[Porphyria cutanea tarda and lupus erythematosus]. / Porphyria cutanea tarda und Lupus erythematodes.

We observed three patients with lupus erythematosus and porphyria cutanea tarda manifesting itself simultaneously or subsequently. The possible coincidence of the two diseases might be the consequence of immunological communities indicated on the assumption of a genetically coined hepatic uroporphyrinogen decarboxylase defect. The treatment of the lupus erythematosus can be performed according to corresponding observations of the course according to general acknowledged principles including the administration of cytostatic drugs, if the chloroquine phosphate therapy which is clearing up the porphyria cutanea tarda is beginning with low dosages. The fluorescence of the fresh kidney bioptate under long-wave UV-light in porphyria cutanea tarda is referred to for the first time.

[Pregnancy in patients with visceral lupus erythematosus, with special reference to kidney involvement]. / Schwangerschaft bei Patientinnen mit Lupus erythematodes visceralis unter besonderer Berücksichtigung der Nierenbeteiligung.

Of 44 female patients with lupus erythematosus visceralis after and with manifestation of the disease, respectively, in eight women ten pregnancies developed, out of them three viable births between the 33rd and 40th week of pregnancy, one stillbirth in the 37th week of pregnancy, one miscarriage in the IIIrd month of pregnancy and five interruptions of pregnancy. It is reported on the course of the disease and pregnancy of five women, taking into particular consideration the lupus nephritis. With the help of the literature and on the basis of own experiences course of the lupus erythematosus visceralis and lupus nephritis during pregnancy, the influence of the lupus erythematosus visceralis on pregnancy and development of the child, peculiarities of the treatment of lupus erythematosus visceralis during pregnancy as well as questions of prevention and interruption of pregnancy are discussed. Finally is tried to draw universal conclusions.

[Protoporphyrinemia and iron metabolism in hemodialysis patients]. / Protoporphyrinämie und Eisenstoffwechsel bei Hämodialysepatienten.

In 24 patients undergoing haemodialysis, two of them with pseudoporphyria, the total erythrocytic protoporphyrin and in four patients separately the zinc protoporphyrin were investigated. 20 patients showed an increase of the erythrocytic protoporphyrin of low to moderate degree, which only partly is to be ascribed to the zinc protoporphyrin. Since, as a rule, there is no iron deficit in haemodialysis, a secondary damage of the ferrochelatase, which physiologically by administration of iron leads to haem (ferroprotoporphyrin), is assumed as cause of the increase of erythrocytic protoporphyrin. Apart from the toxic lesion in particular a deficiency of the ferrochelatase coenzyme pyridoxal phosphate can be taken into consideration. Diagnostic and therapeutic investigations carrying on are proposed. The erythrocytic protoporphyrin does not only contribute to the pseudoporphyria in haemodialysis.

[Follow-up of Wegener's granulomatosis with kidney involvement]. / Verlaufsbeobachtung bei Wegenerscher Granulomatose mit Nierenbeteiligung.

It is reported on long-term courses in five patients with Wegener's granulomatosis. Under a treatment with glucocorticoids and immunosuppressive agents lasting several years in all cases a remission developed. In these cases the participation of the kidneys is fateful, whereby from the very beginning a large proportion of sclerosing glomeruli lead to a greater healing of the defect. While the troubles of the patients under the apparently at present unspecific effect of high doses of prednisolone disappear within a few weeks, the onset of the remission lasts at least nine months. It is tried to form criteria which indicate a remission. The treatment with a low maintenance dose for at least one year after the remission seems to be justified in consideration of the frequency of recidivations.

[Porphyria cutanea tarda, pseudoporphyria and hemodialysis of chronic renal failure patients]. / Porphyria cutanea tarda, Pseudoporphyrie und Hämodialyse chronisch Niereninsuffizienter.

With the help of the observation of a bullous dermatosis in a 52-year-old male with cystic kidneys, terminal renal insufficiency and chronic hemodialytic treatment the authors adopt a definite attitude to the pathogenesis of this coincidence frequently reported on. Since the determination of porphyrin in the urine does not reflect the plasma porphyrin content in the combination, after sonography by means of a liver blind puncture and missing fluorescence of the liver bioptate in the long-wave ultraviolet light a porphyria cutanea tarda could be excluded. The bullous dermatosis observed is to be ascribed to the pseudoporphyrias (idiopathically; phototoxically by drugs). Renal insufficiency and long-term haemodialysis apparently on the one hand further the manifestation of genetically conditioned diseases of porphyria cutanea tarda, on the other hand there are references that the chronic renal insufficiency itself leads to the disturbance of the hemobiosynthesis at the level of the uroporphyrinogen decarboxylase and that plasma porphyrins bound to highly molecular proteins are not capable to dialysis. A classification of skin changes accompanied by vesicles in patients with chronic renal insufficiency who were haemodialysed is proposed.

[Wegener's granulomatosis with pronounced kidney involvement]. / Wegenersche Granulomatose mit ausgeprägter Nierenbeteiligung.

It is reported on the course of histologically ascertained Wegeners' granulomatosis in 4 patients. After initial affection of the upper respiratory tract in form of necrotizing and granulomatous inflammations in all cases a generalisation of the disease with above all early and severe participation of the kidneys developed. Maximum acceleration of sedimentation, C-reactive protein, anaemia, leucocytosis, eosinophilia, thrombocytosis, enlargement of the number of alpha 2-globulins, increase of creatinine, proteinuria, erythrocyturia and leucocyturia are the most frequent pathological laboratory findings, whereas LE-cells, ANF, rheumatoid factor and decrease of the complement never could be proved. By reason of a pathogenic immunoreaction a combined glucocorticoid therapy and immunosuppressive therapy with prednisolone and cyclophosphamide may favourably influence the course of the disease which is otherwise prognostically infaust.