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  • 1
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Experimental dermatology 4 (1995), S. 0 
    ISSN: 1600-0625
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract We measured the concentrations of total porphyrins and their metabolites (uro-, hepta-, hexa-, penta-, copro- and protoporphyrin) in various human tissues: liver, erythrocytes, skin, adipose tissue, and mammary gland. The porphyrin concentrations varied within minor limits, e.g., 3.1 ±2.3 nmol porphyrins/g liver and 0.50±0.10 nmol/g erythrocytes. No significant differences were detectable in other tissues in comparison with liver. In all tissues, the predominant metabolite was protoporphyrin, followed by coproporphyrin, whereas only low concentrations of higher carboxylatcd porphyrins such as uroporphyrin were detectable. It is concluded that porphyrin metabolism and its regulation is similar in all human tissues, perhaps with some small differences in the erythrocytes.
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Archives of dermatological research 288 (1996), S. 517-521 
    ISSN: 1432-069X
    Keywords: Key words Photodynamic therapy ; 5-Aminolevulinic ; acid ; Side effects
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Photodynamic therapy (PDT) with topically applied 5-aminolaevulinic acid (5-ALA) is increasingly used for treating tumours. The efficacy of topical PDT is limited to superficial and initial tumours. The topically applied doses of 5-ALA vary from 0.02 to 7.0 g per session according to the type of lesion. There are no studies on the influence of topically applied 5-ALA on the systemic accumulation of porphyrins or porphyrin precursors. A group of 20 patients with actinic keratoses (AK) and basal cell carcinomas (BCC) were treated by topical PDT with 5-ALA. Prior to and 6 and 24 h after PDT, 5-ALA and total porphyrin concentrations were determined in red blood cells and plasma, respectively. In addition, before and after 5-ALA treatment, 24-h urine samples were collected and porphyrins and porphyrin precursors were measured. There was no significant alteration in porphyrin metabolism. In some patients, a slight but insignificant increase in erythrocyte and plasma porphyrins was found 6 h after 5-ALA PDT. This investigation confirms clearly the safety of this treatment modality and demonstrates that 5-ALA application (up to 7 g) in the course of PDT has no influence on the concentrations of porphyrins and porphyrin precursors measured in various compartments.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1173
    Keywords: Schlüsselwörter Pyoderma gangraenosum ; Cyclosporin A ; Key words Pyoderma gangraenosum ; Cyclosporine A
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Pyoderma gangraenosum can cause great therapeutic problems. High dosed corticosteroids are the treatment of choice. However, recalcitrant pyoderma gangraenosum or side effects from corticosteroid treatment may require therapeutic alternatives. Pyoderma gangraenosum responds excellently to treatment with cyclosporine A. Because of side effects and drug interactions, the patients must be selected and carefully and closely monitored. Six patients with pyoderma gangraenosum, unresponsive to various topical and systemic therapies, were treated with oral cyclosporine A at mean daily doses of approximately 3 mg/kg. Marked improvement of the skin lesions and complete healing occurred in all patients over a period of 3–6 months. Only one patient suffered a relapse after discontinuation of the treatment. No severe irreversible side effects occurred. The results show that low-dose cyclosporine A treatment can be considered a first-line treatment of pyoderma gangraenosum.
    Notes: Zusammenfassung Das Pyoderma gangraenosum bereitet oft große therapeutische Probleme. Behandlung der Wahl ist eine hochdosierte Glukokortikoidtherapie, die häufig zur Abheilung führt. Therapierefraktäre Fälle oder schwere Steroidnebenwirkungen erfordern jedoch therapeutische Alternativen. Das Pyoderma gangraenosum gehört zu den Dermatosen, die hervorragend auf Cyclosporin A ansprechen. Nebenwirkungen und die Interaktionen mit anderen Medikamenten erfordern eine sorgfältige Auswahl und Überwachung der Patienten. Die Ergebnisse der Behandlung von 6 Patienten mit therapierefraktärem Pyoderma gangraenosum werden dargestellt. Alle Patienten sprachen innerhalb eines Zeitraums von 3–6 Monaten gut auf die Therapie an. Eine Cyclosporin-A-Dosis von 3 mg/kg KG oder weniger war bei allen Patienten ausreichend. Lediglich bei einer Patientin kam es nach Absetzen des Cyclosporin A zum Rezidiv. Schwere irreversible Nebenwirkungen wurden nicht beobachtet. Unsere Ergebnisse zeigen, daß niedrig dosiertes Cyclosporin A als Therapie der ersten Wahl beim Pyoderma gangraenosum in Erwägung gezogen werden sollte.
