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  • 1
    Electronic Resource
    Electronic Resource
    Inflammatorischer Typ der Epidermolysis bullosa acquisita (1994)
    Springer
    Der Hautarzt 45 (1994), S. 166-170 
    Details
    ISSN: 1432-1173
    Keywords: Schlüsselwörter: Epidermolysis bullosa acquisita – Bullöses Pemphigoid – Vernarbendes Pemphigoid – Indirekte Immunfluoreszenz – Immunelektronenmikroskopie – Immunoblot ; Key words: Epidermolysis bullosa acquisita – Bullous pemphigoid – Cicatricial pemphigoid – Indirect immunfluorescence – Immunoelectronmicroscopy – Immunoblotting
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract. Epidermolysis bullosa acquisita (EBA) is an autoimmune subepidermal blistering disease, which can present in various clinical forms. We report on a 73-year-old male patient with the inflammatory type of EBA, which is clinically similar to bullous pemphigoid. Histological examination revealed subepidermal blister formation, and direct immunofluorescence of perilesional skin revealed a linear deposition of IgG and C3 at the basement membrane. Indirect immunofluorescence with 0.9% NaCl-separated human skin as substrate showed the presence of circulating antibodies binding to the base of the artificial blister at a titre of 1:320. Indirect immunoelectronmicroscopy revealed immune deposits localized in the area of the sublamina densa. Immunoblotting of dermal extracts disclosed binding of serum antibodies to a 290-kDa protein. Systemic therapy with diaminodiphenyl sulphone, initially in combination with corticosteroids, resulted in complete healing of the skin lesions. With reference to this case report, we discuss the clinical pictures possible in EBA, the differential diagnosis and the treatment options.
    Notes: Zusammenfassung. Die Epidermolysis bullosa acquisita (EBA) ist eine blasenbildende Autoimmundermatose, die verschiedene klinische Verlaufsformen aufweist. Wir berichten über einen 73jährigen Patienten, der an der inflammatorischen, generalisierten Form der EBA leidet, die klinisch an ein bullöses Pemphigoid erinnert. Histologisch fand sich eine subepidermale Blasenbildung, in der direkten Immunfluoreszenz periläsionaler Haut eine lineare Ablagerung von IgG und C3 an der Basalmembran. In der indirekten Immunfluoreszenz auf 0,9% NaCl-sparierter humaner Spalthaut wurden hochtitrig zirkulierende Serumantikörper nachgewiesen, die im Boden der artifiziellen Blase banden. In der indirekten Immunelektronenmikroskopie erkannten die Antikörper Strukturen im Bereich der Sublamina densa. Im Immunoblot dermaler Extrakte banden die Serumantikörper an ein 290-kD-Protein. Unter systemischer Therapie mit Diaminodiphenylsulfon, zeitweise kombiniert mit Kortikosteroiden, wurde Abheilung erzielt. Anhand dieses Fallberichts diskutieren wir das klinische Spektrum der EBA sowie deren Differentialdiagnose und die therapeutischen Optionen.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001050050057
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  • 2
    Electronic Resource
    Electronic Resource
    Distribution of macromolecular components in human dermal connective tissue (1982)
    Springer
    Archives of dermatological research 273 (1982), S. 115-120 
    Details
    ISSN: 1432-069X
    Keywords: Collagens ; Glycosaminoglycans ; Noncollagenous glycoprotein(s)
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Normal human skin was sliced into five horizontal layers and distribution of type I and type III collagens, glycosaminoglycans, and noncollagenous glycoprotein(s) among the five layers was analyzed. No remarkable differences in the relative contents of type I and type III collagens and noncollagenous glycoprotein(s) were detected among the five layers. However, glycosaminoglycan content was higher in the upper layers than in the lower and the ratio of hyaluronic acid to dermatan sulfate was also higher in the upper layers. These results are compared and discussed with our previous data on calf skin.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00509035
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  • 3
    Electronic Resource
    Electronic Resource
    Reply to the letter to the editor by Beutner et al. (1981)
    Springer
    Archives of dermatological research 270 (1981), S. 221-221 
    Details
    ISSN: 1432-069X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00408238
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  • 4
    Electronic Resource
    Electronic Resource
    Does the position of the premature termination codon in COL7A1 correlate with the clinical severity in recessive dystrophic epidermolysis bullosa? (2004)
    Oxford, UK; Malden, USA : Munksgaard International Publishers
    Experimental dermatology 13 (2004), S. 0 
    Details
    ISSN: 1600-0625
