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  • 1
    ISSN: 1600-0625
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract:  Pemphigus foliaceus (PF) is a severe autoimmune bullous disorder, characterized by autoantibodies (autoAb) against desmoglein 1 (Dsg1). As T cells may be critical in the pathology of PF, the aim of the present study was to identify and characterize autoaggressive T-helper cells reactive to Dsg1 in PF patients and healthy individuals. Eight patients with the clinical diagnosis of PF and six HLA class II-matched healthy individuals were examined. By magnetic cell-sorting (MACS) cytokine-secretion assay, Dsg1-responsive T-helper (Th) 1 and Th2 cells were isolated and cloned by limiting dilution. The generated T-cell clones (TCC) were characterized regarding proliferative response, TCR Vβ-chain usage, and cytokine profile upon in vitro stimulation with Dsg1. Both Dsg1-reactive Th1 and Th2 cells were detected in PF patients and controls at similar frequencies. A total of 15 Th1 and Th2 clones were isolated from patients and 27 TCC from healthy controls. Analysis of TCR Vβ-chain usage of autoreactive T cells from both groups revealed no predominance of a specific Vβ chain. Noteworthy, the isolated TCC showed a polarized Th1- or Th2-like phenotype upon in vitro culture and stable expression of Th1 or Th2 cytokines during long-term in vitro culture. In summary, our data demonstrate that T-cell autoreactivity against Dsg1 is not restricted to patients with PF. Moreover, both Th1 and Th2 cells were present in patients and healthy donors, suggesting that the loss of B-cell tolerance against Dsg1 in PF is not exclusively determined by the presence of autoaggressive T cells.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] Focal dermal hypoplasia (FDH) is an X-linked dominant multisystem birth defect affecting tissues of ectodermal and mesodermal origin. Using a stepwise approach of (i) genetic mapping of FDH, (ii) high-resolution comparative genome hybridization to seek deletions in candidate chromosome areas and ...
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1173
    Keywords: Schlüsselwörter: Extraabdominelle Fibromatose – Desmoidtumor – Keloid – Ileus – Gardner-Syndrom ; Key words: Extraabdominal fibromatosis – Desmoid tumor – Keloid – Ileus – Gardner's syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract. The report deals with the clinical features of a 26-year-old man with the extraabdominal form of deep fibromatosis, which became manifest at the age of 18 years as acute ileus caused by diffuse intraabdominal fibromatous tumours. In the later course of the disease extensive fibromatous lesions developed, mainly at the skin. In addition, multiple keloids occurred after excision or after negligible traumas. The pathogenetic events of the clinical picture described may be linked to the activation of mesenchymal cells leading to the generation of either fibromatous tumours or posttraumatic keloids.
    Notes: Zusammenfassung. Vorgestellt wird ein 26jähriger Mann mit der extraabdominellen Form einer tiefen Fibromatose, die sich erstmalig im 18. Lebensjahr als akuter Ileus aufgrund ausgeprägter intraabdomineller Bindegewebstumoren manifestierte. Später traten flächenhafte, fibromatöse Bindegewebstumoren bevorzugt an der Haut auf. Neben diesen spontan entstandenen kutan und subkutan gelegenen Hauttumoren fanden sich auch Keloide nach Schnittverletzungen oder Bagatelltraumata an der Haut. Diesem Krankheitsbild liegt möglicherweise eine Aktivierung mesenchymaler Zellen zugrunde, die bei entsprechender Disposition zu fibromatösen Tumoren oder posttraumatischen Keloiden führt.
    Type of Medium: Electronic Resource
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