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  • 4
    ISSN: 1432-1173
    Keywords: Schlüsselwörter Rumpfhautbasaliom ; Photodynamische Therapie ; Photodynamische Diagnostik ; δ-Aminolävulinsäure ; Mamillenplastik ; Key words Superficial erythematous basal cell carcinoma ; Photodynamic therapy ; δ-Aminolevulinic acid ; Photodynamic diagnosis ; Nipple plastic
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary We treated a large superficial basal cell carcinoma (ca. 10×6 cm) on the right breast in a 48-year old woman with photodynamic terapy (PDT). Fractionated PDT was performed by topical application of δ-aminolevulinic acid (δ-ALA, 20%) with subsequent red light (570–750 nm; 180 J/cm2) in three sessions. Nearly total remission of the tumor resulted; however, a few residual neoplastic islands partly infiltrating the nipple-areola complex could be detected by photodynamic diagnosis (PDD). These fluorescent areas were marked, excised, and the defect was closed by a rotation advancement flap. Total excision of the tumor was verified histologically. By combining PDT and surgery, this large tumor was treated with excellent cosmetic results. This case demonstrates the efficiency of topical PDT with adjunctive plastic surgery controlled by PDD even in large tumors.
    Notes: Zusammenfassung Es wird die Behandlung eines großflächigen Basalioms (ca. 10×6 cm) an der rechten Mamma einer 48jährigen Patientin dargestellt. Aufgrund der Histologie eines oberflächlichen Rumpfhautbasalioms entschlossen wir uns, den Tumor mit der photodynamischen Therapie (PDT) zu behandeln. Hierzu wurden δ-Aminolävulinsäure (δ-ALA, 20%) topisch aufgetragen und das Gewebe mit rotem Licht (570–750 nm; 180 J/cm 2 ) bestrahlt. Die PDT wurde zweimal wiederholt. Hierdurch konnte klinisch eine nahezu vollständige Remission des Tumors erzielt werden. In der photodynamischen Diagnostik (PDD) zeigten sich aber noch deutlich einige persistierende, z.T. die Mamille infiltrierende Basaliominseln. Diese wurden durch Exzision entfernt und anschließend wurde der Defekt mittels einer Mamillenplastik verschlossen. Durch die Kombination der beiden Therapieformen konnte der großflächige Tumor mit einem ausgezeichneten kosmetischen Ergebnis behandelt werden. Die Falldarstellung soll zum einen die Grenzen der topischen PDT, zum anderen die Effizienz der PDD bei gezieltem Einsatz darstellen.
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Der Hautarzt 47 (1996), S. 47-52 
    ISSN: 1432-1173
    Keywords: Schlüsselwörter Quallenverletzung ; Ulzeröse Kontaktdermatitis ; Rezidivierende allergische Exantheme ; Cnidariatoxin-Antigene ; Jellyfish-ELISA ; Therapie von Quallenverletzungen ; Key words Jellyfish injury ; Ulcerated contact dermatitis ; Recurring allergic rash ; Cnidariatoxin antigens ; ELISA ; Treatment of jellyfish injuries
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary We are presenting a 47-year-old woman who was stung by jellyfish while bathing in the sea of Thailand. Immediately after the injury she developed sharp pain and urticarial erythema of the skin of the knees accompanied by muscle cramps of the entire body. After a few days a toxic contact dermatitis with edematous swelling and ulcerations developed, which did not respond to topical antibiotics or corticosteroids. Three weeks later the patient presented with a disseminated urticarial eruption, which at first responded well to topical treatment and systemic corticosteroids. Over the next few weeks, however, a relapse of the eruption and the ulcerations occurred. Raised titres of IgG and IgM antibodies against different jellyfish from the Indian and Pacific Ocean were detected in the patient's serum by the enzyme-linked immunosorbent assay. Antibodies against bees (class 1) and wasps (class 4) were found by the radioallergosorbent test. The clinical features and the immunological findings led to the diagnosis of toxic and allergic contact dermatitis to jellyfish venom. First aid and secondary treatment of jellyfish injuries are suggested.