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract:  Recessive dystrophic epidermolysis bullosa (RDEB) is an inherited skin disease caused by mutations in the gene encoding type VII collagen (COL7A1). The mutations are highly variable and this greatly complicates the study of the genotype–phenotype relationships. To date, three recurrent mutations, specific to Japanese RDEB patients have been reported. By comparing the phenotypes of RDEB patients with different recurrent mutations, the upstream positions of the premature termination codons (PTCs) showed strong correlation with the RDEB clinical disease severity. However, such correlations have not been supported by patients with mutations that were different from these recurrent Japanese patients mutations. In this study, we report a case of RDEB with a very mild clinical phenotype, who was a compound heterozygote harbouring both a recurrent Japanese mutation and a novel deletion mutation resulting in a more upstream PTC. The patient and his mother were shown to have a recurrent donor splice site mutation within intron 81 (6573 + 1G 〉 C), a recurrent Japanese mutation that activates a cryptic donor splicing site and results in a downstream PTC. The patient and his father shared a single-nucleotide deletion within exon 64 (5504delA), which causes a downstream frame shift in five amino acids before creating a PTC. Occurrence of the PTCs in mRNA was confirmed by reverse transcription-polymerase chain reaction (RT)-PCR. The patient's skin showed reduced immunofluorescence staining for COL7A1 and reduced number of abnormal or short anchoring fibrils by electron microscopy. Although the position of the mutation 5504delA PTC was located upstream of the previous mutations reported in combination with the 6573 + 1G 〉 C mutation, the two mutations together give an apparently milder clinical phenotype. Therefore, genotype–phenotype relationships in RDEB cannot be explained purely by the position of PTC.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.0906-6705.2004.00167.x
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  • 5
    Electronic Resource
    Electronic Resource
    Upregulation of P-cadherin expression in the lesional skin of pemphigus, Hailey-Hailey disease and Darier’s disease (2001)
    Copenhagen : Munksgaard International Publishers
    Journal of cutaneous pathology 28 (2001), S. 0 
    Details
    ISSN: 1600-0560
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Background: Autoimmune blistering diseases, pemphigus vulgaris (PV) and pemphigus foliaceus (PF), are known to be caused by binding of autoantibodies to the desmosomal cadherins, desmoglein 3 and desmoglein 1, respectively. Recently, mutations in the genes coding Ca2+ pumps leads to inherited blistering diseases, Hailey-Hailey disease (HHD) and Darier’s disease (DD). Cadherins are a family of Ca2+-dependent cell adhesion molecules and P-cadherin is one of the major cadherins expressed in the epidermis. Although detailed mechanisms of acantholysis of these blistering diseases have not been fully clarified, abnormal expression of cadherins caused by altered Ca2+ concentration due to the binding of autoantibodies to cell surface or by mutations in Ca2+ pumps is suggested to be involved in mechanisms of acantholysis of these atuoimmune and inherited blistering diseases. The purpose of the present study was to determine whether altered P-cadherin expression is present in these diseases.Method: Distribution patterns of P-cadherin in skin specimens from patients with PV (n=2), PF (n=2), HHD (n=4) and DD (n=3), were examined with confocal laser scanning microscopy using two anti-P-cadherin antibodies, 6A9 and NCC-CAD-299.Results: In normal control skin, P-cadherin expression was restricted to the basal layer. In contrast, positive immunostaining of P-cadherin was observed not only in the basal cells, but also in the suprabasal cells in lesional skin of all the acantholytic diseases.Conclusions: The present results clearly demonstrated that upregulation of P-cadherin expression occurs in the acantholysis in all the four blistering diseases PV, PF, HHD and DD. Upregulation of P-cadherin may be involved in the pathomechanism of both the autoimmune blistering diseases and the inherited blistering diseases.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1034/j.1600-0560.2001.028006277.x
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  • 6
    Electronic Resource
    Electronic Resource
    Detection of autoantibodies against bullous pemphigoid and pemphigus antigens by an enzyme-linked immunosorbent assay using the bacterial recombinant proteins (1995)
    Oxford, UK : Blackwell Publishing Ltd
    Experimental dermatology 4 (1995), S. 0 
    Details
    ISSN: 1600-0625
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract To develop a new method to evaluate autoantibodies in various autoimmune bullous skin diseases, we examined reactivity of bullous pemphigoid (BP) and pemphigus vulgaris (PV) patients' sera with partial bacterial fusion proteins of the 230 kD BP antigen (BPAG1) and PV antigen, respectively, by an enzyme-linked immunosorbent assay (ELISA), and compared the results with those of immunoblotting. We used two fusion proteins derived from the mouse BPAG1 and a fusion protein derived from the amino-terminus (EC 1-2) of PV antigen. For both BP and PV sera, the ELISA scores were well correlated with the reactivities on immunoblot assays. The present study indicates that ELISA using recombinant antigen proteins in various autoimmune bullous skin diseases will be a new useful technique to detect the autoantibodies. However, development of recombinant proteins with the entire molecule and correct conformation will be necessary to establish a perfect ELISA system in the future.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1600-0625.1995.tb00232.x
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  • 7
    Electronic Resource
