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  • 1
    ISSN: 1432-1173
    Keywords: Schlüsselwörter: Kongenitale Poikilodermie – Thomson-Syndrom – Hyperkeratosen – Malignität – Myopathie ; Key words: Poikiloderma congenitale – Thomson syndrome – Hyperkeratoses – Malignancy – Myopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary. This report deals with a 15-year-old girl suffering from the verrucous type of Thomson's syndrome. Initial poikilodermatous skin changes developed on both cheeks at the age of 3 months. Subsequently, rapid generalization of typical skin findings was observed. The clinical heterogenity of this syndrome is discussed with reference to the existing literature and the present case. Up to now, very few comparable cases of associated neurological symptoms have been described.
    Notes: Zusammenfassung. Wir berichten über den Fall einer 15jährigen Patientin vom Typus verrucosus des Thomson-Syndroms. Initiale poikilodermatische Hautveränderungen an den Wangenpartien manifestierten sich im 3. Lebensmonat. Nachfolgend kam es zur schnellen Progression der typischen Hautveränderungen auf das gesamte Integument. Anhand dieses Falles wird unter gleichzeitiger Literaturberücksichtigung die klinische Heterogenität dieses Krankheitsbildes illustriert. Über assoziierte neurologische Symptome, wie in unserer Kasuistik, ist bislang nur außerordentlich selten berichtet worden.
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  • 2
    ISSN: 1432-1173
    Keywords: Schlüsselwörter ; Herpes gestationis ; Schwangerschaftsdermatose ; Bullöses Pemphigoid ; BP180 ; Pruritus ; Key words ; Herpes gestationis ; Dermatoses of pregnancy ; Bullous pemphigoid ; BP180 ; Pruritus
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Background and Objective: Pemphigoid gestationis (PG) is a rare pregnancy-associated autoimmune bullous disease characterized by autoantibodies to the 180 kD bullous pemphigoid antigen (BP180). The clinical spectrum of PG is polymorphic and for diagnostic purposes, a skin biopsy is usually taken demonstrating the deposition of autoantibodies. Patients and Methods: From 2 patients, skin biopsies were obtained for histopathologic and immunofluorescence studies. Circulating autoantibodies were characterized by immunoblotting and ELISA using a recombinant form of the immunodominant BP180 NC16 A domain. Results: The 2 PG patients described here did not show blisters but complained about severe itching. In the first case, PG presented in the first trimester of the second pregnancy as an erythema-multiforme-like disease. The second patient developed urticarial plaques a few days after delivery. PG was diagnosed by the detection of autoantibodies against recombinant BP180 NC16 A by immunoblot and ELISA analysis and confirmed by linear deposits of C3 at the cutaneous basement membrane zone on direct immunofluorecence microscopy. Skin lesions healed with oral prednisolon. Conclusions: In our two patients, non-bullous PG could be diagnosed by serological tests. Immunmoblotting and ELISA might be sensitive and specific tools when screening sera of patients with pruritic skin lesions in pregnancy for the presence of autoantibodies to BP180. In some cases, these newer techniques may make a skin biopsy unnecessary.