    Notes: Zusammenfassung Wir berichten über eine 47jährige Patientin, die in Thailand beim Baden im Meer Verletzungen durch eine sogenannte Feuerqualle erlitt. An den betroffenen Hautarealen, an den Knien, traten unmittelbar stechende Schmerzen und urtikarielle Erytheme auf, begleitet von generalisierten Muskelkrämpfen. Nach einigen Tagen entwickelte sich eine toxische Kontaktdermatitis mit ödematöser Schwellung und Ulzerationen, die unter der Behandlung mit antibiotischen und glukokortikoidhaltigen Salben nicht abheilten. Drei Wochen später stellte sich die Patientin erstmals mit einem disseminierten urtikariellen Exanthem vor. Unter Lokaltherapie und der systemischen Gabe von Glukokortikoiden kam es zur Rückbildung des Exanthems, in der Folgezeit jedoch zu rezidivierenden Exanthemschüben und Ulzerationen an den Knien. Im Serum der Patientin wurden im ELISA stark erhöhte IgG- und zum Teil IgM-Antikörper-Titer gegen 9 überwiegend im Indischen und Pazifischen Ozean beheimatete Quallenarten gefunden. Im RAST wurde Klasse 1 gegen Bienen-, Klasse 4 gegen Wespengift nachgewiesen. Aufgrund der Klinik, des Verlaufs und der immunologischen Befunde stellten wir die Diagnose einer toxischen und allergischen Reaktion auf Quallenbestandteile. Sofortmaßnahmen und nachfolgende Behandlung von Quallenverletzungen werden tabellarisch dargestellt.
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  • 6
    ISSN: 1432-1173
    Keywords: Schlüsselwörter Extrakorporale Photopherese ; Diskoider Lupus erythematodes ; Kutaner Lupus erythematodes ; Photochemotherapie ; Key words Extracorporeal photopheresis ; Discoid lupus erythematosus ; Cutaneous lupus erytheamatosus ; Photochemotherapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Lupus erythematosus (LE) is a disease with a wide spectrum of cutaneous and systemic manifestations. Discoid LE (DLE) is the most common form of cutaneous LE; the disseminated form of DLE is rare. We report an encouraging response to treatment with extracorporeal photopheresis (ECP) in a single patient with disseminated DLE who did not respond to conventional therapy. To the best of our knowledge this is the first successful use of ECP in the management of such a patient. Extracorporeal photopheresis is a therapeutic modality that has been under investigation for more than 12 years. Although originally developed for the treatment of cutaneous T-cell lymphoma, ECP has recently been used for the management of autoimmune diseases including systemic scleroderma, pemphigus vulgaris and SLE, as well as prevent organ rejection in patients with cardiac or kidney transplants and graft versus host disease after bone marrow transplantation.
    Notes: Zusammenfassung Der Lupus erythematodes (LE) ist eine Erkrankung, die ein breites Spektrum von kutanen und systemischen Manifestationen zeigt. Der diskoide LE (DLE) wird am häufisten beobachtet, seltener ist die disseminierte Variante des DLE. Wir berichten über eine Patientin mit disseminiert diskoidem Lupus erythematodes (DDLE), die auf alle angewendeten konventionellen Therapien nicht ansprach; ein erstmals bei diesem Krankheitsbild durchgeführter Behandlungsversuch mit extrakorporaler Photopherese (ECP) führte zu einer raschen und deutlichen Besserung der Hautveränderungen. Die ECP stellt seit über 12 Jahren ein inzwischen erprobtes Behandlungsverfahren dar. Obwohl zunächst zur Behandlung des kutanen T-Zellymphom entwickelt, kommt die ECP heute zunehmend erfolgreich bei der Therapie von Autoimmun- und entzündlichen Erkrankungen zum Einsatz, z.B. systemische Sklerodermie, systemischer Lupus erythematodes (SLE), Pemphigus vulgaris, Abstoßungsreaktionen nach Herz- oder Nierentransplantation und „graft-versus-host-disease” (GvHD), nach Knochenmarktransplantation.
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  • 7
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract The porphyrias are disorders that result from the inherited or acquired dysregulation of one of the eight enzymes in the heme biosynthetic pathway. Variegate porphyria (VP) is characterized by deficiencies in protoporphyrinogen oxidase (PPO) and has recently been genetically linked (Z = 6.62) to the PPO gene on chromosome 1q21. In this study, we have identified two sequence variants in the PPO gene in a family with VP. The first is a neutral polymorphism at the -47 position of intron 2; this polymorphism is present in the general population and is unlikely to underlie the VP phenotype. The second is a mutation in the PPO gene in a patient with VP; the mutation consists of an apparently de novo 2-bp insertion in exon 3 of PPO and results in a frameshift and downstream premature termination codon. These data establish that a frameshift mutation in PPO is the underlying mutation in this patient with VP and explain the sporadic occurrence of the phenotype in this family.
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