidem Lupus erythemathosus demonstriert.

Proportions of Mouse Erythrocyte Rosette-Forming Lymphocytes, Immunoglobulin-Bearing Cells and E Rosettes in Patients with Lymphoproliferative Diseases

The proportions of mouse erythrocyte rosette-forming (MERF) lymphocytes, immunoglobulin (Ig)-bearing cells and E rosettes were examined in the peripheral blood of patients with lymphoproliferative diseases. The proportion of MERF lymphocytes was considerably increased in chronic lymphocytic leukaemia. The correlation between the proportions of MERF and Ig-bearing cells, characteristic of healthy individuals, is not observed in the lymphoproliferative diseases. It is suggested that in a part of the patients with these diseases, the study of the proportion of MERF cells provides more accurate information on the proportion of lymphocytes of bursal origin than does counting of the Ig-bearing cells.