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  • 1
    Digitale Medien
    Digitale Medien
    IgA-epidermolysis bullosa acquisita in a child resulting in blindness (1997)
    Oxford, UK : Blackwell Science Ltd
    British journal of dermatology 137 (1997), S. 0 
    Details
    ISSN: 1365-2133
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal bullous disease characterized by IgG autoantibodies directed against type VII collagen, the major component of anchoring fibrils. The classical phenotype of EBA is a non-inflammatory, mechanobullous disease resembling the dystrophic forms of inherited epidermolysis bullosa. Mucous membrane involvement is frequent but usually mild. We report a 1-year-old girl suffering from IgA-EBA, who presented with an initial eruption of disseminated urticarial lesions and tense blisters of the skin but subsequently developed severe oral and ocular lesions reminiscent of cicatricial pemphigoid. Direct immunofluorescence of the skin and buccal mucosa revealed linear IgA and C3 at the basement membrane zone (BMZ). IgA anti-BMZ autoantibodies stained the dermal side of salt-split skin by indirect immunofluorescence and recognized a dermal protein of 290 kDa co-migrating with type VII collagen by immunoblotting. Direct and indirect immunoelectron microscopy revealed IgA deposits overlying the anchoring fibrils. The ocular involvement led to total blindness in spite of intense treatment. This case of childhood IgA-EBA is particularly striking because of the cicatricial pemphigoid phenotype with severe ocular involvement which resulted in blindness. It reinforces the necessity to use modern immunological methods to classify autoimmune bullous diseases in order to allow early and appropriate treatment.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1046/j.1365-2133.1997.18191915.x
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  • 2
    Digitale Medien
    Digitale Medien
    A gamma-camera distortion corrector using a programmable on-line microcomputer
    Amsterdam : Elsevier
    Nuclear Instruments and Methods In Physics Research 211 (1983), S. 561-564 
    Details
    ISSN: 0167-5087
    Quelle: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Thema: Energietechnik , Physik
    Materialart: Digitale Medien
    URL: http://linkinghub.elsevier.com/retrieve/pii/0167-5087(83)90290-9
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  • 3
    Digitale Medien
    Digitale Medien
    Immunoelectron microscopic study of the conjunctiva in cicatricial pemphigoid (1999)
    Springer
    Graefe's archive for clinical and experimental ophthalmology 237 (1999), S. 39-44 
    Details
    ISSN: 1435-702X
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract  · Background: Immune deposits can be found on the conjunctival basement membrane zone of patients affected by cicatricial pemphigoid using immunofluorescence technique. The purpose of this study was to perform direct immunoelectron microscopy on the conjunctiva of patients with scarring conjunctivitis associated with cicatricial pemphigoid · Methods: Conjunctival and skin biopsies were performed in six patients who presented with presumed autoimmune cicatrizing conjunctivitis associated with cicatricial pemphigoid. Specimens were processed for direct immunofluorescence and direct immunoelectron microscopy. · Results: Direct immunofluorescence was positive in all skin samples and in three of six conjunctival samples. Direct immunoelectron microscopy showed immune deposits in the lamina lucida and the lamina densa of all skin and conjunctival samples. · Conclusions: Direct immunoelectron microscopy can be performed on the conjunctiva. It shows the precise localization of cicatricial pemphigoid target antigens within the conjunctival basement membrane zone.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/s004170050192
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