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Porphyria cutanea tarda (PCT) is characterized by decreased activity of the enzyme uroporphyrinogen decarboxylase (URO-D) and the accumulation of uro- and heptaporphyrins in the liver. Apart from increased alcohol exposure and certain drugs, PCT is associated with antibodies to the hepatitis C virus (HCV), with its prevalence increasing from Northern (8-10%) to Southern Europe (71 to 91%). Chronic HCV-infection is thus considered to be a major trigger for PCT and PCT is said to be an important extrahepatic manifestation of HCV-infection in predisposed individuals. Iron overload is common in PCT. Iron is an inhibitory co-factor of URO-D activity in hepatocytes. Accordingly, in support of the critical role of iron, the clinical efficacy of iron removal is coupled to an improvement of hepatic URO-D activities. Up to two thirds of Saxon patients with PCT carry the classical hemochromatosis (HFE) mutations (C282Y and/or H63D). HFE genotyping can help to further classify patients with PCT and associated hemochromatosis. Simple or compound heterozygosity of HFE mutations does not affect the therapeutic response to chloroquine in PCT. Since Patients carrying homozygous mutations (C282Y/C282Y) with hemochromatosis and PCT do not respond to chloroquine, phlebotomy should be first-line treatment to remove toxic iron.

Zusammenfassung: Die strahleninduzierte Dermatitis und Mucositis stellen wichtige unerwunschte, nicht immer vermeidbare Nebenwirkungen der onkologischen Strahlentherapie dar. Sie konnen therapielimitierend sein und haben einen negativen Effekt auf die Lebensqualitat der Patienten. Etablierte und evaluierte Verfahren zur Pravention und Therapie werden diskutiert. Grundlegende Behandlungs-ansatze werden in der fruhzeitig gestorten Barrierefunktion der Haut sowie in der immunologisch-vermittelten Entzundungskaskade gesehen. Dabei ist die Hautreinigung und Pflege mit Wasser und Moisturizern sinnvoll solange keine exsudative Dermatitis besteht. Topische Anwendung von Dexpanthenol hat zumindest bei der Mucositis und beim Sicca-Syndrom durch Studien gesicherte therapeutische Wirkungen. Neue Ansatze bestehen im Einsatz von Zytokinen und Wachstumsfaktoren. Summary: Radition-induced dermatitis and mucositis are important unwanted side-effects of oncological radiotherapy. They are not completely avoidable but may be therapy-limiting and have a negative impact on patient's quality of life. Established and evaluated procedures in prevention and treatment are discussed. Major targets are the reconstitution of the early induced skin barrier dysfunction and the immunological pathways of inflammation. In dry desquamation, skin care with showers and moisturizers is helpful. Topical dexpanthenol is approved by studies for at least radiation-induced mucositis and sicca syndrome. New approaches of treatment are the use of cytokines and growth factors.

Dargestellt wird ein Fall einer bei Menschen nicht japanischer Herkunft nur selten beobachteten sterilen eosinophilen Pustulose Ofuji (SEP). Das distinktive histologische Bild mit intraepidermalen eosinophilenreichen Abszessen und eosinophiler Follikulitis wird erganzt durch eine ausgepragte Bluteosinophilie, eine deutliche eosinophile Knochenmarksreaktion und den offenbar erstmalig beobachteten Mundschleimhautbefall mit histologischem Nachweis eosinophiler Spongiose. Mogliche Ubergange zwischen kutanen und internen Hypereosinophilie-Syndromen werden diskutiert. Trotz des zunachst guten Ansprechens der kutanen Veranderungen auf topische Kortikosteroide ist eine Langzeituberwachung hinsichtlich interner Mitbeteiligung notwendig.

Ein wesentliches Ziel der langjahrigen Nachsorge von Melanompatienten ist die Fruherkennung und Behandlung der Tumorprogression. Daher ist die Kenntnis der Erstlokalisation von Metastasen, deren zeitlicher Abfolge und der Risikogruppen fur die Metastasierung von wesentlicher Bedeutung fur die effektive Nachsorge.

An eighty-two year old patient had scabies and transient acantholytic dermatosis (TAD, Grover's disease) at the same time. Persistent itching papules after lindane therapy finally led us to the microscopic diagnosis of TAD. Different trigger mechanisms can apparently induce the typical acantholysis. In addition to solar radiation, tumors and tumor therapy-dependent conditions, cutaneous infections have been implicated reports (Demodex folliculorum, Malassezia furfur) as causes of TAD. This may be the first report about a relationship between TAD and mite infection with Sarcoptes scabiei.

This case report refers to a German patient with sterile eosinophilic pustulosis of the Ofuji type (SEP), which is rarely described in non-Japanese humans. The distinctive histomorphology, with intraepidermal eosinophilic abscesses and eosinophilic folliculitis, is complemented in this patient by marked eosinophilia, evident eosinophilic bone marrow reaction and involvement of the oral mucosa with eosinophilic spongiosis, which is a feature that had not previously been reported. Possible transitions between cutaneous and internal hypereosinophilic syndromes are discussed. Despite the initial good effect of topical corticosteroids on the cutaneous changes, long-term follow-up to check for internal involvement is necessary.

Der nach einem Vorschlag Hoppe-Seylers 1871 verwandte Terminus Porphyrin („Hamatoporphyrin“) entstammt dem griechischen „porphyros“, das bedeutet purpurrot (Gunther 1911; With 1976). 1867 hatte der aus Budingen bei Marburg geburtige Thudichum erstmals das Absorptionsspektrum von Porphyrinen beschrieben (Watson 1978).