    Notes: Zusammenfassung Hintergrund und Fragestellung: Das Pemphigoid gestationis (PG) ist eine seltene, schwangerschaftsspezifische Autoimmundermatose mit Autoantikörpern gegen das 180 kD bullöse Pemphigoid Antigen (BP180). Das klinische Bild ist polymorph. Die Diagnose des PG wird meist anhand einer Biopsie mit Nachweis der Autoantikörper in der Haut der Patientinnen gesichert. Patienten/Methodik: Bei 2 Patientinnen wurden Hautbiopsien histopatholgisch und immunfluoreszenzoptisch untersucht. Die Autoantikörper wurden mittels Immunoblot und ELISA unter Verwendung einer rekombinanten Form der immundominanten BP180-NC16-A-Domäne charakterisiert. Ergebnisse: Bei den beiden vorgestellten Patientinnen verlief das PG ohne Blasenbildung, jedoch mit starkem Juckreiz. Bei unserer ersten Patientin manifestierte sich die Erkrankung im 1. Trimenon unter dem Bild eines Erythema multiforme, der 2. Fall trat wenige Tage postpartal unter dem Bild einer Urtikaria auf. Die Diagnose wurde jeweils durch den Nachweis von Antikörpern gegen rekombinantes BP180 NC16 A im Immunoblot und ELISA und durch lineare C3-Ablagerungen an der Basalmembran in der direkten Immunfluoreszenz gesichert. Die Hautveränderungen heilten bei beiden Patientinnen unter oraler Prednisolontherapie ab. Schlussfolgerung: Bei den beiden vorgestellten Patientinnen wurde anhand von Serumuntersuchungen ein PG ohne Blasenbildung diagnostiziert. Sowohl Immunoblot als auch ELISA erscheinen aufgrund ihrer hohen Sensitivität und Spezifität als Screeningverfahren zum Nachweis von Autoantikörpern gegen BP180 bei pruriginösen Schwangerschaftsdermatosen geeignet. In der Zukunft dürften sie mitunter die bisher unverzichtbare Hautbiopsie zur Diagnose eines PG ersetzen.
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  • 3
    ISSN: 1365-2230
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Temporal arteritis, a variant of giant cell arteritis, is a systemic granulomatous vasculitis of large and medium-sized arteries. Usually the clinical Features arc dominated by ophthalmological and neurological complications. In rare instances, ischaemic necrosis, especially of the scalp, may lead patients to the dermatologist. We report a 76-year-old woman presenting with a unilateral scalp necrosis, accompanied by a dramatic ipsilateral impairment of vision. Immediately after duplex-sonography of the extracranial vessels and after initiation of corticosteroid therapy, the diagnosis of temporal arteritis was confirmed by temporal art en biopsy. One month later, because of insufficient secondary healing of the ulcer, the defect was covered by a mesh graft. The taking of the graft was delayed due to immunosuppressive therapy, but was complete. The patient unfortunately died as a result of complications related to surgical removal of an aspergilloma in the sphenoid cavity secondary to immunosuppressive therapy. We discuss the technique of artery biopsy and the possibility of surgical management of scalp necrosis in temporal arteritis.
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  • 4
    Electronic Resource
    Electronic Resource
    Oxford, UK : Munksgaard International Publishers
    Allergy 58 (2003), S. 0 
    ISSN: 1398-9995
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
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  • 5
    Electronic Resource
    Electronic Resource
    Oxford [u.a.] : International Union of Crystallography (IUCr)
    Acta crystallographica 42 (1986), S. 743-745 
    ISSN: 1600-5759
    Source: Crystallography Journals Online : IUCR Backfile Archive 1948-2001
    Topics: Chemistry and Pharmacology , Geosciences , Physics
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Type 2 helper T-cell immune responses can be demonstrated in the human atopic disorders atopic dermatitis and allergic asthma/rhinoconjunctivitis. The CD30 (Ki-1) antigen, originally described on Hodgkin and Reed-Sternberg cells, has recently been proposed as a marker of T cells with potent B-cell helper activity producing IL-5 and γ-IFN, as well as on CD4+ and CD8+ T cells with a Th2 cytokine profile. As a soluble form of CD30 (sCD30) is released by CD30+ cells in vivo, we studied its clinical significance in atopic disorders compared with allergic contact dermatitis and healthy controls. Elevated sCD30 levels were associated with atopic dermatitis (P 〈 0.0001), but not with respiratory atopic disorders or allergic contact dermatitis. sCD30 levels in patients with atopic dermatitis were independent of serum IgE. The particular occurrence of serum sCD30 in patients with atopic dermatitis indicates a special regulatory function of CD30+ cells in this disease.
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