    Electronic Resource
    A pemphigus case with long term survival, implicating the spectrum between paraneoplastic pemphigus and pemphigus vulgaris (1997)
    Oxford, UK : Blackwell Publishing Ltd
    International journal of dermatology 36 (1997), S. 0 
    Details
    ISSN: 1365-4632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-4362.1997.tb04168.x
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  • 8
    Electronic Resource
    Electronic Resource
    85th Annual Meeting of the Japanese Dermatological Association May 16–18, 1986 (1987)
    Oxford, UK : Blackwell Publishing Ltd
    International journal of dermatology 26 (1987), S. 0 
    Details
    ISSN: 1365-4632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-4362.1987.tb02281.x
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  • 9
    Electronic Resource
    Electronic Resource
    Pyloric atresia-junctional epidermolysis bullosa syndrome showing novel 594insC/Q425P mutations in integrin β4 gene (ITGB4) (2004)
    Oxford, UK; Malden , USA : Munksgaard International Publishers
    Experimental dermatology 13 (2004), S. 0 
    Details
    ISSN: 1600-0625
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract:  Pyloric atresia-junctional epidermolysis bullosa syndrome (PA-JEB) is an autosomal recessive inherited rare blistering disorder caused by mutations in ITGA6 or ITGB4, genes encoding integrin α6 or β4, respectively. In this study, we have disclosed the mutations in ITGB4 in a Korean patient with PA-JEB. The proband, who showed skin blisters, was diagnosed as having pyloric atresia and died 2 years after birth. Mutational analysis showed a novel 594insC maternal mutation in exon 7, which led to premature termination codon (PTC), and a novel Q425P paternal mutation in exon 11. Q425P mutation was not detected in 200 alleles obtained from a normal healthy Korean control, and was shown to reduce α-helix forming ability in integrin β4 a by Garnier α-helicity plot of the protein, indicating that this mutation is pathogenic but not polymorphism. The phenotype in the present case can be explained by (1) the combination of PTC and missense mutation, and (2) amino-acid substitution occurring for the amino acid not preserved in the integrin β family. Our results contribute to further the accumulation of mutation data for better understanding of the genotype/phenotype correlation in PA-JEB, and may give profound insight into the role of integrins α6 and β4.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.0906-6705.2004.00107.x
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  • 10
    Electronic Resource
    Electronic Resource
    Bullöses Pemphigoid unter dem Bild einer Prurigo simplex subacuta (1995)
    Springer
    Der Hautarzt 46 (1995), S. 789-795 
    Details
    ISSN: 1432-1173
    Keywords: Schlüsselwörter Immunfluoreszenz ; Immunoblot ; Spalthaut ; Bullöses-Pemphigoid-Antigen ; Key words Immunofluorescence ; Immunoblotting ; Split skin ; Bullous pemphigoid antigen
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Bullous pemphigoid (BP) is a bullous autoimmune disease of the elderly; it is characterized by tense bullae on both erythematous and otherwise apparently normal skin. Several clinical variants of BP have been described, and we now add our observations of two BP cases mimicking subacute prurigo. Both patients had suffered from intensely pruritic excoriated papules for several months before presentation. Blisters had never developed and did not occur during follow up. Histology showed changes of chronic dermatitis. In the serum of both patients, indirect immunofluorescence on NaCl-separated human skin revealed the presence of circulating antibodies binding to the roof of the artificial blisters. Perilesional skin biopsies showed linear IgG or C3 deposits in the basement membran zone. Immunoblotting of epidermal and dermal extracts demonstrated the binding of the antibodies to an epidermal 230-kD protein, which is the known major bullous pemphigoid antigen. These cases are described as a basis for discussion of the clinical spectrum of bullous pemphigoid.
    Notes: Zusammenfassung Das bullöse Pemphigoid (BP) ist eine blasenbildende Autoimmundermatose des höheren Lebensalters. Neben dem klassischen klinischen Bild mit prallen Blasen auf gesunder oder erythematöser Haut sind eine Reihe klinischer Varianten beschrieben worden. Wir berichten erstmals über das Auftreten eines bullösen Pemphigoids unter dem Bild einer Prurigo simplex subacuta. Bei zwei Patientinnen bestanden seit mehreren Monaten stark juckende, vielfach exkoriierte Papeln. Blasen hatten nie bestanden und traten auch in der Folge nicht auf. Das histologische Bild entsprach in beiden Fällen einem chronischen Ekzem. Die Diagnose eines BP konnte jeweils mit der indirekten Immunfluoreszenz durch den Nachweis zirkulierender IgG-Antikörper im Serum gesichert werden, die auf NaCl-separierter Spalthaut im Dach der artifiziellen Blase banden. Die direkte Immunfluoreszenz mit linearen IgG- bzw. C3-Ablagerungen an der Basalmembran bestätigte diesen Befund. Im Immunoblot epidermaler Extrakte präzipitierten beide Seren das 230-kD BP-Antigen. Am Beispiel dieser Fälle diskutieren wir das klinische Spektrum des bullösen Pemphigoids.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001050050340
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