The knowledge about the influence of chloroquine phosphate (CQ) on the iron metabolism in porphyria cutanea tarda (PCT) is still insufficient. In a study on 138 PCT patients treated with CQ, we observed decreasing serum iron concentration, transferrin saturation, and hepatic siderosis, as well as an increasing level of transferrin. After a one-year therapy with CQ, the patients showed normal porphyrinuria. Since there were no statistical correlations between the degree of hepatic siderosis and porphyrinuria, and since we repeatedly found remission of the PCT in spite of the continued existence of hepatic siderosis, the removal of iron cannot be regarded as the only principle of action in CQ therapy. In comparison with chloroquine, phlebotomy obviously has--even with regard to the iron deposits--no decisive advantages.

Relations between porphyrias (porphyria cutanea tarda, variegate porphyria) and HLA- or protease inhibitor (Pi-) system were repeatedly found or supposed. Corresponding investigations do not exist for erythropoietic protoporphyria (EP), for which an autosomal dominant mode of inheritance with highly varying expressiveness (additional genes?) is being discussed. Three families with five EP-patients were examined for possible relations between the manifestations of this disease (skin - and liver changes) and the above-mentioned genetic markers. It was remarkable that three of the patients had the HLA A 3, but nobody of the obvious gene-carriers of this disease without clinical manifestations. On the other hand, two ill couples of siblings were genetically different as to HLA A 3. One couple with liver morphological changes each were different as Pi M-carrier and Pi MS-carrier respectively. Thus, relations between the disease and the genetic markers examined could not be proved.

Seventy-three patients with porphyria cutanea tarda (PCT) were treated with chloroquine phosphate, which was administered in doses of 250 mg twice a week for more than 1 year on average. Of these patient, 49% did not have any associated ("inducing") factors. After treatment, serum transaminases and gamma-glutamyltransferase (gamma-GT) always returned to normal values. However, gamma-GT did not reach the normal range in the group without associated factors ("idiopathic group"). Of the 66 patients studied, 25 achieved an approximately physiological distribution of the pattern of urinary porphyrin fractions. Control biopsies were performed in 66 patients. Of them, 48 patients showed an improvement in liver damage. A statistically significant correlation was observed between a lasting increase in urinary prophyrin content and unchanged pathological liver morphology. A pathogenesis concept for PCT is discussed as a disease between inheritance (genetic enzyme defect) and environment ("inducing factors"). Even in the "idiopathic group" the improvement of pathological liver changes after therapeutic removal of the pathological prophyrin content in the liver cells shows that porphyrins could also cause liver damage.

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.

The development of modern medicine has very often pushed older methods of treatment to the background. It will be tried to represent the importance of iontophoresis as a special form of electrotherapy for dermatology according to studies of literature and own observations. Iontophoresis is for most of the 40 to 50 dermatologic indications named in literature without any importance for the present. This is also to be said for their diagnostic and experimental application. Because of the synergistic effect of the galvanic current and the medicament sluiced in, iontophoresis is said to be recommended following literature, for the treatment of the rest of therapeutically only with difficulty to be influenced idiopathic hyperhidrosis manuum et pedum, constitutional hypertrichosis, the lymphedema and also the induratio penis plastica and of thrombophlebitis. It will be reported about own experiences in the treatment of lymphedema.

Our investigations in 134 patients showed corresponding to literature porphyria cutanea tarda (PCT) diagnosed by biochemical methods not to be a paraneoplastic dermatosis (but one possible exception acquainted). Relations between PCT and extrahepatic non-porphyrin producing tumours are improbable. Nevertheless but extremely seldom an irregular urinary porphyrin excretion associated with cutaneous changes of hepatic porphyria should lead to the presumption of a porphyrin producing hepatoma. PCT lasting for decades apparently presents a higher frequency of hepatocellular carcinoma in patients suffering from liver cirrhosis than in cirrhotics without PCT. It is supposed that this possible progredience of liver disease in PCT into hepatocellular carcinoma may be prevented by chloroquine phosphate therapy.

All the 18 patients with acrodermatitis chronica atrophicans (ACA) showed the specific borrelia antibody at the indirect immunofluorescence assay. The patients exhibited a large spectrum of arthritic and neurologic complaints besides the typical cutaneous symptoms. Changes of proteins, of liver-typical parameters and of the electrocardiogram are also assigned to the Lyme-borreliosis. The penicillin therapy - three times accompanied by a Jarisch-Herxheimer-reaction - led to regression of the clinical changes. The secessions of borrelia-antibody-titers happens only very delayed. That is why it can not be used for the direct therapy control. The increased documented polysymptomatic of Lyme-borreliosis requires a wider knowledge and the country-wide possibility of specific diagnostic.

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.

Familiar occurrence of porphyria cutanea tarda (P. C. T.) has led to search for genetic markers of this disease. One of the three contradictory studies in literature showed significant increased frequencies of HLA-A3 and -B7 in the P. C. T. These results are important for the hypothesis that a disorder of iron metabolism linked to the HLA system (HLA-A3) supports a manifestations of the disease. Our investigations don't support generally this hypothesis. In 90 P. C. T.-patients significantly higher frequencies of any HLA-A, -B and -C-locus were not to be found. But we found a statistic guaranteed higher frequency of HLA-A9 in the subgroup (n = 34) of alcoholic induced P. C. T. An association between HLA-A9 and liver diseases is reported somewhere else. The higher percentage of HLA-A3 incidence in this group was not significant. Significant higher frequency of HLA-A9 possibly refers to liver diseases by P. C. T. HLA-A3--association is imagineable with iron-over loaded disorder in this group.